Ptha 2431 - Management Of Neurological Disorders - Martin And Kessler Book And Miele Lecture - Exam 2

Front 1

What is NDT (Bobath concept)? ***

Back 1

- based on developmental progression from spinal cord to cortex
- begins with reflexive behavior and progresses to volitional activity
- requires stimulus to get response

Front 2

What is motor control? ***

Back 2

- movement starts by pattern within the brain
- does not require stimulus
- activity becomes more complex by feedback loop

- ability to maintain and change posture and movement

- process involved: motor, cognitive and perceptual development
--- first self-movement, then in relation to environment

- includes which muscles to use, order of activation, and timing

Front 3

List some motor control problems. ***

Back 3

- overcoming gravity
- lifting a large head
- stability versus mobility,
- task specific movement

Front 4

What are the time scales used for:
- motor control
- motor learning
- motor development? ***

Back 4

- milliseconds to move or react

- hours, days or months to change a movement pattern or plan

- months, years or decades to change from reflex to voluntary isolated, timed, sequenced control

Front 5

What are the two theories of motor control discussed? ***

Back 5

- hierarchic model (traditional)

- systems model

Front 6

Describe the hierarchic model of motor control development. ***

Back 6

- spinal reflex to tonic brain stem reflex, to midbrain righting reactions to equilibrium

- lower structures inhibited by higher ones

- integration is how less mature responses are incorporated into voluntary movement

Front 7

How does motor control develop according to the hierarchic model of motor control? ***

Back 7

- mobility – begins with random uncontrolled movement
- stability – maintaining posture against gravity

- controlled mobility – attempts to crawl, creep
- skill – creeping, walking

Front 8

How does postural control develop according to the hierarchic model of motor control? ***

Back 8

- cephalocaudal and proximal to distal progression
1. righting reactions – get up, turn around
2. protective reactions – to stay up using extremities
3. equilibrium reactions – postural control

Front 9

Describe the systems model of motor control development (3 characteristics.) ***

Back 9

1. more complex interaction of nervous, cardiopulmonary, musculoskeletal systems to affect posture and movement systems

2. self-organizing by changing relationships between systems produce different motor responses

3. feedback loop - closed versus open

Front 10

What are the components of the postural control system according to the systems model of motor control? ***

Back 10

- limits of stability
- environmental adaptation
- musculoskeletal system

- predictive central set – postural readiness (body knows how to react)
- motor coordination
- eye-head stabilization

- sensory organization
- sway strategies - ankle, hip, stepping

Front 11

What are the 5 characteristics of the life span concept of development? ***

Back 11

development is:
- contextual
- life-long
- embedded in history
- multidimensional
- plastic and flexible

Front 12

What are the developmental time periods? ***

Back 12

- infancy (birth - 2 years)
- childhood (2-10/12 years; M/F)
- adolescence (10/12 - 18/20 years; M/F)
- adulthood

(Levinson further divided adulthood:)
- early adulthood (18/20 - 40 years; M/F)
- middle adulthood (40-65 years)
- older adulthood (65-death)
--- young-old - 65-74 years
--- middle-old - 75-84 years
--- old-old - 85 and older

Front 13

What is neuromotor development? ***

Back 13

- progression of infant through predictable stages of sensorimotor development from simple to complex tasks

- development of posture and movement, especially 0-18 months

Front 14

What is the first stage seen in neuromotor development (hierarchic theory)? What are the characteristics of this stage? ***

Back 14

primitive reflexes (spinal cord/simple reflexes)
- spinal level: present in gestation and at birth; controlled at spinal cord level
- protective/survival mechanisms

- gross movement pattern – flexion/extension pattern
- “Integrated” by 8 weeks normal development

(e.g., flexor withdrawal, extensor thrust, crossed extension, rooting grasp)

Front 15

What is the second stage seen in neuromotor development (hierarchic theory)? What are the characteristics of this stage? ***

Back 15

tonic reflexes (brain stem/lowest level reflexes)
- muscle tone & posture changes due to head/neck motion
- positive support or motion of body part – associated reaction
(e.g., asymmetric tonic neck reflex, tonic labyrinthine reflex)

Front 16

What is the third stage seen in neuromotor development (hierarchic theory)? What are the characteristics of this stage? ***

Back 16

righting reaction (midbrain level)

- keeps head and body in vertical alignment (get you up, turn you around)
- roll, sit up, get on hands and knees
- protect from falls
- neck righting, body righting, optical righting, labyrinthine righting

Front 17

What is the fourth stage seen in neuromotor development (hierarchic theory)? What are the characteristics of this stage? ***

Back 17

- equilibrium reactions (cortical level)

- cortical level balance and coordinated movement
- requires balanced cocontraction of antagonistic muscles – flexors vs extensors
- adaptive response to change in COG
- begins at 6 months - tilt, postural fixation.

Front 18

Summary of normal neuromotor development. ***

Back 18

- primitive and tonic reflex (SC & BS) > righting and equilibrium (midbrain & cortex)
- cephalo-caudal and proximal to distal progression
- large muscle groups develop before small, fine control muscles
- asymmetry > symmetry > controlled asymmetry
- progression of extension in prone > flexion in supine > lateral flexion in sidelying

- mobility > stability > controlled mobility > skilled movement
--- mobility: spontaneous random, limited control
--- stability: ability to hold posture without support
--- controlled mobility: ability to maintain posture while changing position
--- skilled movement: distal control on proximal stability – walk, eat, speak, self care, hand manipulation

- weightbearing needed for weight shift for transitional movement
- while generally predictable, each child is different

Front 19

Bear position ***

Back 19

hands and feet (bear crawl)

Front 20

Righting ***

Back 20

- "Get you up and turn you around”
- to keep head up and align body

Front 21

Equilibrium ***

Back 21

higher level ability to stay up against gravity – balance

Front 22

Tone ***

Back 22

resting muscle readiness for movement; preloading

Front 23

Primitive reflexes ***

Back 23

- reflex movements that dominate movement in early life, but may limit control later
(e.g., STNR, ATNR, Moro)

- chart on page 31!!!!

Front 24

Spasticity ***

Back 24

hyperactive stretch reflex, excessive response to movement

Front 25

Sensorimotor ***

Back 25

- interdependent relationship between sensory and motor systems:
1. sensory input affects voluntary and involuntary motor control
2. movement causes increased sensory input

Front 26

Associated reaction ***

Back 26

- voluntary responses of the involved limb due to action in another body part

(e.g., resist hip flexion on left, right hip will want to react; Soques’/Ramistes’ phenomenon—p. 290)

Front 27

Reflex integration ***

Back 27

older term referring to change from use of primitive reflex to higher cortical level of control

Front 28

Crawl ***

Back 28

using arms and legs to move with abdomen on floor

(NOT what we traditionally think of as "crawling")

Front 29

Creep ***

Back 29

moving on hands and knees

(what we typically think of as "crawling")

Front 30

Cruise ***

Back 30

walking while holding on to furniture

Front 31

Spastic movement ***

Back 31

- hyperactive stretch reflex
- persistent primitive reactions
- stereotypical movement
- hypertonus

Front 32

Athetoid movement ***

Back 32

- writhing rotational movements

- fluctuating tone

Front 33

Ataxic movement ***

Back 33

- limited trunk stability

- difficulty in controlling small midrange movement

Front 34

Hypotonic movement ***

Back 34

low muscle tone regardless of position or movement

Front 35

Motor development:
neck righting ***

Back 35

(body on head)

0-6 months

Front 36

Motor development:
head righting ***

Back 36

- labyrinthine righting: 6 months onward

- optical righting: 8 months onward

Front 37

Motor development:
Landau reflex ***

Back 37

- normal response of infants when held in a horizontal prone position to maintain a convex arc with the head raised and the legs slightly flexed

- 6 months to 2.5 years

(the reflex is poor in those with floppy infant syndrome and exaggerated in hypertonic and opisthotonic infants)

Front 38

Motor development:
protective extension ***

Back 38

- extending limbs to protect from falling (e.g., to protect oneself from falling forward while sitting; essential for independent sitting)

- 6 months onward

Front 39

Motor development:
body-on-body righting ***

Back 39

- 6-12 months onward

Front 40

Motor development:
period of astasia ***

Back 40

- inability to stand due to a limitation or absence of muscular coordination

- may appear at 2 months and disappear around 10 months

Front 41

Motor development (reflexes):
rooting ***

Back 41

- birth to 3-4 months awake

- to 7 months while sleeping

Front 42

Motor development (reflexes):
sucking ***

Back 42

- birth to 3-4 months

Front 43

Motor development (reflexes):
galant ***

Back 43

- a newborn reflex elicited by holding the newborn in ventral suspension (face down) and stroking along the one side of the spine--the normal reaction is for the newborn to laterally flex toward the stimulated side

- birth to 2 months

Front 44

Motor development (reflexes):
crossed extension ***

Back 44

-

- birth to 1-2 months

Front 45

Motor development (reflexes):
flexor withdrawal ***

Back 45

-

- birth to 6-8 weeks

Front 46

Motor development (reflexes):
Moro (startle) ***

Back 46

-

- birth to 4 months

Front 47

Motor development (reflexes):
automatic stepping ***

Back 47

-

- birth to several weeks

Front 48

Motor development (reflexes):
grasp ***

Back 48

-

- birth to 3-4 monts

Front 49

Motor development (reflexes):
asymmetrical tonic neck reflex ***

Back 49

-

- birth to 4-6 months

Front 50

Motor development (reflexes):
symmetrical tonic neck reflex ***

Back 50

-

- birth to 4-6 months

Front 51

Motor development (reflexes):
tonic labyrinthine ***

Back 51

-

- 4 months

Front 52

Motor development (reflexes):
positive support ***

Back 52

-

- from 3 months to 8 months

Front 53

Developmental problem signs:

birth to 3 months ***

Back 53

- limited random movement
- easy and frequent startle response
- poor head control

- stiffness
- use of head and neck hyperextension for movement
- feeding problems

- respiratory problems
- irritability

Front 54

Developmental problem signs:

4-8 months ***

Back 54

- hypo or hypertonia
- mass movement patterns

- limited variety of movement
- asymmetry/absent bilateral play – preference of one side

Front 55

Developmental problem signs:

9-12 months ***

Back 55

- limited trunk control
- limited variety of movement
- limited transitional movement

- poor protective response
- poor manual skills
- poor balance

- obligatory reflexes
- abnormal tone

Front 56

Cerebral palsy ***

Back 56

- brain injury from conception to 3 years of age that affects movement

- (a “junk” diagnosis for early brain damage)

Front 57

With what is cerebral palsy associated? ***

Back 57

- low birth weight

- low APGAR score

Front 58

Prenatal risk factors for cerebral palsy ***

(table 6-1, p. 125)

Back 58

- maternal infection - damage brain's motor centers
--- rubella
--- herpes simplex
--- toxoplasmosis
--- cytomegalovirus

- placental abnormalities (detachment, inflammation) - oxygen deprivation
- Rh incompatibility - kernicterus
- maternal diabetes - mother's metabolic deficits cause stunted growth and delayed tissue maturation
- toxemia - mother's BP so high, baby in danger of not receiving sufficient blood flow, thus oxygen
- brain maldevelopment
- genetic disorders
- exposure to teratogens

Front 59

Perinatal risk factors for cerebral palsy ***

(table 6-1, p. 125)

Back 59

- asphyxia
- prolonged or difficult labor
- breech birth
- prolapsed umbilical cord
- compression of brain
- rupture of blood vessels in brain

- rupture of blood vessels or compression of brain
- premature separation of placenta, placenta previa
- prematurity*
- low birth weight*

* the two biggest risk factors for CP

Front 60

Postnatal risk factors for cerebral palsy ***

(table 6-1, p. 125)

Back 60

- CVA
- intraventricular hemorrhage
(MVA, shaken baby, near-drowning, lead exposure)

- brain infections
--- meningitis*
--- encephalitis*
* account for 60% of acquired CP

- seizures
- head trauma
- near-drowning

Front 61

Age at onset and age at integration for:
suck-swallow reflex ***

(table 3-1, p. 31)

Back 61

- 28 weeks gestation

- 2-5 months

Front 62

Age at onset and age at integration for:
rooting reflex ***

(table 3-1, p. 31)

Back 62

- 28 weeks gestation

- 3 months

Front 63

Age at onset and age at integration for:
flexor withdrawal reflex ***

(table 3-1, p. 31)

Back 63

- 28 weeks gestation

- 1-2 months

Front 64

Age at onset and age at integration for:
crossed extension reflex ***

(table 3-1, p. 31)

Back 64

- 28 weeks gestation

- 1-2 months

Front 65

Age at onset and age at integration for:
Moro reflex ***

(table 3-1, p. 31)

Back 65

- 28 weeks gestation

- 4-6 months

Front 66

Age at onset and age at integration for:
plantar grasp reflex ***

(table 3-1, p. 31)

Back 66

- 28 weeks gestation

- 9 months

Front 67

Age at onset and age at integration for:
positive support reflex ***

(table 3-1, p. 31)

Back 67

- 35 weeks gestation

- 1-2 months

Front 68

Age at onset and age at integration for:
asymmetric tonic neck reflex ***

(table 3-1, p. 31)

Back 68

- birth

- 4-6 months

Front 69

Age at onset and age at integration for:
palmar grasp reflex ***

(table 3-1, p. 31)

Back 69

- birth

- 9 months

Front 70

Age at onset and age at integration for:
symmetric tonic neck reflex ***

(table 3-1, p. 31)

Back 70

- 4-6 months

- 8-12 months

Front 71

Which primitive reflexes are present (onset) at 28 weeks gestation? ***

(table 3-1, p. 31)

Back 71

- suck-swallow
- rooting
- flexor withdrawal
- crossed extension
- moro
- plantar grasp

Front 72

Which primitive reflexes are present (onset) at 35 weeks gestation? ***

(table 3-1, p. 31)

Back 72

- positive support

Front 73

Which primitive reflexes are present (onset) at birth? ***

(table 3-1, p. 31)

Back 73

- asymmetric tonic neck

- palmar grasp

Front 74

Which primitive reflexes are present (onset) at 4-6 months? ***

(table 3-1, p. 31)

Back 74

symmetric tonic nec

Front 75

Which primitive reflexes are integrated at 1-2 months? ***

(table 3-1, p. 31)

Back 75

- flexor withdrawal
- crossed extension
- positive support

Front 76

Which primitive reflexes are integrated between 3-6 months? ***

(table 3-1, p. 31)

Back 76

- suck-swallow (2-5 months)
- rooting (3 months)

- moro (4-6 months)
- asymmetric tonic neck (4-6 months)

Front 77

Which primitive reflexes are integrated at 9-12 months? ***

(table 3-1, p. 31)

Back 77

- symmetric tonic neck (8-12 months)

- plantar grasp (9 months)
- palmar grasp (9 months)

Front 78

Functional limitations and treatment focus for:
increased muscle stiffness
in children with spasticity ***
(table 6-5, p. 132)

Back 78

- stereotypical movement patterns

- decrease stiffness, increase movement

Front 79

Functional limitations and treatment focus for:
slow, labored movement
in children with spasticity ***
(table 6-5, p. 132)

Back 79

- poor static and dynamic balance
- postural insecurity

- establish head and trunk righting and equilibrium reactions, extremity protective reactions

Front 80

Functional limitations and treatment focus for:
increased trunk rotation
in children with spasticity ***
(table 6-5, p. 132)

Back 80

- poor movement transitions

- practice movement transitions involving trunk rotation (i.e., rolling, coming to sit, and walking)

Front 81

Functional limitations and treatment focus for:
decreased ROM
in children with spasticity ***
(table 6-5, p. 132)

Back 81

- reaching, walking

- increase ease of movement in all ranges
- vary speed and excursion of goal-directed movements

Front 82

Functional limitations and treatment focus for:
skeletal malalignment
in children with spasticity ***
(table 6-5, p. 132)

Back 82

- scoliosis
- musculoskeletal deformities

- position properly for function
- use orthoses

Front 83

Functional limitations and treatment focus for:
muscle weakness
in children with spasticity ***
(table 6-5, p. 132)

Back 83

- movements against gravity

- strengthen through movement experiences

Front 84

Functional limitations and treatment focus for:
inaccurate muscle recruitment
in children with spasticity ***
(table 6-5, p. 132)

Back 84

- inefficient movement
- high energy cost

- use novel environments and encourage appropriate sequences of muscle activation

Front 85

Pathophysciology of cerebral palsy caused by:
intraventricular hemorrhage
(table 6-2, p. 129) ***

Back 85

- spastic diplegia

Front 86

Pathophysciology of cerebral palsy caused by:
hypoxic-ischemic injury
(table 6-2, p. 129) ***

Back 86

- spastic quadriplegia
- spastic hemiplegia

Front 87

Pathophysciology of cerebral palsy caused by:
selective neuronal necrosis of the cerebellum
(table 6-2, p. 129) ***

Back 87

ataxia

Front 88

Pathophysciology of cerebral palsy caused by:
status marmoratus (hypermyeliniation in basal ganglia)
(table 6-2, p. 129) ***

Back 88

athetosis

Front 89

Classification of seizures:
generalized seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 89

- generalized seizure

- seizures that are generalized to the entire body
- always involve a loss of consciousness

Front 90

Classification of seizures:
tonic-clonic seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 90

- grand mal seizure (older term)

- begin with a tonic contraction (stiffening) of the body, then change to clonic movements (jerking) of the body

Front 91

Classification of seizures:
tonic seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 91

stiffening of the entire body

Front 92

Classification of seizures:
clonic seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 92

- minor motor seizure (older term)

- myoclonic jerks start and stop abruptly

Front 93

Classification of seizures:
atonic seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 93

- drop attacks (older term)

- sudden lack of muscle tone

Front 94

Classification of seizures:
absence seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 94

- petit mal seizure (older term)

- nonconvulsive seizure with a loss of consciousness;
- blinking, staring, or minor movements lasting a few seconds

Front 95

Classification of seizures:
akinetic seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 95

- lack of movement, "freezing" in place

Front 96

Classification of seizures:
partial seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 96

- focal seizure (older term)

- seizures not generalized to the entire body
- a variety of sensory or motor symptoms may accompany this type of seizure

Front 97

Classification of seizures:
simple partial seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 97

- Jacksonian seizure (older term)

- no loss of consciousness or awareness

Front 98

Classification of seizures:
simple partial seizure with motor symptoms

Give older term and manifestations ***
(table 6-4, p. 131)

Back 98

- jerking may begin in one small part of the body and spread to other parts

- usually limited to one half of the body

Front 99

Classification of seizures:
simple partial seizure with sensory symptoms

Give older term and manifestations ***
(table 6-4, p. 131)

Back 99

- sensory aura may precede a motor seizure

Front 100

Classification of seizures:
complex partial seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 100

- psychomotor seizure (older term)
- temporal lobe seizure (older term)

- loss of consciousness occurs during the seizure at either the beginning or the end of the event
- may develop from a simple partial seizure or develop into a generalized seizure
- may include automatisms such as lip smacking, staring, or laughing

Front 101

Classification of seizures:
unclassified seizure

Give older term and manifestations ***
(table 6-4, p. 131)

Back 101

- seizures that do not fit into the above categories, including some neonatal and febrile seizures

Front 102

Types of generalized seizure ***

(table 6-4, p. 131)

Back 102

- tonic-clonic (grand mal)
- tonic
- clonic (minor motor)
- atonic (drop attacks)
- absence (petit mal)
- akinetic

Front 103

Types of partial seizure ***

(table 6-4, p. 131)

Back 103

- simple partial (Jacksonian)
--- with motor symptoms
--- with sensory symptoms

- complex partial (psychomotor, temporal lobe)

Front 104

What are the five characteristics that identify a theoretic approach as having a life span perspective? ***

Back 104

development is:
- contextual
- life-long
- embedded in history
- multidimensional
- plastic and flexible

Front 105

List the developmental time periods and the age ranges
(include breakdown of adulthood). ***
(table 4-1, p. 49)

Back 105

- infancy (birth to 2 years)

- childhood (2 years to 10/12 years

- adolescence (10/12 years to 18/20 years)
- early adulthood (18/20 years to 40 years)

- middle adulthood (40 years to 65 years)

- older adulthood (65 years to death)
--- young-old (65-74 years)
--- middle-old (75-84 years)
--- old-old (85 and older)

Front 106

Describe Piaget's stages of cognitive development:
infancy ***

(table 4-2, p. 50)

Back 106

- sensorimotor stage

- pairing of sensory and motor reflexes leads to purposeful activity

Front 107

Describe Piaget's stages of cognitive development:
preschool ***

(table 4-2, p. 50)

Back 107

- preoperational stage

- unidimensional awareness of environment

Front 108

Describe Piaget's stages of cognitive development:
school age ***

(table 4-2, p. 50)

Back 108

- concrete operational stage

- solves problems with real objects
- classification
- conservation

Front 109

Describe Piaget's stages of cognitive development:
pubescence ***

(table 4-2, p. 50)

Back 109

- formal operational stage

- solves abstract problems
- induction
- deduction

Front 110

Describe Erikson's stages of development:
infancy ***

(table 4-3, p. 51)

Back 110

- trust versus mistrust stage

characteristics of:
- self-trust
- attachment

Front 111

Describe Erikson's stages of development:
late infancy ***

(table 4-3, p. 51)

Back 111

- autonomy versus shame or doubt stage

characteristics of:
- independence
- self-control

Front 112

Describe Erikson's stages of development:
childhood (preschool) ***

(table 4-3, p. 51)

Back 112

- initiative versus guilt stage

characteristics of:
- initiation of own activity

Front 113

Describe Erikson's stages of development:
school age ***

(table 4-3, p. 51)

Back 113

- industry versus inferiority stage

characteristics of
- working on projects for recognition

Front 114

Describe Erikson's stages of development:
adolescence ***

(table 4-3, p. 51)

Back 114

- identity versus role confusion stage

characteristics of
- sense of self:
--- physically
--- socially
--- sexually

Front 115

Describe Erikson's stages of development:
early adulthood ***

(table 4-3, p. 51)

Back 115

- intimacy versus isolation stage

characteristics of:
- relationship with significant other

Front 116

Describe Erikson's stages of development:
middle adulthood ***

(table 4-3, p. 51)

Back 116

- generativity versus stagnation

characteristics of
- guiding the next generation

Front 117

Describe Erikson's stages of development:
late adulthood ***

(table 4-3, p. 51)

Back 117

- ego integrity versus despair stage

characteristics of
- sense of wholeness
- sense of vitality
- wisdom

Front 118

List the stages in Maslow's hierarchy. ***

Back 118

bottom to top:
- physiologic/survival needs (food, water, elimination)
- safety
- love, belongingness, affection
- esteem
- self-actualization

Front 119

Gross motor milestones:
- head control ***

(table 4-4, p. 56)

Back 119

4 months

Front 120

Gross motor milestones:
- rolling ***

(table 4-4, p. 56)

Back 120

6 to 8 months

Front 121

Gross motor milestones:
- sitting ***

(table 4-4, p. 56)

Back 121

8 months

Front 122

Gross motor milestones:
- creeping ***

(table 4-4, p. 56)

Back 122

9 months

Front 123

Gross motor milestones:
- cruising ***

(table 4-4, p. 56)

Back 123

10 months

Front 124

Gross motor milestones:
- walking ***

(table 4-4, p. 56)

Back 124

12 months

Front 125

Fine motor milestones:
- palmar grasp reflex ***

(table 4-5, p. 56)

Back 125

birth

Front 126

Fine motor milestones:
- raking ***

(table 4-5, p. 56)

Back 126

5 months

Front 127

Fine motor milestones:
- voluntary palmar grasp ***

(table 4-5, p. 56)

Back 127

6 months

Front 128

Fine motor milestones:
- radial palmar grasp ***

(table 4-5, p. 56)

Back 128

7 months

Front 129

Fine motor milestones:
- radial digital grasp ***

(table 4-5, p. 56)

Back 129

9 months

Front 130

Fine motor milestones:
- inferior pincer grasp ***

(table 4-5, p. 56)

Back 130

9-12 months

Front 131

Fine motor milestones:
- superior pincer grasp ***

(table 4-5, p. 56)

Back 131

12 months

Front 132

Fine motor milestones:
- three-jaw chuck ***

(table 4-5, p. 56)

Back 132

12 months

Front 133

Norms for important stages of development: ***
1-2 months

(table 4-6, p. 59)

Back 133

- internal body processes stabilize
- basic biological rhythms established
- spontaneous grasp and release are established

Front 134

Norms for important stages of development: ***
3-4 months

(table 4-6, p. 59)

Back 134

- forearm support develops
- head control is established
- midline orientation is present

Front 135

Norms for important stages of development: ***
4-5 months

(table 4-6, p. 59)

Back 135

-antigravity control of extensors and flexors begins
- bottom lifting is present

Front 136

Norms for important stages of development: ***
6 months

(table 4-6, p. 59)

Back 136

- strong extension-abduction of limbs is present
- complete trunk extension is present
- pivots on tummy

Front 137

Norms for important stages of development: ***
7-8 months

(table 4-6, p. 59)

Back 137

- spontaneous trunk rotation begins
- trunk control develops along with sitting balance

Front 138

Norms for important stages of development: ***
9-10 months

(table 4-6, p. 59)

Back 138

- movement progression is seen in crawling, creeping, pulling to stand, and cruising

Front 139

Norms for important stages of development: ***
11-12 months

(table 4-6, p. 59)

Back 139

- independent ambulation occurs

Front 140

Norms for important stages of development: ***
16-17 months

(table 4-6, p. 59)

Back 140

- carries or pulls an object while walking
- walks sideways and backward

Front 141

Norms for important stages of development: ***
20-22 months

(table 4-6, p. 59)

Back 141

- easily squats and recovers toy

Front 142

Norms for important stages of development: ***
24 months

(table 4-6, p. 59)

Back 142

- arm swing is present during ambulation

Front 143

What determines the functional abilities of children with spina bifida? ***

Back 143

the extent of the impairment is directly related to:
- the level of the cyst and
- level of the spinal cord defect

Front 144

Spina bifida occulta ***

(table 7-1, p. 156)

Back 144

- vertebral defect in which posterior elements of the vertebral arch fail to close;
- no sac
- vertebral defect usually not associated with an abnormality of the spinal cord

Front 145

Spina bifida cystica ***

(table 7-1, p. 156)

Back 145

vertebral defect with a protruding cyst of meninges or spinal cord and meninges

Front 146

Spina bifida aperta (meningocele or myelomeningocele) ***

Back 146

- meningocele with CSF and meninges

- myelomeningocele contains spinal cord in cyst

Front 147

Meningocele ***

(table 7-1, p. 156)

Back 147

- cyst containing CSF and meninges and usually covered with epithelium

- clinical symptoms variable

Front 148

Myelomeningocele ***

(table 7-1, p. 156)

Back 148

- cyst containing CSF, meninges, spinal cord, and possibly nerve roots
- cord incompletely formed or malformed
- most common in the lumbar area
- the higher the lesion, the more deficits present

Front 149

Causes of spina bifida ***

Back 149

many causal factors implicated
- genetic predisposition
- alcohol exposure/FAS
- Irish/Irish-American background
- lack of folic acid

Front 150

Functional ability related to level of SC lesion:
thoracic ***

(table 7-2, p. 158)

Back 150

muscle function:
- trunk weakness
- T7-T9 upper abdominals
- T9-T12 lower abdominals
- T12 has weak quadratus lumborum

potential deformity:
- positional deformities of hips, knees, and ankles secondary to frog-leg posture

Front 151

Functional ability related to level of SC lesion:
high lumbar (L1-L2) ***

(table 7-2, p. 158)

Back 151

muscle function:
- unopposed hip flexors and some adductors

potential deformity:
- hip flexion, adduction
- hip dislocation
- lumbar lordosis
- knee flexion and plantar flexion

Front 152

Functional ability related to level of SC lesion:
midlumbar (L3) ***

(table 7-2, p. 158)

Back 152

muscle function:
- strong hip flexors, adductors
- weak hip rotators
- antigravity knee extension

potential deformity:
- hip dislocation, subluxation
- genu recurvatum

Front 153

Functional ability related to level of SC lesion:
low lumbar (L4) ***

(table 7-2, p. 158)

Back 153

muscle function:
- strong quadriceps
- medial knee flexors against gravity
- ankle dorsiflexion and inversion

potential deformity:
- equinovarus (club foot)
- calcaneovarus or
- calcaneocavus foot

Front 154

Functional ability related to level of SC lesion:
low lumbar (L5) ***

(table 7-2, p. 158)

Back 154

muscle function:
- weak hip extension, abduction
- good knee flexion against gravity
- weak plantar flexion with eversion

potential deformity:
- equinovarus (club foot)
- calcaneovarus or
- calcaneocavus foot

Front 155

Functional ability related to level of SC lesion:
sacral (S1) ***

(table 7-2, p. 158)

Back 155

muscle function:
- good hip abductors
- weak plantar flexors

no potential deformities noted

Front 156

Functional ability related to level of SC lesion:
sacral (S2-S3) ***

(table 7-2, p. 158)

Back 156

muscle function:
- good hip extensors
- good ankle plantar flexors

no potential deformities noted

Front 157

What positions should be avoided in children with myelomeningocele? ***

(box 7-1, p. 163)

Back 157

- frog-leg position in prone or supine
- W sitting
- ring sitting
- heel sitting
- cross-legged sitting

Front 158

What are the vital components of a physical therapy program for patients with myelomeningocele? ***

(box 7-2; page 170)

Back 158

- proper positioning in sitting and sleeping
- stretching
- strengthening

- pressure relief and joint protection
- mobility for short and long distances
- transfers and activities of daily living
- self care

Front 159

What is Down Syndrome? ***

Back 159

- autosomal (non-sex chromosomal) disorder

- may be trisomy of 21 in all cells or mosaic (not in all cells)

Front 160

Who is more likely to have a baby with Down Syndrome? ***

Back 160

- women over 35

- mother or father may carry the abnormal gene; more likely the mother

Front 161

What are some of the areas affected by Down Syndrome? ***

Back 161

- cognition
- muscle tone
- heart and lungs

- head size/shape
- cervical spine
- eyes, nose, mouth
- hands
- hips
- toes

Front 162

How is the muscle tone affected in a child with Down Syndrome? ***

Back 162

global hypotonia

Front 163

How is the head affected in a child with Down Syndrome? ***

Back 163

- smaller head
- brachycephalic (wide/flat head)
- flattened occiput

Front 164

How are the eyes, nose and mouth affected in a child with Down Syndrome? ***

Back 164

- Brushfield's spots - grey flecks in infant iris
- epicanthic folds

- flattened bridge of nose
- open mouth with large, protruding tongue without central fissure

Front 165

How are the hands affected in a child with Down Syndrome? ***

Back 165

- short, broad hands
- simian crease
- curved 5th finger
- missing one of 5th phalanges

Front 166

How are the toes affected in a child with Down Syndrome? ***

Back 166

gap between the first two toes

Front 167

How are the hips affected in a child with Down Syndrome? ***

Back 167

- formed differently
- subject to dislocation

Front 168

How are the heart and lungs affected in a child with Down Syndrome? ***

Back 168

- congenital heart disease is common (40%)
- lung hypoplasia
- pulmonary hypertension

Front 169

How is the spine affected in a child with Down Syndrome? ***

Back 169

- unstable C2 (atlanto-axial joint)
- scoliosis

Front 170

What is arthrogryposis? ***

Back 170

- a.k.a., arthrogryposis multiplex congenita (AMC)
- rare congenital disorder characterized by multiple joint contractures
- can include muscle weakness and fibrosis
- non-progressive disease

- many known subgroups of AMC, with differing signs, symptoms, causes etc
- some cases, few joints may be affected, nearly full ROM
- most common type of arthrogryposis, called amyoplasia, hands, wrists, elbows, shoulders, hips, feet and knees are affected
- most severe types, nearly every joint is involved, including the jaw and back

Frequently, the contractures are accompanied by muscle weakness, which further limits movement. AMC is typically symmetrical and involves all four extremities with some variation seen.[1][5]

Front 171

What is cystic fibrosis? ***

Back 171

- a.k.a., CF or mucoviscidosis
- autosomal recessive genetic disorder
- affects most critically the lungs, and also the pancreas, liver, and intestine
- characterized by abnormal transport of chloride and sodium across an epithelium, leading to thick, viscous secretions

- difficulty breathing is the most serious symptom and results from frequent lung infections
- other symptoms, including sinus infections, poor growth, and infertility affect other parts of the body

- caused by a mutation in the gene for the protein cystic fibrosis transmembrane conductance regulator (CFTR)
- this protein is required to regulate the components of sweat, digestive juices, and mucu
- CFTR regulates the movement of chloride and sodium ions across epithelial membranes, such as the alveolar epithelia located in the lungs

- although most people without CF have two working copies of the CFTR gene, only one is needed to prevent cystic fibrosis due to the disorder's recessive nature
- CF develops when neither gene works normally (as a result of mutation) and therefore has autosomal recessive inheritance

Front 172

What is Fragile X? ***

Back 172

- a.k.a., FXS, Martin–Bell syndrome, or Escalante's syndrome
- genetic syndrome that is the most common known single-gene cause of autism
- most common inherited cause of mental retardation among boys

- results in a spectrum of intellectual disability ranging from mild to severe as well as
- physical characteristics such as
--- elongated face
--- large or protruding ears
--- large testes (macroorchidism)

-behavioral characteristics such as
--- stereotypic movements (e.g. hand-flapping), and
--- social anxiety

Front 173

Who is most often affected by Fragile X? ***

Back 173

boys

Front 174

What are some of the symptoms of Fragile X? ***

Back 174

- hypotonia
- poor coordination
- poor motor planning

- mitral valve prolapse
- seizures

- scoliosis
- prominent jaw
- hyperextensible finger joints

Front 175

What is Rett Syndrome? ***

Back 175

- a.k.a. cerebroatrophic hyperammonemia
- neurodevelopmental disorder of the grey matter of the brain
- almost exclusively affects females

- clinical features include
--- small hands and feet
--- deceleration of the rate of head growth (including microcephaly in some)
--- scoliosis, growth failure, and constipation are very common and can be problematic
--- repetitive stereotyped hand movements, such as wringing and/or repeatedly putting hands into the mouth, are also noted

- prone to gastrointestinal disorders
- up to 80% have seizures

- typically have no verbal skills
- about 50% of individuals affected are not ambulatory

- signs of this disorder are most easily confused with those of Angelman syndrome, cerebral palsy and autism

Front 176

Who is most typically affected by Rett Syndrome? ***

Back 176

- females

Front 177

What is usually the first sign of Rett Syndrome? ***

Back 177

- hand clapping
- hand wringing
- hand clenching

Front 178

How does Rett Syndrome affect neuromuscular function? ***

Back 178

- hypotonia early
- then hypertonia
- progressive loss of all neurologic function

Front 179

Some other effects of Rett Syndrome? ***

Back 179

- respiratory pattern deterioration
- seizures
- kyphosis, scoliosis
- contractures
- hip dysplasia
- equinovarus

Front 180

What are neuromuscular diseases? ***

Back 180

diseases of the
- motor neuron
- neuromuscular junction
- muscle

Front 181

List some neuromuscular diseases. ***

Back 181

- Duchenne/Becker
- other muscular dystrophies
- spinal muscular atrophy

Front 182

What is Duchenne Muscular Dystrophy? ***

Back 182

- recessive X-linked form of muscular dystrophy, affecting around 1 in 3,600 boys
- results in muscle degeneration and eventual death
- caused by a mutation in the dystrophin gene, located on the human X chromosome, which codes for the protein dystrophin, an important structural component within muscle tissue
- while both sexes can carry the mutation, females rarely exhibit signs of the disease

- symptoms usually appear in male children before age 5 but may be visible in early infancy
- progressive proximal muscle weakness of the legs and pelvis associated with a loss of muscle mass is observed first
- eventually this weakness spreads to the arms, neck, and other areas
- early signs may include pseudohypertrophy (enlargement of calf and deltoid muscles), low endurance, and difficulties in standing unaided or inability to ascend staircases
- as the condition progresses, muscle tissue experiences wasting and is eventually replaced by fat and fibrotic tissue (fibrosis)
- by age 10, braces may be required to aid in walking but most patients are wheelchair dependent by age 12
- later symptoms may include abnormal bone development that lead to skeletal deformities, including curvature of the spine
- due to progressive deterioration of muscle, loss of movement occurs, eventually leading to paralysis
- intellectual impairment may or may not be present but if present, does not progressively worsen as the child ages
- average life expectancy for patients afflicted with DMD is around 25

Front 183

What is Becker Muscular Dystrophy? ***

Back 183

- same genetics, but a milder form of MD
- similar to Duchenne Muscular Dystrophy, however, it is milder due to the fact that some dystrophin is available (vice none in DMD)

- diagnosed later
- pt has stronger, longer life

Front 184

What are some of the first signs of Duchenne's Muscular Dystrophy? ***

Back 184

- pseudohypertropy - usually in the calves and deltoids--muscle is initially edematous, rapidly becomes atrophic

- Gower sign (pt must use arms and hands to "walk up" own body to rise from squat to stand)

Front 185

How does Duchenne's Muscular Dystrophy progress? ***

Back 185

- progresses to severe weakness, then
- respiratory failure at late teens to early 20s
- multiple contractures as muscles fibrose

Front 186

Describe the postural abnormalities of a DMD patient. ***

Back 186

- shoulders and arms are held back awkwardly when walking
- lumbar lordosis
- protruding belly (weak abdominal muscles)

- weak gluteal muscles
- thin, weak thighs, especially anterior (quadriceps group)
- genu recurvatum
- thick "musculature" of lower leg (the "muscle" is mostly fat and not strong)
- tightness in Achilles tendon
- weak anterior tibialis causing foot drop
- toe walking


- poor balance
- awkward, clumsy walking

Front 187

How should patients with MD exercise? ***

Back 187

- submaximal resistive (without overexertion)
- stretching with great care to avoid injury, as normal resistance won't happen
- respiratory exercise, especially inspiration
- standing slows scoliosis
- progress to powered mobility with positioning

- bracing, assistive devices, technology
- e-stim may help

Front 188

What is SMA? ***

Back 188

- spinal muscular atrophy
- an incurable autosomal recessive disease caused by a genetic defect in the SMN1 gene which codes SMN, a protein necessary for survival of motor neurons
- results in death of neuronal cells in the anterior horn of spinal cord and subsequent system-wide muscle wasting (atrophy)

- manifests in various degrees of severity which all have in common general muscle wasting and mobility impairment
- other body systems may be affected as well, particularly in early-onset forms
- SMA is the most common genetic cause of infant death

Front 189

Symptoms of SMA ***

Back 189

- usually autosomal recessive
- 1/15,000-25,000 births

- anterior horn cells affected - fewer, then they deteriorate

Front 190

What are the three types of SMA we discussed? ***

Back 190

- Type I - Werdnig-Hoffman - onset 0-3 mo, death by one year
- Type II - Werdnig-Hoffman - onset 3 mo – 4yr, “shortened life span”
- Type III - Kugelberg-Weylander - onset 5-10 yr, slow progression, milder

Front 191

Treatment for SMA ***

Back 191

- trunk strengthening
- stretching – gentle, slow
- respiratory exercises, postural drainage

- standers for hip development (if not standing by 16-18 mos)
- gait trainers for supported walking as long as possible to maintain bone
- bracing
- wheelchair (power chair when needed) with positioning

Front 192

What is plagiocephaly? ***

Back 192

- abnormal shaping of the head
- condition characterized by an asymmetrical distortion (flattening of one side) of the skull

- describes a diagonal asymmetry across the head shape, particularly a flattening which is to one side at the back of the head, often with some facial asymmetry

(brachycephaly describes a very wide head shape with a flattening across the whole back of the head--see "Down Syndrome")

Front 193

What is torticollis? ***

Back 193

- a.k.a. "wry neck"
- tightening of the sternocleidomastoid and associated muscles
(thus neck is flexed, head is tilted to affected side, and rotated to opposite side)

- muscles affected are principally those supplied by the spinal accessory nerve.

Front 194

What are some of the suspected causes of plagiocephaly/torticollis? ***

Back 194

- high birth weight
- multiple births
- "back to sleep" program
- some evidence for genetic connection

Front 195

What must be ruled out for plagiocephaly/torticollis? ***

Back 195

bony neck deformity

Front 196

What condition may mimic torticollis? ***

Back 196

problems with one eye or one ear

Front 197

What is the treatment for torticollis? ***

Back 197

- stretch short side neck and trunk and
- strengthen long side, in rocking and play

- teach positioning for stretch
- spend play time in prone

- often developmental delays present due to torticollis
- if delays present, Standard of Care in P.T. is to work with child until able to pull to stand and cruise independently

- PT is usually effective
- TOT collar may help
- if not better in 3 months, further medical testing needed
- BOTOX, muscle lengthening, spine surgery or other approach may be needed

Front 198

What may be required treatment for plagiocephaly? ***

Back 198

if changes in positioning/handling do not work:
- helmet or
- surgical intervention

Front 199

What is craniosynostosis? ***

Back 199

- condition in which one or more of the fibrous sutures in an infant skull prematurely fuses by ossification, changing the growth pattern of the skull
- because the skull cannot expand perpendicular to the fused suture, it compensates by growing more in the direction parallel to the closed sutures

- sometimes the resulting growth pattern provides the necessary space for the growing brain, but results in an abnormal head shape and abnormal facial features
- in cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased intracranial pressure leading possibly to visual impairment, sleeping impairment, eating difficulties, or an impairment of mental development combined with a significant reduction in IQ

Front 200

What is ADHD and what is its prevalence? ***

Back 200

- attention deficit-hyperactivity disorder

- psychiatric and neurobehavioral disorder characterized by either significant difficulties of inattention or hyperactivity and impulsiveness or a combination of the two

- one of the most common psychiatric disorders in children
- 9-10% of all children
- diagnosed two to four times more frequently in boys

Front 201

What are the grounds for an ADHD diagnosis? ***

Back 201

at least:
- six of nine symptoms of inattention and/or
- six of nine symptoms of hyperactivity/impulsivity
have persisted for at least six months to a degree that is more frequent and severe than one's peers

- some symptoms were present before seven years of age
- impairment from the symptoms is present in two or more settings (e.g., at school or work and at home)

- significant impairment in functioning at school, work, or social settings
- clear evidence of significant impairment in school, work, or social functioning

Front 202

What are the 9 symptoms of inattention? ***

Back 202

- often fails to give close attention to details or makes careless mistakes in schoolwork or other activities
- often does not follow through on instructions and fails to finish schoolwork, chores, or duties in the workplace not due to failure to understand instructions

- often has difficulty organizing tasks and activities
- often avoids, dislikes, or is reluctant to engage in tasks that require sustained mental effort (such as schoolwork or homework)
- often loses things necessary for tasks or activities (e.g., toys, school assignments, pencils, books, or tools)

- often has difficulty sustaining attention in tasks or play activities
- often does not seem to listen when spoken to directly

- is often easily distracted by extraneous stimuli
- is often forgetful in daily activities

Front 203

What are the 9 symptoms of hyperactivity/impulsivity? ***

Back 203

hyperactivity:
- often fidgets with hands or feet or squirms in seat
- often leaves seat in classroom or in other situations in which remaining seated is expected
- often runs about or climbs excessively in situations in which it is inappropriate (in adolescents or adults, may be limited to subjective feelings of restlessness)
- often has difficulty playing or engaging in leisure activities quietly
Is often "on the go" or often acts as if "driven by a motor"
- often talks excessively

impulsivity:
- often blurts out answers before questions have been completed
- often has difficulty awaiting turn
- often interrupts or intrudes on others (e.g., butts into conversations or games)

Front 204

What is sensory processing disorder? ***

Back 204

- a neurological disorder causing difficulties with taking in, processing, and responding to sensory information about the environment and from within the own body (visual, auditory, tactile, olfaction, gustatory, vestibular, and proprioception)

- sensory information may be sensed and perceived in a way that is different from most other people
- result can be unusual ways of responding or behaving, finding things harder to do

- term SPD is now often used (though not without controversy) instead of the earlier term sensory integration dysfunction which was originally used by occupational therapist A. Jean Ayres as part of her theory that deficits in the processing and interpretation of sensation from the body and the environment could lead to sensorimotor and learning problems in children

- some state sensory processing disorder is a distinct diagnosis, while others argue that differences in sensory responsiveness are features of other diagnoses
- SPD is not recognized in any standard medical manuals such as the ICD-10[3] or the DSM-IV-TR

- SPD is often associated with a range of neurological, psychiatric, behavioral and language disorders
- no known cure; however, there are many treatments available

Front 205

How does SPD present? ***

Back 205

- may be only ONE sensory dysfunction (only sight, or only sound)
- may be multiple sensory dysfunction

- may be under-responder
- may be over-responder

- may be combination of over-/under-responder

Front 206

“The exact cause of Sensory Processing Disorder–like the causes of ADHD and so many other neurodevelopmental disorders–has not yet been identified. However, preliminary studies and research suggest some leading contenders.”- from Sensational Kids: Hope and Help for Children With Sensory Processing Disorder by Lucy Jane Miller, PhD, OTR

What are some of the leading contenders? ***

Back 206

- genetics (parent has ADHD)
- pre-natal exposure to stimulants (cocaine)

- birth complications (O2 deprivation not severe enough to cause CP)
- environmental (cultural, parenting, super-stimulating environment)

Front 207

What are some of the areas impacted by SPD? ***

Back 207

- physical
- social
- emotional
- psychological

Front 208

Treatment for SPD ***

Back 208

- sensory regulation
- integrated listening therapy (meditative, calming music)

- proprioceptive/kinesthetic
- vestibular

- balance activities
- coordination activities

Front 209

What is ASD? ***

Back 209

- autism spectrum disorder

- range of conditions classified as pervasive developmental disorders in the Diagnostic and Statistical Manual of Mental Disorders

- includes autism, Asperger syndrome, pervasive developmental disorder not otherwise specified (PDD-NOS), childhood disintegrative disorder, and Rett syndrome, although usually only the first three conditions are considered part of the autism spectrum

- typically characterized by social deficits, communication difficulties, stereotyped or repetitive behaviors and interests, and in some cases, cognitive delays

- these diagnoses share some common features, individuals with these disorders are thought to be "on the spectrum" because of differences in severity across these domains

- characterized by delays or abnormal functioning before the age of three years in one or more of the following domains:
(1) social interaction;
(2) communication; and
(3) restricted, repetitive, and stereotyped patterns of behavior, interests, and activities

Front 210

Treatment for ASD ***

Back 210

- physical therapy now considered an important part of treatment (not just OT and speech)
- balance and coordination deficits
- gross motor and fine motor delays

- sensory processing issues need to be addressed
- must work in tandem with other therapies (ST, speech, etc.)

Front 211

Within what three domains does movement occur?

Back 211

- physical
- psychological
- social

Front 212

What factors can affect acquisition of motor skills?

Back 212

- surroundings in which a person develops (physical, psychological, or social)
- time
- life experiences
- life experiences of family, friends, and teachers
- cultural practices
- child-rearing practices

Front 213

What comprises the concept of motor development?

Back 213

any change in movement abilities that occur across the span of life, so changes in the way a person moves after childhood are also included

Front 214

According to Piaget, what occurs in the developmental time period of infancy?

Back 214

- sensorimotor stage
- establishment of trust with caregivers
- development of autonomy
- incorporation of sensory information into movement and exploration of environment

Front 215

According to Piaget, what occurs in the developmental time period of childhood?

Back 215

- fostering of initiative to plan and execute movement strategies and to solve daily problems
- awareness of surrounding environment, at least one dimension at a time
- use of symbols such as language
- uses objects to represent things that can be thought of but are not physically present (imaginative)

- preoperational thinking
- reasoning centered around self
- self-regulation learned with help of parents regarding appropriate play behavior and toileting
- establishment of self-image

age 3-5:
- sharing, taking turns, repeating plot to a story

school-age:
- concrete operations - furtherance of thinking abilities
- works industriously for recognition on school projects, etc.
- able to classify objects by characteristics (color, shape, texture, etc.)

Front 216

According to Piaget, what occurs in the developmental time period of adolescence?

Back 216

- identity formed
- values embraced
- knowledge of self, goals, and how to achieve them
- pursuit of career/vocation moves individual way from childhood egocentrism

formal operations
- abstract problems solved by inductive and deductive reasoning
- logical decision making

Front 217

According to Piaget, what occurs in the developmental time period of adulthood?

Back 217

- up to 5-year transition from late adolescence (17) to early adulthood (22)--early adulthood transition

- middle adulthood - begins at 40, with 5 year transition from early adulthood (35-40); ends with a 5-year transition into older adulthood (age 60-65)

- young-old - 65-74
- middle-old - 75-84
- old-old - 85 and older

Front 218

According to Piaget, what is preoperational thought?

Back 218

- any procedure for mentally acting on objects
- characterized by sparse and logically inadequate mental operations

- child learns to use and to represent objects by images, words, and drawings
- child is able to form stable concepts as well as mental reasoning and magical beliefs
- child still unable to perform operations; tasks that the child can do mentally rather than physically
- thinking is still egocentric
- child has difficulty taking the viewpoint of others

two substages:
- symbolic function (ages 2-7) - can think in images and symbols
--- language and pretend play
--- egocentric
--- animism

- intuitive though (ages 4-7) - begin use of primitive reasoning
--- why?
--- centration - focusing all attention on one characteristic
--- conservation - awareness that altering a substance's appearance does not change its basic properties (children do not have at this stage)
--- do not yet understand concrete logic
--- cannot mentally manipulate information

Front 219

According to Piaget, what is concrete operational thought?

Back 219

- ages 7-11
- characterized by appropriate use of logic
- can mentally reverse information
- can only solve problems with concrete objects, not abstract concepts or hypothetical tasks
- child develops an ability to think abstractly and to make rational judgments about concrete or observable phenomena, which in the past he needed to manipulate physically to understand

- seriation - ability to sort by characteristics of objects (color, size, shape, etc.)
- transitivity - recognizes relationships among objects in a serial order (e.g., places books in height order on a bookshelf)
- classification - ability to name and identify sets of objects by characteristics
- decentering - accounts for multiple aspects of a problem to solve it
- reversibility - objects can be changed, then returned to original state (e.g., inflate/deflate/inflate a balloon)
- conservation - understaning that quantity, length, or number of items is unrelated to arrangement or appearance of the objects or items
elimination of egocentrism - ability to view things from another's perspective (even if incorrectly)

Front 220

According to Piaget, what is formal operational thought?

Back 220

- adolescence into adulthood

- logical use of symbols related to abstract concepts
- hypothetical and deductive reasoning
- ability to think about abstract concepts

Front 221

According to Piaget, how does an infant learn to understand the world?

Back 221

- by associating sensory experiences with physical actions (schemas)

- sensorimotor stage of development

Front 222

Describe Maslow's hierarchy.

Back 222

- each higher level depends on mastering the one before

- the last level mastered is not forgotten or lost, but is built on by the next

Front 223

Regardless of the age of the child, the ______ ______must be taken into account when planning therapeutic intervention.

Back 223

cognitive level

Front 224

How did Erikson characterize the stages an individual goes through to establish personality?

Back 224

each stage representing a struggle between two opposing traits

Front 225

The one overriding concept about which all developmentalists agree is the concept that development is ______.

Back 225

sequential

(disagreement involves:
- composition of the sequence
- which specific skills are part of the sequence
- whether one skill in the sequence is a prerequisite for the next)

Front 226

Motor development is epigenetic, meaning:

Back 226

human beings grow and develop from a simple organism to a more complex one through progressive differentiation

Front 227

Motivation to move comes from....

Back 227

intellectual curiosity

Front 228

Movement is a way of _____ _____ over the environment.

Back 228

asserting control

Front 229

Learning to control the environment begins with learning to control....

Back 229

one's own body

Front 230

To interact within the environment, the child must be...

Back 230

oriented within space

Front 231

We learn spatial relationships by:

Back 231

- oreinting first to our own bodies, then
- using ourselves as a reference point to map our movement within the environment

Front 232

Visualizing movement can help improve _____ _____.

Back 232

motor performance

(motivation and concentration are also important)

Front 233

How does a child typically work on developing gross motor milestones?

Back 233

- works on several at a time; overlapping (e.g., perfecting rolling while learning sitting balance)

- some children bypass stages (e.g., going from creeping to cruising without crawling)

Front 234

What are the four directional concepts of development?

Back 234

- cephalic to caudal (head control before trunk control; UE before LE)
- proximal to distal (stable base needed before movement)

- mass to specific (dissociation; simple to complex)
- gross to fine

Front 235

What is dissociation?

Back 235

- the ability to separate movement in one body part from movement in another body part

- characteristic of mature movement

(e.g., turns head without turning trunk, rolls over segmentally instead of log rolling)

Front 236

What is reciprocal interweaving?

Back 236

- reappearance of certain patterns of movement at different times during development
- cyclic changes in motor control
- at each stage of development, abilities emerge, merge, regress, or are replaced

(e.g., scapular adduction - initially used by infant for upper trunk extension in prone; reappears when walking as high guard position of arms)

Front 237

What factors affect movement?

Back 237

- biomechanics of the situation
- muscle strength
- level of neuromuscular maturation and control

Front 238

Full-term babies are born with what type of tone? How does this progress and change?

Back 238

- physiologic flexion - limbs and trunk are naturally flexed
- gravity, body weight and early reflexes help overcome this

progresses to:
- antigravity extension - easiest to achieve early on because extensors are in lengthened position from physiologic flexion (flexors are shortened)

then:
- antigravity flexion (supine)
- lateral flexion
- rotation

Front 239

Developmental process is described by Bly as a trend from:

Back 239

- random movements of entire body, to
- asymmetric movements, to
- bilateral symmetric movements of the head and trunk against gravity in prone, supine, and side-lying positions, to

- alternating reciprocal movements of limbs, to
- unilateral symmetric movements of head and trunk that result in lateral bending, then finally
- bilateral diagonal movement (e.g., creeping)

Front 240

Head control

Back 240

- infant should have good head control by 4 months
- should be able to keep ear in line with acromion when pulled to sit

- when held upright and tilted vertically, head should tilt in opposite direction
- when prone, should be able to lift head against gravity past 45 degrees

- should be able to flex head when supine at 5 months

Front 241

Rolling

Back 241

- log roll at 4-6 months (head and trunk as one unit)
- segmental rolling at 6-8 months (separate upper and lower rotation)

- prone to supine precedes supine to prone because extensor control precedes flexor control

Front 242

Sitting

Back 242

- independent sitting by 8 months of age
(alone, no kyphosis, no hand support, can rotate head/trunk)
- change in functional orientation (also may see reciprocal interweaving)

Front 243

Creeping, crawling, and cruising

Back 243

(narrative and table 4-4 contradict each other on this one)

table 4-4
- creeping - 9 mos (reciprocal - opposite arm/leg)
- cruising - 10 mos

text
- cruising - 9 mos
- creeping - 9-10 mos (reciprocal - opposite arm/leg)

Front 244

Walking

Back 244

- 12-18 mos

- last major gross motor milestone
- wide BOS
- legs abducted and externally rotated
- lordotic lumbar spine
- UEs in high guard with scapular adduction

Front 245

What are the two most important gross motor milestones? Why? ***

Back 245

- head control and sitting

- because if you cannot control your head and trunk, control of extremities will be very difficult or impossible)

Front 246

Prehension

Back 246

act of grasping

Front 247

Kinetic chain of prehension

Back 247

- to grasp, one must reach

- reaching patterns depend upon shoulder position and influence the ability to grasp objects

Front 248

Hand regard

Back 248

- 2 months

- ATNR places hand in perfect position to be seen/regarded

Front 249

Reflexive and voluntary palmar grasp

Back 249

- involves just the fingers coming into the palm, thumb not involved

- reflexive (in response to stimulus--i.e., touch, esp on ulnar side of hand) present from birth

- voluntary develops by 6 mos

Front 250

Evolution of voluntary grasp

Back 250

- after palmar grasp becomes voluntary at 6 mos, it progressively changes

- radial palmar grasp at 7 mos as thumb able to adduct (radial side of hand & thumb; able to pick up small objects, e.g., 1'' cubes)

- radial digital grasp at 9 mos as thumbs begin to oppose; can hold objects with ends of fingers rather than having to bring them into palm

- inferior pincer grasp at 9-12 mos (thumb on lateral side of index finger)
- superior pincer grasp at 12 mos (thumb tip to index finger tip)
- three-jaw chuck may be seen at 12 mos (wrist extended and ulnarly deviated, object held between thumb and first two fingers)

Front 251

Release of grasp

Back 251

- transferring objects hand-to-hand at 5-6 mos because one hand can be stabilized by the other

- true, voluntary release seen at 7-9 mos and is usually assisted by the infant being externally stabilized

- mature control of release at 12 mos (e.g., dropping object into container); 15 mos pellet into bottle (finer control)

- accuracy of release continues refinement through childhood with ball throwing

Front 252

Cerebral palsy

Back 252

- disorder of posture and movement
- secondary to damage to immature brain before, during, or after birth

- static encephalopathy (permanent, but not progressive)

characterized by
- decreased functional abilities
- delayed motor development
- impaired muscle tone and movement patterns

- despite static nature of damage, clinical manifestations may appear to change as child ages, because while movement demands increase, child's motor abilities may not be able to change quickly enough to meet those demands

Front 253

Congenital CP vs. acquired CP

Back 253

- before/during birth = congenital CP

- after birth up to age 3 = acquired CP

Front 254

At which stage of development is brain damage/CP likely to be most severe?

Back 254

- the earlier the damage occurs, the more likely the symptoms will be moderate to severe
- damage early in gestation more likely to produce quadriplegia
- damage later may result in LE diplegia

- first trimester especially, the infant's nervous system is extremely vulnerable

Front 255

Risk of CP increases with increasing ______ and ____.

Back 255

- prematurity

- low birth weight

Front 256

Any condition that causes ______, ______, or _____ to the brain can cause CP.

Back 256

- anoxia
- hemorrhage
- damage

Front 257

Kernicterus

Back 257

result of Rh incompatibility, causes:
- CP
- high-frequency hearing loss
- visual problems
- discoloration of teeth

Front 258

teratogen

Back 258

any agent or condition that causes a defect in the fetus
(e.g., radiation, drugs, infection, chronic illness)

Front 259

Quadriplegic CP

Back 259

- bilateral brain damage
- involvement of entire body
- UEs usually more severely affected

- difficulty developing head and trunk control
- may or may not be able to ambulate; if they do, probably not until middle childhood

Front 260

Diplegic CP

Back 260

- bilateral brain damage
- primarily LE involvement
- trunk is almost always affected as well
- often related to premature birth, especially at around 32 weeks/2 months premature

* some definitions of diplegia state all four limbs are involved with the LEs more severely involved than UEs

Front 261

"The CP of prematurity"

Back 261

spastic diplegia

Front 262

Four types/distributions of involvement in spastic CP

Back 262

- spastic quadriplegia with dominant extension
- spastic quadriplegia with dominant flexion
- spastic diplegia
- right/left spastic hemiplegia

Front 263

Tone in child with CP

Back 263

- may remain low
- may increase to normal
- may increase beyond normal to hypertonia
- may fluctuate from high to low to normal (characteristically seen in dyskinetic or athetoid type of CP)

Front 264

Abnormal tone in CP
- possible causes
- variances

Back 264

- may be CNS response to the brain damage, rather than the result of the damage itself

- may vary with position and gravity (display extension while lying supine, flexion when sitting)
- may vary in different parts of body (spastic diplegia with hypertonic LE muscles and hypotonic trunk muscles)

Front 265

How is the degree of abnormal tone assessed?

Back 265

relative to the degree of resistance encountered with passive movement

(usually, the greater the resistance to passive movement, the greater difficulty the child has in attempting to move)

Front 266

What is the most common type of abnormal tone seen in children with CP?

Back 266

- spasticity - velocity-dependent increase in muscle tone
- hypertonus - increased resistance to passive motion that may not be affected by speed of movement

- these terms are often used interchangeably

Front 267

Describe the abilities of a child with CP who has mild to moderate spasticity. Severe spasticity.

Back 267

- ability to move actively through at least part of the available ROM

- extreme difficulty in moving, with inability to complete full ROM
- may have difficulty even initiating movement without an inhibitory technique

Front 268

Prolonged increased tone predisposes the individual to...

Back 268

contractures and deformities because in most cases the antagonist cannot adequately oppose the spastic muscle

Front 269

Which muscles are typically most affected by hypertonus?

Back 269

- antigravity muscles

- UE flexors (scapular retractors/adductors, elbow, forearm, wrist, and finger flexors)

- LE flexors and extensors (hip flexors, hip adductors, knee flexors--esp. medial hamstrings, ankle plantar flexors)

Front 270

Continual low tone in an infant impedes development of what two important actions?

Back 270

- head and trunk control

- mature breathing patterns

Front 271

How may increased trunk tone affect breathing?

Back 271

- it may impair breath control and speech by
- hampering normal excursion of diaphragm and chest wall

Front 272

Spasticity in CP is of what origin?

Spastic paralysis is of what origin?

Back 272

- cerebral

- UMN lesion

Front 273

Rigidity in CP

Back 273

- uncommon

- indicates severe damage to deeper areas of brain, rather than to cortex

Front 274

Dyskinesia

Back 274

- disordered movement

Front 275

Athetosis

Back 275

- most common dyskinetic syndrome
- decreased static and dynamic postural stability

- disordered movement of extremities, especially within midranges
(movement in midrange is especially difficult due to lack of postural stability on which to superimpose the movement--limb goes further away from body, motor control diminishes)

- involuntary movements result from attempts to control posture and movement and can be seen in child's entire extremity, distally in hands/feet, or proximally in mouth and face (feeding and speech difficulties)

- child needs external support to improve movement accuracy and efficiency
- lack postural stability to allow purposeful movements to be controlled for completion of functional tasks

- muscle tone often fluctuates from low to high to normal to high so child has difficulty maintaining postural alignment in all but most firmly supported positions and exhibits slow, repetitive involuntary movements

Front 276

Ataxia

Back 276

- loss of coordination due to cerebellar damage
- demonstrates loss of coordination and low postural tone
- often diplegic distribution, with trunk and LEs most affected

- difficult for child to maintain midline stability of head and trunk in any posture
- movement is jerky and irregular
- may achieve upright standing, but need wide BOS

- postural reactions slow to develop, most notable balance deficiency during gait
- walk with large lateral displacements to maintain balance (almost staggering)
- lack of stability and poor timing of postural corrections
- with practice they can adjust to wide displacements of COG and walk without falling
- arm movements frequently compensate for excessive weight shifting of trunk

- hard for clinician to allow child to ambulate independently using what looks like precarious gait
- may need helmet, ADs do not seem to help unless weighted, and even then may still be a deterrent

Front 277

Pathophysiology of spastic quadriplegia

Back 277

- most often associated with grade III intraventricular hemorrhage in immature infants

Front 278

The most common cause of spastic diplegia is:

Back 278

- periventricular leukomalacia (PVL)

- because the fibers of the corticospinal tract that go to the LEs are most exposed

Front 279

Which is the most common type of CP? From what can it result?

Back 279

- spastic hemiplegia

- can result from unilateral brain damage secondary to an intraventricular hemorrhage or other hypoxic event

- selective neuronal necrosis can result from hypoxic-ischemic injury in the mature neonate

Front 280

Premature infants born at or before 32 weeks gestation are especially vulnerable to _____ _____ damage around the ventricles due to hypoxia and ischemia.

Back 280

white matter

Front 281

Spastic diplegia, quadriplegia and hemiplegia can be caused by varying degrees of which injuries? What determines how the neurologic deficit manifests?

Back 281

- intraventricular hemorrhage
and
- hypoxic-ischemic injury

determined by:
- which fibers of corticospinal tract are involved
- whether damage is bilateral or unitlateral

Front 282

Athetosis

Back 282

- involves damage to the basal ganglia, and has been associated with hemolytic disease of the newborn (HDN)/erythroblastosis fetalis, anoxia, and respiratory distress

Front 283

Ataxia

Back 283

related to damage to the cerebellum

Front 284

Deficits associated with CP, in the order in which they may become apparent in the infant: (Box 6-1, p. 130) ***

Back 284

earliest signs of motor dysfuction often manifest in problems with feeding and breathing

- feeding and speech impairments
- breathing inefficiency
- visual impairments
- hearing impairments
- mental retardation
- seizures

Front 285

Feeding/speech difficulties in CP

Back 285

- poor suck-swallow reflex
- uncoordinated sucking and breathing
- persistence of infantile oral reflexes (rooting or suck-swallow)
- exaggerations of normal reflexes such as tonic bite or tongue thrust

- hyposensitivity (low tone or atonic CP) or hypersensitivity (spastic hemiplegia or quadriplegia) response to touch in and around the mouth

- lip closure needed for eating and for making "p," "b" and "m" sounds
- proper tongue movement needed for sucking, swallowing, and later chewing; also for producing various sounds

Front 286

Breathing difficulties in CP

Back 286

- developing infants are typically belly breathers
- later develop ability to use rib cage to increase volume of inspired air

- gravity promotes developmental changes in configuration of rib cage that place diaphragm in more advantageous position for effective inspiration

- if child is delayed in developing upright posture due to lack of head/trunk control, this developmental change is delayed; instead may develop:
- structural deformity of ribs (flaring)
- poor breath control
- shorter breath length that is inadequate for sound production
- abnormally increased tone in trunk may allow only short bursts of air to be expelled, resulting in staccato speech

Front 287

MR and CP

Back 287

- children with CP have many other problems related to CNS damage (vision and hearing impairments, speech and feeding difficulties, etc.)

- often also have MR (38-92%)
- no direct correlation between severity of motor involvement and degree of MR

- generally, children with spastic hemiplegia or diplegia, athetosis or ataxia have normal or above average intelligence
- children with spastic quadriplegia, rigidity, atonia or mixed types of CP are more likely to have MR
- even children of normal intelligence who have CP may be at increased risk of cognitive delays or learning disabilities

Front 288

Relationship of severity of CP and IQ (Table 6-3, p. 130)

Back 288

- mild CP (independent ambulation) & IQ > 70 (functionally independent)
- moderate CP (supported ambulation) & IQ 50-70 (needs assistance)
- severe CP (nonambulatory) & IQ < 50 (dependent)

Front 289

Site of brain damage in CP may become the focal point of:

Back 289

abnormal electrical activity, which can cause seizures

Front 290

Approximately ___ percent of children with CP experience seizures that must be managed by medication.

Back 290

50

(a smaller percentage have a single seizure episode related to high fever or increased intracranial pressure)

Front 291

Children with CP or MR are more likely to develop _____ than typically developing children.

Back 291

seizures

Front 292

Seizures are classified as _____, _____, or ______.

Back 292

- generalized
- partial
- unclassified

Front 293

Generalized seizures are named for:

Back 293

the type of motor activity the person exhibits (tonic, clonic, atonic, absence)

Front 294

Partial seizures can be ____ or ____, depending on whether the child experiences a ____ ___ _________.

Back 294

- simple or complex

- loss of consciousness (complex)

Front 295

Partial seizures can have either ____ or _____ manifestations, or both.

Back 295

- sensory

- motor

Front 296

Unclassified seizures are:

Back 296

those that do not fit into the other categories (generalized, simple/complex partial)

Front 297

Which children with CP are more likely to demonstrate seizures?

Back 297

those with CP caused by CNS infections, CNS malformations, or gray matter damage are more likely to demonstrate seizures than children whose CP is caused by white matter damage or unknown events.

Front 298

How does age of onset of CP correlate to the type of CP?

Back 298

patients with quadriplegia tend to demonstrate earlier onset than those with hemiplegia

Front 299

Why is vision important to motor development in the first three years of life?

Back 299

- it is critical for the development of balance in that time frame
- difficulties may exacerbate neuromotor problems that typically accompany CP

Front 300

How are eye muscles affected by abnormal tone?

Back 300

- turning in (exotropia)
- turning out (exotropia)
of one or both eyes
- eye crossing (strabismus)

Front 301

How prevalent is strabismus in children with CP? In which CP population is it highest?

Back 301

- it is present in 50% of patients

- it is highest in children with quadriplegia and diplegia

Front 302

What is nystagmus? In which CP patients is it most common? What complications can it cause?

Back 302

- occurs when the eyes move back and forth rapidly in a horizontal, vertical, or circular direction

- most common in children with ataxia

- may complicate the task of balancing the head or trunk

Front 303

How do some children with CP compensate for nystagmus? For what may this be mistaken?

Back 303

- by tilting the head/neck into extension

- the tilting of the head/neck may be mistaken for neck retraction and/or abnormal extensor tone

- usually neck retraction is to be avoided, but if done in compensation for nystagmus to give the most stable visual input, it may not be avoidable

Front 304

What vision deficit is common in children with hemiplegic CP?

Back 304

- homonymous hemianopia (loss of vision in half the visual field)

Front 305

Why should every child with hemiplegic CP have a detailed vision assessment?

Back 305

- because homonymous hemianopia is common in this population

- visual impairments often make head and/or trunk control more difficult

Front 306

What types of actions should tip the clinician to a need for a vision assessment?

Back 306

- hemiplegic CP (homonymous hemianopia common in this population)
- difficulty developing head/trunk control
- difficulty exploring immediate surroundings
- difficulty in reaching for objects
- not following familiar face
- not turning to examine a new face

Front 307

Approximately what percentage of children with CP have hearing, speech and language problems?

Back 307

1/3

Front 308

What can cause hearing, speech, and language problems in children with CP?

Back 308

- poor motor control of oral muscles
- respiratory impairment

- expressive (Broca's) aphasia or
- receptive (Wernicke's) aphasia
when initial damage that caused the CP affects those areas

Front 309

Impairments and functional limitations of children with spastic CP

Back 309

impairments
- too much muscle tone
- too little ROM

functional limitations
- poor head/trunk control
- poor performance of/difficulty in transitions
- poor execution of ambulation
- difficulties in use of extremities for balance and reaching

Front 310

Impairments and functional limitations of children with athetoid or ataxic CP

Back 310

impairments
- too much mobility
- too little stability

functional limitations
- poor head/trunk control
- poor performance of/difficulty in transitions
- poor execution of ambulation
- difficulties in use of extremities for balance and reaching

Front 311

Impairments and functional limitations of children with hypotonic CP

Back 311

similar to those of children with Down syndrome (q.v.)

Front 312

Why do children with spastic CP have difficulty developing head control? How do they attempt to overcome this?

Back 312

- increased tone
- persistent primitive reflexes
- exaggerated tonic reflexes
- absent or impaired sensory input

- substitutions and/or compensatory movements

Front 313

Why do children with spastic CP have difficulty developing trunk control? How do they attempt to overcome this?

Back 313

- extensor or flexor tone in trunk; lack of balance between flexors and extensors
- causes lack of lateral flexion and/or rotation
- lack of trunk rotation makes transitional movements difficult or impossible

- lack of hip extension
- round the upper back to compensate for lack of hip extension
- may push body backwards over flexed and adducted legs to W-sit
(avoid W sitting, as it can impede further development of trunk control and LE dissociation)

Front 314

Influence of tonic reflexes on functional movement:
TLR in supine
(impairment and functional limitation)

Back 314

impairment
- contractures
- abnormal vestibular input
- limited visual field

functional limitations
- rolling from supine to prone
- reaching in supine
- coming to sit
- sitting

Front 315

Influence of tonic reflexes on functional movement:
TLR in prone
(impairment and functional limitation)

Back 315

impairment
- contractures
- abnormal vestibular input
- limited visual field

functional limitation
- rolling from prone to supine
- coming to sit
- sitting

Front 316

Influence of tonic reflexes on functional movement:
ATNR
(impairment and functional limitation)

Back 316

impairments
- contractures
- hip dislocation
- trunk asymmetry
- scoliosis

functional limitation
- segmental rolling
- reaching
- bringing hand to mouth
- sitting

Front 317

Influence of tonic reflexes on functional movement:
STNR
(impairment and functional limitation)

Back 317

impairment
- contractures
- lack of UE and LE (dissociation)
- lack of trunk rotation

functional limitation
- creeping
- kneeling
- walking

Front 318

What is an obligatory reflex?

Back 318

dominates the posture

Front 319

How do obligatory tonic reflexes affect the movement of children with spastic CP?

Back 319

- produce increased tone and postures that can interfere with adaptive movement

- they do not interfere with an infant's ability to move, but the retention of those reflexes and their exaggerated expression seem to impair acquisition of postural responses (e.g., head/neck righting and use of extremities for protective extension

(retention of these tonic reflexes occurs because of the lack of normal development of motor control associated with CP)

Front 320

List the tonic reflexes

Back 320

- tonic labyrinthine reflex (TLR)
- asymmetric tonic neck reflex (ATNR)
- symmetric tonic neck reflex (STNR)

Front 321

How does TLR affect the child with spastic CP?

Back 321

- TLR affects tone relative to head's relationship with gravity
- when supine, induces an extensor tone
- when prone, induces a flexor tone

- present at birth, integrated by 6 months
- thought to assist unfolding of the physiologic flexor tone present at birth

- if TLR persists, can affect ability to develop antigravity motion (flex while supine, extend while prone
- can prevent child from reaching with arms in supine
- can prevent child from pushing with arms in prone to assist coming to sit

can affect posture in sitting
- if child loses head control posteriorly, labyrinths sense body as supine and extensor tone is produced, potentially causing child to fall backwards or fall out of a chair
- if child loses head control anteriorly, labyrinths sense body as prone and flexor tone is produced

Front 322

How does ATNR affect the child with spastic CP?

Back 322

causes UE
- extension on face side and
- flexion on skull side
(archer pose)

- usually only present in UEs of typically developing child from birth to 4-6 months
- in child with CP, may also affect LEs, persist, and become obligatory

if ATNR persists and becomes obligatory in child with spastic CP:
- child will not be able to roll over or bring extended arm/hand to mouth
- assymetry can affect the trunk, predisposing to scoliosis
- hip dislocation on flexed side in extreme cases

Front 323

How does STNR affect the child with spastic CP?

Back 323

- causes arms and legs to flex or extend, depending on head position
--- flex head, flex arms, extend legs
--- extend head, extend arms, flex legs
(arms follow head, legs do the opposite)

- usually only present in typically developing infant to attaining 4-point stance (4-6 months to 8-12 months)

if STNR persists and becomes obligatory in child with spastic CP:
- reciprocal creeping prevented; child must "bunny hop"
- child either sits on the heels or thrusts forward, depending on head position
- 4-point position and any dissociation of extremities needed for creeping are difficult

Front 324

What difficulties does a child with spastic CP encounter in making movement transitions?

Back 324

- lacks the ability to control or to respond appropriately to shifts in COG that should result in righting, equilibrium or protective reactions

- child is fearful and doesn't feel safe due to precarious static and dynamic balance
- awareness of this poor postural stability may lead to expectation of falling

- inability to generate sufficient muscle activity for static balance is further compounded by difficulty in anticipating postural changes in response to body movement

Front 325

What difficulties does a child with spastic CP encounter in mobility and ambulation?

Back 325

- impaired LE separation hinders reciprocal leg movement for creeping/walking

- some children learn to move by "bunny hop" or "commando crawl" (dragging stiff legs)
--- this increases LE tone in extensor muscle groups and
--- may interfere when child tries to pull to stand and to cruise

- child may only be able to attain standing position on tiptoes with legs crossed
- cruising may not be possible due to lack of LE separation laterally
- walking limited by lack of separation in sagittal plane

- adequate trunk control may be lacking to provide stable base for stance leg
- adequate force production may be lacking to produce controlled movement of swing leg

- absent trunk rotation may prompt arm movements to initiate weight shifts in LEs or substitute for lack of LE movement
- arms may remain in high guard to reinforce weak trunk muscles by sustaining an extended posture and delay onset of arm swing

Front 326

What difficulties does a child with spastic CP encounter in extremity usage?

Back 326

reaching in any position may be limited by
- inability to bear weight on an extremity, or to
- shift weight onto an extremity and produce appropriate balance response

- WB on UEs necessary for propped sitting and protective extension when other balance responses fail
- WB on LEs is crucial to independent ambulation

- child with spasticity is at risk of contractures and deformities secondary to joint and muscle stiffness and due to muscle imbalances from increased tone
- spasticity may be present only in extremities, with low muscle tone in the trunk

- in an effort to overcome gravity, the child may try to use abdominal muscles to sit from supine
- excessive exertion can increase overall tone and can result in lower extremity extension and possible scissoring (hip adduction) of the legs through associated reactions

Front 327

What difficulties does a child with athetosis or ataxia encounter in mobility and ambulation?

Back 327

- most severe impairments and functional limitations relate to postural instability

- lack consistent head and trunk control
- uses large, uncompensated movements around long axes of trunk/limbs

- may use abnormal movements, such as asymmetric tonic neck posture, to provide additional stability for functional movement
- overuse of this posture can predispose child to scoliosis or hip subluxation

Front 328

Stages for therapeutic intervention for children with CP

Back 328

- early intervention
- preschool
- school age/adolescent
- adult

Front 329

What is the primary emphasis of physical therapy for children with CP?

Back 329

- fostering motor development
- learning functional motor skills

Front 330

How does the sensory feedback loop interfere with motor development in children with CP?

Back 330

when a typical child learns to move, its own movements provide sensory feedback for learning

- when a child with CP learns to move, he/she tends to develop stereotypical patterns because they have difficulty controlling movement against gravity
- they move incorrectly
- they learn to move incorrectly
- they continue to move incorrectly, setting up a cycle for more and more abnormal movement

Front 331

Functional limitations and treatment focus for impairment of:
low or fluctuating muscle tone

Back 331

functional limitation
- postural instability
- poor balance and safety

treatment focus
- hold postures
- cocontraction in midline

Front 332

Functional limitations and treatment focus for impairment of:
wide, uncoordinated movements

Back 332

functional limitations
- poor movement transitions
- unsafe movement

treatment focus
- control and direct movement with resistance
- resist reciprocal movements

Front 333

Functional limitations and treatment focus for impairment of:
lack of midrange control

Back 333

functional limitations
- reaching
- walking

treatment focus
- hold in midrange
- work in small increments of range

Front 334

Functional limitations and treatment focus for impairment of:
lack of use of hands for support

Back 334

functional limitations
- poor movement transitions
- unsafe movement

treatment focus
- weight bearing through arm
- use UE weight bearing for safe movement transitions

Front 335

Functional limitations and treatment focus for impairment of:
lack of graded movement

Back 335

functional limitations
- difficulty grasping
- difficulty changing position

treatment focus
- facilitate shoulder position
- stabilize trunk or opposite extremity

Front 336

Functional limitations and treatment focus for impairment of:
emotional lability

Back 336

functional limitations
- poor judgment of balance risk

treatment focus
- modify behavior

Front 337

Treatment focus for children with spastic CP

Back 337

- mobility in all postures

- transitions between postures

Front 338

How is the tendency to develop contractures countered for children with spastic CP?

Back 338

- range of motion
- positioning
- development of active movement

Front 339

Which areas are prone to tightness in children with spastic CP?

Back 339

quadriplegic CP
- shoulder adductors
- elbow
- wrist
- finger flexors
- show LE tightness as well

diplegic CP
- hip flexors
- hip adductors
- knee flexors
- ankle plantar flexors

- all these joints may be involved unilaterally in hemiplegia

Front 340

Useful techniques to inhibit spasticity:

Back 340

- weight bearing
- weight shifting
- slow, rhythmic rocking
- rhythmic rotation of trunk and body segments

- active trunk rotation, dissociation of body segments, and isolated joint movements should be included in treatment activities and HEP
- appropriate handling can increase likelihood the child will receive more accurate sensory feedback for motor learning

Front 341

Benefits of supine placement of child with spastic CP

Back 341

- enables early weight bearing with knees bent and feet flat on support surface

- to counteract total extension influence of TLR, flex body with
--- upper trunk on wedge
--- legs over bolster
- flexion of the head and upper trunk can decrease effects of supine TLR

- dangling or presenting objects at eye level can facilitate arm use for play or exploration

Front 342

Benefits of side-lying placement of child with spastic CP

Back 342

- best position to dampen effect of tonic reflexes because of neutral head position (do not prop head too high--lateral flexion)
- easy to protract shoulders and pelvis
- easy to rotate trunk in preparation for rolling and coming to sit
(probably meaning it's easy to produce dissociation too)

- side child is lying on is weight bearing and should be elongated (either passively before child is placed in side-lying or as a result of lateral weight shift as the child's position is changed)

Front 343

Benefits of prone placement of child with spastic CP

Back 343

- promotes WB through UEs
- provides some stretch to hip and knee flexors
- head and trunk control facilitated through active extension and promoting hand-eye relationships
- movement while prone can promote UE protective extension and weight shift

Front 344

Benefits of quadruped placement of child with spastic CP

Back 344

- main advantage is WB in all four extremities
- trunk must work against gravity
- great opportunity for dissociated movements of limbs/trunk and upper trunk/lower trunk

Front 345

Benefits of kneeling placement of child with spastic CP

Back 345

- another dissociated posture
- enables practice keeping hips and trunk extended while knees are flexed
- hip flexors can be stretched and balance responses practiced without having to control all of the LE joints
- playing in kneeling is developmentally appropriate
- with support, child can practice moving into half-kneeling

Front 346

Benefits of standing placement of child with spastic CP

Back 346

- obvious WB benefits for long bone growth, etc.
- prolonged stretch on heel cords and knee flexors while promoting active head and trunk control
- provides appropriate visual input for social interaction with peers

Front 347

Benefits of sitting placement of child with spastic CP

Back 347

- almost no better functional position
- WB through extremities while active head and trunk control promoted
- extended trunk dissociated from flexed LEs
- righting and equilibrium reactions can be faciltiated
- can encourage ADLs and transitions

Front 348

Define motor control and motor learning. *** &

Back 348

motor control
- ability to maintain and change posture and movement
- result of neurologic and mechanical processes

motor learning
- process that brings about a permanent change in motor behavior as a result of practice or experience

Front 349

How does sensation contribute to motor control and motor learning? *** &

Back 349

- it is used in motor control as a means of feedback and as a way to prepare the body's posture in anticipation of movement
- needed to learn a new motor skill

Front 350

How can the stages of motor control be used in treatment? *** &

Back 350

they can be used to guide intervention

Front 351

How do the components of the postural control system affect balance? *** &

Back 351

- balance is maintained by controlling posture
- postural control system is affected by
--- limits of stability (BOS)
--- environmental demands
--- flexibility and strength of musculoskeletal system
--- readiness of the posture for movement
--- motor coordination
--- ability of the sensory systems to prepare and guide movement while maintaining balance

Front 352

How is a postural response determined when visual and somatosensory input conflict? *** &

Back 352

the vestibular system provides the defining input to make a postural response when a conflict exists between the information from the visual system and that from the somatosensory system

Front 353

When in the life span can "adult" sway strategies be consistently demonstrated? *** &

Back 353

children demonstrate consistent adult sway strategy responses between 7 and 10 years of age

Front 354

How much attention to a task is needed in the various phases of motor learning? *** &

Back 354

the highest amount of attention is needed in the initial stage of motor learning, with a decreasing amount of attention needed in subsequent stages

Front 355

Give an example of an open task and of a closed task. *** &

Back 355

- driving a car is an example of an open task

- riding on an escalator is an example of a closed task

Front 356

Which type of feedback loop is used to learn movement? To perform a fast movement? *** &

Back 356

- closed loop is used to learn movement because feedback is required to perfect performance
- open loop is used to move quickly, with little or no feedback used

Front 357

How much and what type of practice are needed for motor learning? *** &

Back 357

- much practice is needed to learn a new motor skill
- the best type of practice to use for learning is one that closely resembles the task to be learned, with feedback given intermittently

Front 358

What are the characteristics that identify a developmental theory as being life span in approach? *** &

Back 358

a theory has a life span perspective if it meets Baltes' five criteria that development is:
- contextual
- lifelong
- embedded in history
- multidimensional
- plastic and flexible

Front 359

What theorist described a pyramid of needs that the individual strives to fulfill? *** &

Back 359

Maslow describes a hierarchy of needs that an individual strives to achieve

Front 360

What is an example of a directional concept of development? *** &

Back 360

major examples of the directional concept of development are that development proceeds:
- cephalic to caudal
- proximal to distal
- mass to specific
- gross to fine

Front 361

What three processes guide motor development? *** &

Back 361

the three processes that guide motor development are
- growth
- adaptation
- maturation

Front 362

When does a child typically achieve gross- and fine-motor milestones? *** &

Back 362

major milestones achieved in first year of life:
gross motor:
- 4 months - head control
- 8 months - sitting alone
- 12 months - walking alone

fine motor:
- 2 months - hand regard
- 6 months - voluntary grasp
- 12 months - superior pincer grasp

Front 363

What are the typical postures and movements of a 4-month old and a 6-month old? *** &

Back 363

typical 4-month old's posture
- symmetric - regardless of position
- head control - head kept in line with body when pulled to sit
- mobile when prone
- moves prone to supine with some trunk rotation
- sits propped on arms if placed in that position
- upper trunk extended, but lower trunk still rounded
- may transition to and from many positions, including four-point, propped sitting, prone, and supine

Front 364

What motor abilities cons***ute fundamental motor patterns? *** &

Back 364

- running
- jumping
- throwing
- catching
are considered fundamental motor patterns

Front 365

Why do motor patterns continue to change throughout the life span? *** &

Back 365

motor patterns continue to change across the life span because the patterns are the result of the interaction among the mover, the task, and the environment

Front 366

What role does decreased activity play in an older adult's posture? *** &

Back 366

- decreased activity can accentuate the effect gravity has on an older individual's ability to remain erect
- extending against gravity, as in rising from a chair or walking with an erect posture, may become more difficult as a result of ***ulative changes in body systems related to normal or pathologic aging

Front 367

What gait changes can have an impact on functional abilities in older adults? *** &

Back 367

- decreased speed of gait
- shortened stride length,
- increased double-limb support time
may contribute to less function in stair climbing, stepping over objects, and crossing the street in a timely manner

Front 368

What two activities should always be part of any therapeutic intervention? *** &

Back 368

positioning and handling should always be included in therapeutic intervention

Front 369

What are the purposes of positioning? *** &

Back 369

positioning is used to
- provide support
- encourage functional movement

Front 370

What sensory inputs help to develop body and movement awareness? *** &

Back 370

- touch and movement are important in developing body and movement awareness

- all sensory input is used early on to learn to move

Front 371

Identify tow of the most important handling tips. *** &

Back 371

the two most important handling tips:
- child should be allowed to do as much of any movement as possible
- handling should be decreased as the child gains more control

Front 372

Define key points of control. *** &

Back 372

key points of control are proximal joints from which to guide movement or reinforce a posture

Front 373

Give three reasons to use adaptive equipment. *** &

Back 373

adaptive equipment can be used
- to achieve postural alignment
- to prevent contractures and deformities
- to provide mobility
(other goals in table 8-5)

Front 374

What are the two most functional postures (positions to move from)? *** &

Back 374

the two most functional postures are
- sitting
- standing

- sitting is functional because so many ADLs can be performed in this position; it provides a stable postural base for UE movement

- standing is functional because it affords the ability to move through the environment in an erect posture, thus combining the mobility of the limbs with the stability of the head and trunk

Front 375

What are the disadvantages of using a quadruped position? *** &

Back 375

the major disadvantage of the quadruped position is that it is a flexed posture and it is difficult for some children to learn to move in this position

Front 376

Why is side sitting a difficult posture? *** &

Back 376

- side-sitting is a difficult posture to master because it requires trunk rotation to have one's hands free
- because of the flexed LEs, the lower trunk is rotated in one direction
- the upper trunk must be rotated in the opposite direction to free the hands

Front 377

Why is standing such an important activity? *** &

Back 377

- standing is an important activity because the loading forces placed on the long bones of the LEs produce positive physiologic changes
- it is also important because it is socially acceptable to be at eye level with one's peers
- the sensory input for being upright is beneficial to perceptual development

Front 378

Why may the clinical manifestations of CP appear to worsen with age even though the pathologic features are static? *** &

Back 378

- clinical manifestations of cerebral palsy may appear to worsen with age because the child's motor abilities cannot adapt to the increased environmental demands

- lack of function of the originally damaged areas of the brain can interfere with the function of other areas of the brain

Front 379

Name the two greatest risk factors for CP. *** &

Back 379

- prematurity
- low birth weight

- two greatest risk factors for CP

Front 380

What is the most common type of abnormal tone seen in children with CP? *** &

Back 380

spasticity, or increased muscle tone, is the most common type of abnormal tone seen in children with cerebral palsy

Front 381

How may abnormal tonic reflexes interfere with acquisition of movement in a child with CP? *** &

Back 381

- tonic reflexes such as the ATNR, STNR, or TLR can interfere with the acquisition of coordinated movement because they can dominate a child's posture if they are obligatory

e.g.,
- an obligatory ATNR may prevent the child from rolling
- an obligatory STNR may prevent the child from performing reciprocal extremity movements
- an obligatory TLR may prevent development of antigravity head and trunk control

Front 382

Compare and contrast the focus of physical therapy intervention in a child with spastic CP and in a child with athetoid CP. *** &

Back 382

- focus of physical therapy intervention in children with spastic cerebral palsy is on mobility because their tone is increased and movements are limited to stereotypical patterns

- focus of physical therapy intervention in children with athetoid cerebral palsy is on stability because these children lack the ability to sustain static postures and maintain head and trunk stability while moving their extremities

Front 383

What is the role of the PTA when working with a preschool-age child with CP? *** &

Back 383

- PTA implements certain aspects of the treatment plan formulated by PT

- activities can include those
--- promoting postural reactions
--- transitional movements
--- strength
--- endurance
--- self-care skills

Front 384

What type of orthosis is most commonly used by children with CP who ambulate? *** &

Back 384

the most frequently used orthosis in ambulatory children with CP is some type of AFO

Front 385

At what age should a child with CP begin to take some responsibility for his/her own therapy program? *** &

Back 385

the school-age child should be taking some degree of responsibility for his/her own therapy program

Front 386

What medications are used to manage spasticity in children with CP? *** &

Back 386

- many medications have been used to decrease spasticity in children with CP
- medications of choice at this time:
--- botulinum toxin
--- baclofen

Front 387

What are the expected life outcomes that should be used as a guide for goal setting for children with disabilities? *** &

Back 387

Giangreco and colleagues (1993) identified five life outcomes that could be used as a guide for goal setting:
- having a safe, stable home in which to live now and in the future
- having access to a variety of places within a community
- engaging in meaningful activities
- having a social network of personally meaningful relationships
- being safe and healthy

Front 388

What type of paralysis can be expected in a child with MMC? *** &

Back 388

the classic presentation is flaccid paralysis; however, if some innervation occurs below the level of the myelomeningocele, spastic paralysis can be present

Front 389

What complications are seen in a child with MMC that may be related to skeletal growth? *** &

Back 389

complications related to skeletal growth can include:
- tethered spinal cord
- scoliosis
- kyphosis
- lordosis

Front 390

What are the signs of shunt malfunction in a child with MMC? *** &

Back 390

common signs of shunt malfunction in a child:
- irritability
- seizures
- vomiting
- lethargy

Front 391

What position is important to use in preventing the development of hip and knee flexion contractures in a child with MMC? *** &

Back 391

prone positioning is important to prevent development of hip and knee flexion contractures

Front 392

What precautions should be taken by parents to protect skin integrity in a child with MMC? *** &

Back 392

areas of skin insensitivity should be protected by clothing and should be inspected on a routine basis

Front 393

What determines the type of orthosis used by a child with MMC? *** &

Back 393

- age
- level of lesion
- strength of innervated musculature
must be taken into account when choosing an orthosis for a child with myelomeningocele,

Front 394

What is the relationship of motor level to level of ambulations in a child with MMC? *** &

Back 394

the lower the level of lesion, the more functional the level of ambulation will be and the more likely that level of ambulation will be continued through the life span

Front 395

When is the functional level of mobility determined for an individual with MMC? *** &

Back 395

the ultimate functional level of mobility can be determined during the school years when physical maturity peaks

Front 396

What developmental changes may contribute to a loss of mobility in the adolescent with MMC? *** &

Back 396

- changes in body weight
- body proportions
- immobilization from skin breakdown or orthopedic surgery
- spinal deformity
- joint pain
- ligamentous laxity
can contribute to a loss of mobility in an adolescent with myelomeningocele

Front 397

When is the most important time to intervent therapeutically with an individual with MMC? *** &

Back 397

physical therapy intervention is crucial at an early age to teach functional movement and is important any time the child makes the transition from one mode of mobility to another or from one setting to another

Front 398

What is the leading cause of inherited mental retardation? *** &

Back 398

DS is the leading chromosomal cause of mental retardation

Front 399

When one parent is a carrier for CF, what chance does each child have of being affected? *** &

Back 399

each child has a 1 in 4 chance of having CF

Front 400

What genetic disorder produces muscle weakness without cognitive impairment? *** &

Back 400

SMA is a progressive disease of the nervous system that produces muscle weakness but no mental retardation

Front 401

What are the three mechanisms by which chromosome abnormalities occur? *** &

Back 401

chromosome abnormalities occur by one of three mechanisms:
- nondisjunction
- deletion
- translocation

Front 402

What are the two most common clinical features in children with most genetic disorders involving the CNS? *** &

Back 402

children with most genetic disorders commonly present with hypotonia and some degree of mental retardation

Front 403

What principles of motor learning are important to use when working with children with cognitive impairment? *** &

Back 403

the motor learning principles of practice and repetition are important to use in any intervention with a child with mental retardation

Front 404

What types of interventions are appropriate for a child with low tone? *** &

Back 404

appropriate interventions for a child with low tone include
- approximation
- weight bearing in proper postural alignment
- activities that increase muscular work against gravity

Front 405

What interventions can be used to prevent secondary complications in children with low tone? *** &

Back 405

- soft splints can be used to prevent elbow or knee hyperextension
- positioning can be used to encourage midline orientation
- a strong balanced trunk can improve trunk control for controlled movement transitions that require trunk rotation and can support adequate respiratory function for breathing and phonation

Front 406

What interventions are most often used with a child with OI? *** &

Back 406

- interventions for children with OI should focus on developmental activities within safe limits
- all rotations should be actie
- orthoses are used to support and protect joints
- positioning should be used to minimize joint deformities
- the pool is an excellent medium for exercise

Front 407

What physical therapy goal is most important when working with a child with a progressive genetic disorder? *** &

Back 407

- the ultimate physical therapy goal is to provide education and support for the family while managing the child's impairments

- management is a total approach with preventive and supportive aspects

Front 408

What is the focus for treatment for the child with athetosis?

Back 408

- stability in weight bearing
- use of developmental postures that provide trunk or extremity support

Front 409

What are some useful techniques for assisting the child with athetosis to develop stability?

Back 409

- approximation
- weight bearing
- moving within small ranges of motion with resistance as tolerated

Front 410

How can the therapist provide information about joint and postural alignment to a child with athetosis?

Back 410

sensory cueing, possibly using
- mirrors
- weight vests
- heavier toys that provide some resistance but do not inhibit movement

Front 411

What is the most difficult muscular control issue for a child with athetosis? What types of activities can help?

Back 411

- grading movement within the midrange, where stability is typically the greatest

- playing "statues"
- holding ballet positions
- holding any other fixed posture (e.g., stork standing)

Front 412

Name two other ways (besides holding of fixed postures) of helping improve the stability of a child with athetosis.

Back 412

- use of hand support in sitting, kneeling, and standing

- visually fixing on a target

Front 413

What are the 5 life outcomes that should be highly valued for all children, even those with severe disabilities (per Giangreco, et. al.)? How should they be used?

Back 413

- having a safe, stable home in which to live now and in the future
- having access to a variety of places within a community
- engaging in meaningful activities
- having a social network of personally meaningful relationships
- being safe and healthy

- these outcomes should be used to guide goal setting for children with disabilities across the life span

Front 414

What is plasticity?

Back 414

the ability of the nervous system to be flexible in its response to injury and development

Front 415

What problems are frequently identified in an infant with CP during a physical therapy examination?

Back 415

- lack of head control
- inability to track visually
- dislike of the prone position
- fussiness
- asymmetric postures secondary to exaggerated tonic reflexes
- tonal abnormalities
- feeding or breathing difficulties

Front 416

What two activities are typically developing infants working on in the first three years of life?

Back 416

- establishing trust in their caregivers

- learning how to move about safely within their environment

Front 417

What abilities should the PTA bring to the treatment team of an infant with CP?

Back 417

- knowledge of positioning and handling techniques
- use of adaptive equipment
- management of impaired tone
- developmental activities that foster motor abilities and movement transitions within a functional context

Front 418

What are the general goals of physical therapy in early intervention?

Back 418

- promote infant-parent interaction
- encourage development of functional skills and play
- promote sensorimotor development
- establish head and trunk control
- attain and maintain upright orientation

Front 419

What should handling and positioning in the supine (“en face”) position promote? Which position is preferred? How is reaching encouraged?

Back 419

- orientation with the head in the midline
- symmetry of the extremities

- flexed position is preferred so the shoulders are forward and the hands can easily come to midline

- reaching is encouraged by making sure visually interesting toys are within the infant’s grasp

Front 420

Besides supine, what other position is important? Why?

Back 420

- prone

- because this is the position from which the infant first moves into (antigravity) extension

Front 421

What should be done if the infant does not like being in the prone position?

Back 421

- some infants do not like being prone, and the caregiver has to be encouraged to continue to put the infant in this position for longer periods

- carrying the infant in prone can increase the child’s tolerance for the position

- the infant should not sleep in prone because of the risk of SIDS

Front 422

What should the carrying position of the infant do and not do?

Back 422

- carrying positions should accentuate the strengths of the infant
- they should avoid as much abnormal positioning as possible

- infant should be allowed to control as much of his/her body as possible for as long as possible before external support is given

Front 423

Neurologic development occurs at the same time at which…

Back 423

- the child’s musculoskeletal and cognitive systems are maturing

- motor learning must take place if any permanent change in motor behavior is to occur

Front 424

A _____ posture facilitates feeding an social interaction

Back 424

- flexed

- the more upright the child is, the easier it is to promote a flexed posture of the head and neck

Front 425

What are the stages of physical therapy for CP?

Back 425

- first stage – early intervention (birth to 3 years)
- second stage – preschool
- third stage – school age and adolescence
- fourth stage - adulthood

Front 426

For what reasons would gentle ROM exercises be used on infants and toddlers in the first phase of physical therapy intervention for CP? What precautions should be observed?

Back 426

- difficulty reaching to midline
- difficulty separating LEs for diapering

- infants do not have complete ROM in LEs normally, so hips should never be forced into full adult-range of adduction or extension

Front 427

Adequate floor time in supine and prone must be balanced with…

Back 427

- benefits of being upright

Front 428

Why are supine and prone floor time necessary?

Back 428

- supine and prone time are needed to encourage movement against gravity
- gravity must be conquered to sit upright and to stand

- body movement during play is crucial to body awareness
- movement within the environment is necessary for spatial orientation to the world

Front 429

What are the two most functional positions for a person? Why?

Back 429

- sitting and standing

- because upright orientation can be achieved with either position

Front 430

When are standing programs typically begun for CP patients? What are the goals? What are the suggested frequency and duration for a standing program?

Back 430

- 12-16 months of age

- improve bone density and development
- manage contractures

- Stuberg recommends 60 minutes of standing, 4-5 times weekly
- Salter reported standing for 45 minutes 3X daily controlled contractures and promoted bone development

Front 431

List some ambulation predictors and potential time frames for ambulation for pediatric patients with CP.

Back 431

- vary, based on type and distribution of disordered movements and achievement of motor milestones
- with or without assistive device

- spastic quadriplegia – greatest variation
- independent sitting by age 2, or ability to scoot on buttocks have good chance of ambulating

- spastic hemiplegia – more likely to ambulate
- high end of normal – about 18 -21 months

- spastic diplegia – slower
- age 2-6, up to age 8

Front 432

With what tasks to children with CP need assistance to attempt ambulation?

Back 432

- weight bearing equally on LEs
- initiating reciprocal limb movement
- balancing

- postural reactions involving the trunk are usually delayed
- extremity protective responses are often delayed as well
- impairments in transitional movements from sitting to standing can impede independence

- children with hemiplegia may avoid movements initiated with the involved side of the body

Front 433

How likely are children with CP likely to ambulate by diagnosis? By motor function? (Table 6-8, p. 141)

Back 433

diagnosis
- monoplegia – 100%
- hemiplegia – 100%
- ataxia – 100%
- diplegia – 85-90%
- spastic quadriplegia – 0-70%

motor function
- sits independently by age 2 – 100%
- sits independently by age 3-4 – 50% community ambulation
- presence of primitive reactions beyond 2 years – poor outlook
- absence of postural reactions beyond 2 years – poor outlook
- independently crawled symmetrically or reciprocally by 2 ½ - 3 years – 100%

Front 434

What mobility devices are available to young children with CP? Are they appropriate? Why or why not?

Back 434

- motorized wheelchairs
- prone scooters
- adapted tricycles
- battery-powered riding toys
- manual wheelchairs

- they are appropriate for children as young as 17-20 months and teach young children that they can control the environment around them, rather than being controlled by it

Front 435

What are the goals of the second stage of physical therapy intervention for CP —preschool period?

Back 435

- establish a means of independent mobility
- promote functional movement
- improve performance of ADLs such as grooming and dressing
- promote social interaction with peers

Front 436

What types of activities might a PTA do with a preschooler in the second stage of physical therapy intervention for CP?

Back 436

- promoting postural reactions to improve head and trunk control
- teaching transitions
- stretching to maintain adequate muscle length for function
- strengthening and endurance exercises for promoting function and health
- practice of self-care skills

Front 437

In what way is the second stage of physical therapy intervention for CP a period of monitoring and reexamination?

Back 437

- may question continued need for either assistive or orthotic device
- may need to add an assistive or orthotic device because of changing musculoskeletal status, body weight, seizure status, or safety concerns
- previous degree of motor control may have been adequate for a small body, but growth may produce a loss of control

Front 438

What changes in status of a preschooler in the second stage of physical therapy intervention for CP should be brought to the attention of the PT?

Back 438

- difficulties with a task previously performed without problems
- request a reexamination any time negative changes in motor performance occur
- positive changes should be documented as well, as they may necessitate updating the POC

Front 439

What specific gait difficulties are seen in CP patients with spastic quadriplegia? Spastic diplegia?

Back 439

spastic quadriplegia
- depends on motor involvement, and gait may never be functional—only therapeutic exercise during sessions

spastic diplegia
- lack of lower extremity dissociation
- decreased single-limb and increased double-limb support time
- limited postural reactions during weight shifting

Front 440

With what motions do spastic diplegia CP patients have the most difficulty? How can therapy help remedy these deficiencies?

Back 440

- problems dissociating one leg from the other and dissociating leg movements from trunk
- they often fix/stabilize with hip adductors to substitute for lack of trunk stability in upright necessary for initiation of LE motion

- practicing coming to stand over a bolster can deter LE adduction while child works on muscle strengthening and weight bearing
- practicing lateral trunk postural reactions may automatically result in LE separation as the LE opposite the weight shift is automatically abducted
- addition of trunk rotation to lateral righting may even produce external rotation of opposite leg

- as double-limb support time decreases and step length becomes more appropriate, can have them step over an object or on/off a step
- single-limb balance
- holding vertical poles to decrease amount of support and to facilitate upper trunk extension

Front 441

For what are AFOs used? How are they prescribed? What precautions are necessary?

Back 441

- keep subtalar joint in neutral position
- controlling (over)pronation
- maintaining musculotendinous gains following surgery or casting

- PT must establish wearing schedule
- may be worn day and night

- skin must be inspected regularly for breakdown or excessive pressure
- areas of redness lasting >20 minutes after brace removal should be reported to supervising PT

Front 442

For what is a supramalleolar orthosis (SMO) used?

Back 442

- for a child with unstable ankles who needs medial-lateral stability
- allows the child to freely dorsiflex and plantar flex while restricting mediolateral movement
- may be indicated for a child with mild hypertonia
- in a child with hypotonia or athetoid CP, SMO may provide sufficient stability within a tennis shoe to allow ambulation

Front 443

What assistive devices should be avoided with the preschooler in the second stage of physical therapy intervention for CP?

Back 443

- walkers that do not require the child to control the head and trunk as much as possible (passive)
- walkers that induce increased LE extension and toe walking
- exercise saucers can be dangerous as walkers
- jumpers should be avoided in children with increased LE muscle tone

Front 444

If a child with CP has not achieved independent functional ambulation by age 3, what should be considered?

Back 444

alternative mobility
- adapeted tricycle
- manual wheelchair
- mobile stander
- battery-powered scooter
- power wheelchair

Front 445

What two issues are of greatest concern to parents and caregivers of a child with CP using power mobility?

Back 445

- accessibility
- independence

- home or environment needs to be accessible
- needs of caregiver must be considered
- child must be adequately trained

Front 446

The second stage of physical therapy intervention for CP is the period where the child is most likely to require __________ for spasticity or musculoskeletal deficits. What agents are used?

Back 446

- medical and/or surgical management

medications – usefulness limited, generally due to side effects
- diazepam (Valium) – causes drowsiness and increased drooling
- baclofen (Lioresal) – causes drowsiness and increased drooling
- dantrolene (Dantrium)
(baclofen delivered via pump directly to spinal cord takes less medication for a greater effect; child not considered for this before age 3, and it takes up to 6 mos to see results; also expensive)

botulinum toxin – small amount injected into spastic muscle; produces weakness and decline in spasticity for 3-6 months; makes it easier to:
- position a child
- fit an orthosis
- improve function
- provide information about the appropriateness of muscle lengthening

Front 447

Orthopedic surgery is often inevitable for children with CP. For what reasons is it done?

Back 447

- decrease pain
- correct or prevent deformity
- improve function

lengthen soft tissues
- lengthen tendons
- release spastic muscle groups
- lengthen tight adductors or hamstrings

Front 448

What types of surgeries are performed on the tendons, muscles, and nerves of children with CP?

Back 448

- tenotomy – tendon is completely severed
- partial tendon release – severing part of tendon or moving tendon attachment
- neurectomy – severing the nerve to a spastic muscle

- implantation of baclofen pump – catheter threaded below skin around to the back, where it is inserted trhough the lumbar spine into the intrathecal space, allowing direct delivery of medication into CSF; greatest advantage is adjustable dosage, with smaller amounts of medication needed for good results and fewer systemic complications

Front 449

Describe the process of lengthening the heel cord in a child with CP. What can go wrong?

Back 449

- surgical lengthening of heel cord done to improve walking
- enables patient to achieve a flat (plantigrade) foot
- results in more dorsiflexion and weaker plantar flexion

- overlengthening can occur, resulting in calcaneal gait or too much dorsiflexion during stance
--- may predispose child to crouched posture and development of hamstring and hip flexion contractures

Front 450

What types of orthopedic surgery are done on the hips children with CP? What are some issues that cause hip problems?

Back 450

- done for hip subluxation or dislocation
---minimal level - soft tissue release of the adductors, iliopsoas, or proximal hamstrings
--- higher level – osteotomy in which angle of femur is changed by severing, then derotating the femur and providing internal fixation; occasionally the acetabulum must be reshaped
--- highest level – hip replacement/arthrodesis

- hip may sublux secondary to muscle imbalances from an obligatory ATNR

Front 451

What is a selective posterior (dorsal) rhizotomy (SDR)? Who are the best and worst candidates for this procedure?

Back 451

- a treatment for spasticity in children with CP
- dorsal roots identified by electromyographic response as producing spasticity are selectively cut to decrease afferent activity
- through careful selection, touch and proprioception remain intact

- best candidates have spastic diplegia, hemiplegia, and quadriplegia with good cognitive abilities
- children with cerebellar or basal ganglia deficits are not good candidates

Front 452

What is the focus of the third stage of physical therapy intervention for school-age and adolescent children with CP?

Back 452

- safeguard all previous gains
- continue independent mobility
- develop independent skills related to ADLs and instrumental ADLs
- foster fitness and development of a positive self image
- foster community integration
- develop vocational plan
- foster social interaction with peers

- begin shifting some responsibility for the therapy program to the patient
- find activities that motivate a rebellious teen (everyone loves a contest)
- find out what important motor task the patient wants to accomplish
- channel concern for appearance
- deal with changes from adolescent growth spurts (less stable gait, decrease in static/dynamic balance, bone growth outpaces muscle growth causing contractures, more body mass on smaller muscle mass could threaten continued functional independence)

Front 453

What is the focus of the fourth stage of physical therapy intervention for adults with CP?

Back 453

- independence in mobility and ADLs
- healthy lifestyle
- community participation
- independent living
- a vocation

Front 454

What factors determine the ability of an adult with CP to live and work independently?

Back 454

- cognitive status
- degree of functional limitations
- adequacy of social and financial support

Front 455

What is myelomeningocele? Spina bifida?

Back 455

- MMC is a complex congenital anomaly primarily affecting the nervous system (but also musculoskeletal and urologic systems.) It is due to faulty development of the spinal cord, especially the lower segments—the caudal end of the neural tube fails to close before the 28th day of gestation

- accompanying the spinal cord dysplasia, is a bony defect called spina bifida; it occurs when the posterior vertebral arches fail to close and form the spinous process

Front 456

What are the three types of myelodysplastic defects?

Back 456

spina bifida occulta
- incomplete vertebral closure
- no sac/cyst
- area of skin over defect may be marked by tuft of hair or dimple
- vertebral defect usually not associated with an abnormality of spinal cord

meningocele (one of two types of spina bifida cystica/aperta)
- incomplete vertebral closure
- cyst of meninges and CSF, usually covered with epitheliam
- clinical symptoms variable

myelomeningocele (one of two types of spina bifida cystica/aperta)
- incomplete vertebral closure
- cyst of CSF, meninges, spinal cord, and possibly nerve roots
- defect may be covered by skin, or only by meninges
- cord incompletely formed or malformed
- most common in lumbar area
- higher the lesion, the more sensory/motor deficits

other types mentioned
anencephaly
- lack of closure cephalically
- failure of the brain to develop beyond brain stem
- infants rarely survive for any length of time after birth

encepalocele
- brain tissue protrudes from skull
- results in visual impairments

Front 457

What are some potential causes of spina bifida and MMC?

Back 457

- folic acid deficiency in the diet
- genetic predisposition
- exposure to alcohol/FAS
- Irish/Irish-American ethnicity

Front 458

How is a neural tube defect diagnosed and treated prenatally?

Back 458

- testing levels of alpha fetoprotein – too high may mean an open neural tube
- high-resolution ultrasonography

- delivery by cesarean section before labor begins to decrease risk of CNS infection and/or damage to spinal cord
- fetal surgery between 24-30 weeks gestation to
--- decrease need for placing a shunt for hydrocephalus that typically develops after closure of the myelomeningocele
--- improve LE function

Front 459

Is there a clear correlation between level of spina bifida/MMC lesion and impairment?

Back 459

no
- bony defect may involve more than one vertebral level
- spinal cord may be partially formed or malformed
- part of the spinal cord may be intact at one of the involved levels and may have innervated muscles below the MMC
- in any case, the presentation varies widely

Front 460

Does spina bifida/MMC result in spasticity or flaccidity below the lesion?

Back 460

it may result in either, or a combination of the two
- if the nerve roots are damaged or the cord is dysplastic, the infant will have a flaccid type of motor paralysis
- if part of the spinal cord is intact below the MMC and has innervated muscles, potential exists for spastic type of motor paralysis
- in some cases, the child may demonstrate an area of flaccidity at the level of the MMC with spasticity present below the flaccid muscles
- either type presents difficulty in managing ROM and use of orthoses for ambulation

Front 461

What type of functional movement will a child have with spina bifida/MMC thoracic involvement at T12?

Back 461

- trunk weakness
- some pelvic control due to innervation of quadratus and abdominal muscles
--- T7-T9 upper abdominals
--- T9-T12 lower abdominals
- no gluteus maximus (innervated at L5-S1)
- “frog leg” posture potentially deforming to hips, knees, and ankles

Front 462

What type of functional movement will a child have with spina bifida/MMC involvement at high lumbar level (L1-L2)?

Back 462

- affects LEs
- hip flexors are innervated (and unopposed)
- some hip adductors are innervated
- may cause hip flexion/adduction, hip dislocation, lumbar lordosis, knee flexion, plantar flexion

Front 463

What type of functional movement will a child have with spina bifida/MMC involvement at the mid lumbar level (L3)?

Back 463

- can flex at the hips (strong)
- hip adductors are strong
- hip rotators are weak
- can extend at the knees
- no ankle or toe movement
- may cause hip dislocation/subluxation and genu recurvatum

Front 464

What type of functional movement will a child have with spina bifida/MMC involvement at low lumbar level (L4-L5)?

Back 464

- strong quadriceps
- can flex the knees
- can dorsiflex and invert the ankles
- only weakly extend and abduct hips
- weak plantar flexion and eversion
- may cause equinovarus, calcaneovarus, or calcaneocavus foot

Front 465

What type of functional movement will a child have with spina bifida/MMC involvement at sacral level S1?

Back 465

- weak plantar flexion for pushoff
- good hip abduction

Front 466

What type of functional movement must a child have to be classified as having a level S2 or S3 lesion?

Back 466

- good plantar plantar flexors; must have muscle grade of at least 3/5
- good hip extensors; gluteal muscles must have grade of 4/5
- the lesion is considered “no loss” if the child has normal bowel/bladder function and normal LE muscle strength

Front 467

What position results from classic lower motor neuron presentation of flaccid paralysis in children with spina bifida/MMC?

Back 467

- frog-leg position induced by gravity
- position of comfort – hip abduction, ER, knee flexion and plantar flexion

Front 468

What other congenital LE deformities may be present in children with spina bifida/MMC? What additional deformities are they at risk of acquiring due to muscle imbalances?

Back 468

congenital deformities
- talipes equinovarus (congenital clubfoot)
- pes equinus (flat foot) – extremely flexed toes; pt walks on dorsal surface of toes and heel does not touch ground
- convex pes valgus (rocker-bottom foot) with a vertical talus

deformities due to muscle imbalance
- hip dislocation
- hip dysplasia and subluxation
- genu varus/valgus

(e.g., in a child with L1 or L2 motor level, the hip flexors and adductors may pull so strongly because of increased tone that the hip is dislocated; muscle imbalances due to the level of innervation may be intensified by increased tone)

Front 469

What is the most common foot deformity seen in children with MMC who have an L4/L5 motor level?

Back 469

- talipes equinovarus (congenital clubfoot)

Front 470

How do physical therapists treat talipes equinovarus (congenital clubfoot) in the early management stages?

Back 470

- taping
- gentle manipulation
- gentle corrective ROM

Front 471

Which is recommended for treatment of talipes equinovarus (congenital clubfoot), splinting or serial casting? Why? Ultimately, for all but the mildest cases, what is the recommended treatment?

Back 471

- splinting, because of pressure problems over bony prominences

- surgical correction

Front 472

Between what ages do most children with MMC begin to ambulate?

Back 472

- between 1-2 years

Front 473

What foot characteristics are required for the child with MMC to ambulate?

Back 473

- plantigrade foot (one that can be flat and in contact with the ground)
- 10 degrees of dorsiflexion for proper toe clearance (does not have to be active range)

Front 474

What are two causes of neuropathic fractures in children with MMC?

Back 474

- overly aggressive therapeutic exercise
- lack of stabilization during transfers

Front 475

What two types of scoliosis are seen in children with MMC?

Back 475

- congenital scoliosis – related to the vertebral anomalies (e.g., hemivertebra) that are present in addition to the bifid spine; this type is inflexible

- acquired scoliosis – results from muscle imbalances in the trunk; this type is flexible; managed by orthosis until spinal fixation is appropriate (since children with MMC go through puberty earlier, this allows for earlier spinal surgery with little loss of child’s mature trunk height)

- also, rapid onset of scoliosis can occur secondary to a tethered spinal cord or to a condition called hydromyelia (q.q.v.)

Front 476

What spinal deformities may be present in a child with MMC?

Back 476

- congenital scoliosis (inflexible)
- acquired scoliosis (flexible)
- rapidly occurring scoliosis caused by tethered spinal cord or hydromyelia
- kyphosis – thoracic or entire spine (as the flexed posture of an infant)
- lordosis – exaggerated or reversed

Front 477

What is an Arnold-Chiari malformation? What systems can it affect? What is one key symptom?

Back 477

- most children with MMC have this malformation
- involves the cerebellum, medulla, and cervical spinal cord
- cerebellum is not fully developed, and hindbrain can displace downwardly through foramen magnum
- flow of CSF disrupted, causing hydrocephalus
- accumulation of CSF can exert pressure on and affect the function of cranial nerves (nerve palsies) and brain stem
- may be manifested by swallowing difficulties

Front 478

Precautions for ventriculoperitoneal shunt

Back 478

- physician’s guidance
- avoid spending prolonged time in head-down position as it could disrupt CSF flow
- shunts can become blocked or infected or otherwise malfunction; be aware of the signs

Front 479

What is CNS deterioration in children with MMC? What two conditions can potentially cause it?

Back 479

- change in motor/sensory status or functional abilities affecting any or all areas of child’s functions such as mobility, ADLs, and school performance

can be caused by
- hydromeyelia – accumulation of CSF in central canal of spinal cord characterized by
--- rapidly progressing scoliosis
--- UE weakness
--- increased tone
--- sensory changes
--- ascending motor loss in LEs

- tethered spinal cord – scar tissue from MMC repair does not allow cord to move up as child grows
--- at birth, cord is at L3, rises to L1 in adulthood due to skeletal growth
--- adhesions can form due to scarring from surgical repair of back lesion
--- spinal cord is then anchored at lesion site
--- can cause rapidly progressing scoliosis, decline in motor/sensory function, pain, increased tone, changes in gait, loss of previously attained bladder and bowel control
--- prompt surgical correction can usually prevent permanent neurological damage

Front 480

How may MMC affect sensory impairment?

Back 480

- sensory losses in child with MMC less likely to correspond to motor level of paralysis
- one part of the dermatome may be intact, but skip areas with no sensation may be present
- often only light touch and/or pinprick are tested; if negative, patient may still feel vibration
- skin damage is a big risk and skin inspection needs to be an integral part of POC

Front 481

How does MMC affect bladder and bowel function?

Back 481

- most children with MMC have some degree of bowel/bladder dysfunction
- sacral levels of spinal cord (S2-S4) are responsible for voiding and defecation reflexes
- if not intact, motor and sensory loss can result in reflex emptying or inhibition of voiding
- if tone in bladder wall is increased, bladder cannot hold typical amount of urine and empties reflexively
- mismanagement of voiding can result in kidney damage
- by age 3-4, children should be working on gaining urinary continence
- by age 6, independent in self-intermittent catheterization
- for self-intermittent catheterization sitting balance without hand support and ability to do toilet transfer are needed; these skills should be incorporated into early and middle physical therapy POCs

Front 482

To what substance are over 50% of children with MMC allergic? What does the therapist need to consider?

Back 482

- latex

- toys/balloons containing latex should be avoided in therapy

Front 483

What does the physical therapist do during the first stage of intervention for a child with MMC (birth to ambulation?

Back 483

- establishes motor and sensory levels of function
- evaluates muscle tone, degree of head and trunk control, ROM limitations
- checks for musculoskeletal deformities

Front 484

What are the goals for physical therapy during the first stage of intervention for a child with MMC (birth to ambulation)?

Back 484

- prevention of secondary complications (contractures, deformities, skin breakdown)
- promote age-appropriate sensorimotor development
- prepare child for ambulation
- educate family about appropriate strategies to manage child’s condition

Front 485

What postoperative positioning is used in the first stage of physical therapy intervention for a child with MMC?

Back 485

- child placed prone or side lying until surgical wound heals
- holding infant high on the shoulder with support under arms fosters head control and may be the easiest position for the infant with MMC to maintain a stable head
- as the back heals, brief periods of supine and supported upright sitting
- when a shunt is inserted, follow physician’s orders for positioning

Front 486

Why is prone positioning important in the first stage of physical therapy intervention for a child with MMC?

Back 486

- to prevent development of hip and knee flexion contractures
- to develop head control

Front 487

What effects can gravity have on children with MMC during the first stage of physical therapy intervention?

Back 487

- while supine, paralyzed LEs assume positions of comfort—abducted and externally rotated hips, flexed knees, etc.
- in children with partial innervation of LEs, hip flexion and adduction can produce hip flexion contractures and can lead to hip dislocation because of the lack of muscle pull from hip extensors or abductors
- genu recurvatum is seen when strong quadriceps are not opposed by equally strong hamstrings to balance the knee extension posture
- when only anterior tibialis function is present, a calcaneovarus foot results (dorsiflexed, inverted, and adducted foot—basically walking on the outer edge)

Front 488

Why is it detrimental to adduct the hips completely early in development of children with MMC?

Back 488

- because the hip joints are incompletely formed and may sublux or dislocate if adducted beyond neutral
- aim for positioning in neutral hip, neutral knee, and neutral ankle/foot position

Front 489

What is important to remember about skin that is anesthetic?

Back 489

- it does not sweat
- it cannot conserve heat or give off heat, thus must be protected

Front 490

How often should PROM be done with children with MMC during the first stage of physical therapy intervention? How should they be performed?

Back 490

- two to three times daily
- exercises may be combined for efficiency (e.g., hip and knee flexion on one side and hip and knee extension on the other, bilateral hip abduction, etc.)

- ROM exercises should be done gently, with the hands near the joints for short lever arms
- hold the motion briefly at the end range
- even in presence of contractures, aggressive stretching is not indicated (serial casting if persistent PROM exercise doesn’t work)
- ROM exercises are easy to forget once infant becomes more active, but they are still important
- once child is able, he/she should be responsible for doing own daily ROM

Front 491

How should foot/ankle ROM be done on children with MMC during the first stage of physical therapy intervention?

Back 491

- individually (one foot at a time)
- subtalar joint must be kept neutral when doing dorsiflexion to ensure movement occurs at correct joint
- if foot goes into varus or valgus, motion will take place at midfoot (vice hindfoot)
- rocker-bottom foot may be caused by allowing motion at the wrong place

Front 492

What are the signs and symptoms of shunt malfunction?

Back 492

infant
- bulging fontanelle
- sunset sign of eyes (only part of iris is visible because of downward gaze
- excessive rate of growth of head circumference
- thinning of skin over scalp

school-age children
- personality changes
- memory changes

all ages
- irritability
- seizures
- vomiting
- lethargy
- headaches
- edema, redness along shunt tract

Front 493

Why might head control in prone be more difficult for children with MMC during the first stage of physical therapy intervention? How can this be overcome?

Back 493

- difficulty lifting head made larger by hydrocephalus
- hypotonic neck and trunk muscles

- extra support from a bolster or small half-roll under the chest assists in distributing weight farther down the trunk; also helps bring UEs under the body to assume prone-on-elbows position

Front 494

Why is head control in supine important to children with MMC during the first stage of physical therapy intervention?

Back 494

- to balance development of axial extension with axial flexion
- if supported on a wedge, can encourage chin tuck or forward head lift into flexion

Front 495

What is the leading chromosomal cause of MR and the most frequently reported birth defect?

Back 495

- Down Syndrome

- 1 in 700 births

Front 496

What causes Down Syndrome?

Back 496

- an extra 21st chromosome, or trisomy 21 in all or most of body’s cells
- 95% of cases result from failure of chromosome 21 to split completely during formation of egg or sperm (why advanced maternal/paternal age is a factor)
- when abnormal gamete joins normal gamete the result is 3 copies of chromosome 21
- fewer than 5% have a third chromosome 21 attached to another chromosome; this type of DS is caused by translocation
- mosaic type DS - the least common type of DS; some body cells have three copies of chromosome 21 and others have a normal complement of chromosomes
- severity of the syndrome is related to the proportion of normal to abnormal cells

Front 497

What are the characteristic features of a child with DS?

Back 497

- hypotonicity
- joint hypermobility
- upwardly slanting epicanthic folds
- flat nasal bridge and facial profile
- small oral cavity that sometimes causes tongue to seem to protrude
- delayed development and impaired motor control
- feeding problems
- congenital heart disease (40-66%),usually surgically correctable
- pes planus
- thoarolumbar scoliosis
- patellar and possibly atlantoaxial instability (15-20%) requiring surgical fusion
- visual impairments – myopia,astigmatism, cataracts, nystagmus, esotropia (crossed eyes)
- mild to moderate hearing loss – either sensorineural (damage to CN VIII) or conductive (too much fluid in ears); needs to be identified early and treated aggressively so as to not hinder ability to interact and develop appropriate language

Front 498

What is an “educable” person?

Back 498

- one who is capable of learning basic skills such as reading and arithmetic, and is capable of self-care and independent living (individuals with mild MR are generally considered educable)

Front 499

Are persons with moderate MR “educable”?

Back 499

- they are trainable, but very limited in educational attainments
- they can profit from simple training for self-care and vocational tasks

Front 500

What are the signs and symptoms of atlantoaxial instability?

Back 500

- hyperreflexia
- clonus (repetitive, involuntary muscle spasm)
- Babinski sign
- torticollis
- increased loss of strength
- sensory changes
- loss of bowel or bladder control
- decrease in motor skills

Front 501

What is the chief cause of difficulty in learning motor skills in children with DS? What are the reasons for these defects?

Back 501

- lack of postural tone, and
- hypermobile joints (to a degree)

- hypotonia is related to structural changes in the cerebellum, but also to changes in other CNS structures and processes, which are indicative of missing or delayed neuromaturation in DS
- ligamentous laxity and joint hypermobility are thought to be due to a collagen defect

Front 502

How does the child with DS typically compensate for low tone and hypermobile joints in motor tasks?

Back 502

- typically by locking extremity joints like elbows and knees
- they substitute positional stability for muscular stability
- e.g., W sitting to provide trunk stability, instead of dynamically firing trunk muscles in response to weight shifting
- children with DS often avoid activating trunk muscles for rotation and prefer to advance from prone to sitting by walking up with hands over widely abducted legs

Front 503

How do children with DS compare to neurotypical children with respect to attainment of developmental milestones over the first year? (Skill and month achieved from table 8-1 on page 187)

Back 503

neurotypical vs. DS
- rolls from supine to prone – 6 mos/7 mos
- prone on elbows – 6 mos/7 mos
- pivots in prone on stomach – 6 mos/12 mos
- sits with back erect – 8 mos/11 mos

- pulls to stand – 9 mos/18 mos
- cruises around furniture – 9 mos/21 mos
- creeps on hands and knees – 10 mos/15 mos
- walks with assistance – 12 mos/30 mos

Front 504

How do children with DS compare to neurotypical children with respect to attainment of developmental milestones after the first year? (Skill and month achieved from table 8-1 on page 187)

Back 504

neurotypical vs. DS
- squats during play – 22 mos/36 mos
- walks down stairs, one hand held – 22 mos/36 mos
- throws a ball – 24 mos/30 mos
- kicks a ball after demonstration – 24 mos/36 mos
- jumps off floor, both feet – 30 mos/48 mos
- walks up stairs, alternating feet – 30 mos/54 mos

Front 505

What long-term health challenges face children with DS?

Back 505

- reduced pulmonary function and fitness, lack of cardiorespiratory endurance and weak abdominal muscles – poor fitness leads children with DS to be less active, and 25% become overweight; fitness needs to be explored as an area of physical therapy intervention

- of the 40-66% of DS children born with congenital heart disease, 76% survive; this percentage drops to 50% by age 30; most heart defects can be surgically corrected

- children with DS have 15-20% higher chance of acquiring leukemia by age 3, although the cure rate is high

- every person with DS who lives past 30 years develops pathologic signs of Alzheimer’s disease, such as amyloid plaques and tangles, but not all of these individuals develop the dementia that is usually part of this condition

- adults with DS who live past 50 years are more prone to regression in adaptive behavior than are adults with MR without DS

Front 506

What are some of the impairments to be addressed by physical therapy intervention for a child with DS?

Back 506

- delayed psychomotor development
- hypotonia
- hyperextensible joints and ligamentous laxity
- impaired respiratory function
- impaired exercise tolerance

Front 507

What is AMC?

Back 507

- arthrogryposis multiplex congenital (a.k.a., multiple congenital contractures)
- nonprogressive neuromuscular syndrome
- results in multiple joint contractures and usually requires surgical intervention to correct misaligned joints
- no cognitive deficit is present
- incidence is 1 in 3,000
- multiple causes identified
- pathogenesis has been related to the muscular, nervous, or joint abnormalities associated with intrauterine movement restriction, with the primary insult likely in the first trimester

- 1/3 of cases have genetic cause
--- gene that causes neuropathic form is found on chromosome 5
--- distal AMC is inherited as an autosomal dominant trait with the defective gene traced to chromosome 9

- if muscles around a fetal joint do not provide enough stimulation (muscle pull) the result is joint stiffness
- if the anterior horn cell does not function properly, muscle movement is lessened and contractures and soft tissue fibrosis occur

Front 508

Which impairments of AMC are typically addressed by physical therapy intervention?

Back 508

- impaired ROM
- impaired functional mobility
- limitations in ADLs, including donning/doffing orthoses

- early intervention focuses on head and trunk control, which may be difficult depending on extent of limb involvement—e.g., child may have difficulty using arms for support when initially learning to sit or catch him/herself if balance is lost
- most become ambulatory, but may need assistance finding ways to go up and down stairs
- adapted tricycle can provide alternative mobility before walking is mastered

Front 509

What are the cornerstones of physical therapy intervention in children with AMC? How are they performed?

Back 509

- ROM exercises
- stretching exercises

- stretching is initially done 3-5 times per day, with each affected joint moved 3-5 times and held for 20-30 seconds at end of available range
- this is a large task because of the multiple joint involvement in this condition
- as child ages, frequency of stretching can be decreased
- school-age child should begin taking over responsibility for his/her own stretching program
- stretching is less important once skeletal growth has ceased, flexibility is still needed to prevent further deformities
- joint preservation and energy conservation techniques are legitimate strategies for adults with AMC

Front 510

How is positioning affected by the contractures in AMC?

Back 510

- positioning depends on the types of contractures
- if UEs are more extended, it will hamper child’s acceptance of prone position and will require chest to be supported by a roll or wedge; this is an excellent position to stretch hip flexion contractures while encouraging the development of the motor abilities of the prone progression
- too much flexion and abduction in LEs may be controlled by lateral towel rolls or Velcro strap
- quadruped is not a good position because it reinforces flexion in UEs and LEs
- prone positioning program should be continued throughout the life span

Front 511

What are the primary means of floor mobility for children with AMC?

Back 511

- rolling and scooting on the bottom

Front 512

What are the time frames for acquisition of independent sitting and to begin experiencing weight bearing in standing?

Back 512

- independent sitting is often delayed due to child’s inability to attain the position
- most gain independent sitting by 15 months
- placement in sitting and encouragement of static sitting balance with our without hand support should begin early, at around 6 months of age
- focus on dynamic balance and transitions into/out of sitting while using trunk flexion and rotation should follow
- weight bearing in standing should begin at around 9 months

Front 513

What is the standing-time goal for a 1-year-old child with AMC?

Back 513

2 hours a day

Front 514

By what age is ambulation achieved by most children with AMC?

Back 514

- 18 months

- because clubfoot is often a part of the AMC presentation, it must be dealt with

Front 515

How is clubfoot addressed in AMC?

Back 515

- early surgical correction often requires later surgical revision, so current recommendations support surgery after the child is stronger and wants to walk, at around the end of the first year

- the operation should be performed before age 2 to avoid having to do more bony surgery as opposed to soft tissue corrections

Front 516

Upon what does use of orthoses and assistive devices for ambulation depend for a child with AMC?

Back 516

- depends on strength of LE extensors and the types of contractures at the hip, knee, and ankle
- less than fair muscle strength at a joint usually indicates need for an orthosis at that joint (e.g., if quadriceps is less than 3/5 on MMT, a KAFO is indicated)
- children with knee extension contractures tend to require less orthotic control than those with knee flexion contractures
- children with weak quadriceps or knee flexion contractures may need to walk with the knees of the KAFO locked
- functional ambulation also depends on child’s ability to use an assistive device; may not be possible due to UE contractures—adapataions to walkers and crutches may be needed
- power mobility may be of benefit in the community and keeping up with peers for a child with weak LEs and poor UE function

Front 517

What is cystic fibrosis?

Back 517

- autosomal recessive disorder of the exocrine glands caused by a defect on chromosome 7
- the most lethal genetic disease in whites
- pancreas does not secrete enzymes to break down fat and protein in 85% of these individuals
- produces respiratory compromise because abnormally thick mucus builds in the lungs, creating a chronic obstructive lung disorder
- parent can be a carrier, giving children ¼ chance of having the disorder (if it’s autosomal recessive, wouldn’t it take BOTH parents being carriers to produce the ¼ chance of having the disease and ½ chance of being a carrier?)

Front 518

Pathophysiology of CF

Back 518

- genetic defect has been localized, but exact mechanism that causes the disease is still unidentified
- ability of salt and water to cross the cell membrane is altered, high salt content in sweat
- thick secretions obstruct mucus-secreting exocrine glands, most severely the lungs and pancreas
- affects multiple systems- GI, reproductive, sweat glands, respiratory system
- pancreatic enzymes and diet help manage pancreatic involvement, but as life expectencies have increased, increased incidence of CF-related diabetes due to damage to beta cells

- lungs normal at birth, but soon thick secretions begin to block airways and affect pulmonary function
- secretions also provide good environment for bacterial growth
- inflammation of the airways brings in infiltrates that eventually destroy the airway walls
- repeated infections and thick secretions produce chronic airway obstruction, eventually leading to bronchiectasis (destruction and widening of the large airways)

- two biggest factors for prognosticating survival are nutrition and pulmonary function

Front 519

What interventions does physical therapy provide to CF patients?

Back 519

help with the following impairments:
- retained secretions
- impaired ability to clear airways
- impaired exercise tolerance
- chest wall deformities
- nutritional deficits

Front 520

What is chest physical therapy (CPT)?

Back 520

- bronchial drainage in specific positions with percussion, rib shaking, vibration, breathing exercises, and retraining
- focused on reducing symptoms
- parents are taught to perform postural drainage 3-5 times daily

Front 521

What is postural drainage?

Back 521

- use of gravity or body position to aid in draining mucus from the lungs; classic 12 positions
- almost always accompanied by percussion and vibration
--- percussion – manually applied with cupped hand while person is in drainage position for 3-5 minutes
--- vibration – chest is vibrated by the therapist as the child exhales to encourage coughing
- percussion and vibration are only applied to areas with retained secretions

- positioning depends direction in which the bronchial branch being drained points
- each segment of the lungs has an optimal position for draining aided by gravity
- coughing used to bring up the mucus loosened by CPT; laughing, crying, or tracheal tickle can induce

Front 522

What is the forced expiration technique?

Back 522

- child in a gravity-aided position is asked to “huff” several times after taking medium-sized breath
- followed by several relaxed breaths using the diaphragm
- sequence of huffing and diaphragmatic breathing repeated as long as secretions are being expectorated
- force of the huffs can be magnified by manual resistance over the epigastric area, or by having the child actively adduct the arms and compress the chest wall laterally
- can be taught to children as young as 4-5 years old

Front 523

What are some alternative forms of airway clearance undergoing research?

Back 523

- positive expiratory pressure (PEP) delivered via mask – easy to use, quicker than CPT, accepted by patients, effective in removing secretions; maintains pressure in lungs, keeping airways open and allowing air to get behind the mucus; combined with forced expriation (huffing) to expectorate

- autogenic drainage – sequence of breathing exercises performed at different lung volumes

- use of a Flutter valve – reported to be an acceptable alternative to CPT; does same thing as PEP mask; also used with autogenic drainage

Front 524

What muscles should be targeted to assist respiration?

Back 524

- focus on shoulder girdle and chest wall
- pectoralis major/minor
- intercostals
- serratus
- erector spinae
- rhomboids
- latissimus dorsi
- abdominals

- stretches to maintain optimal length-tension relationships of chest wall musculature
- respiratory efficiency can be lost when too much of the work of breathing is done by the accessory neck muscles

Front 525

What are breathlessness positions, and lateral basal expansion?

Back 525

- breathlessness positions allow the upper body to rest, to allow the diaphragm to work most easily

- lateral basal expansion is encouraged by manual contact on the lateral borders of the ribs to encourage full expansion of the bases of the lungs

Front 526

What role does exercise play in the lives of children with CF? What types of activities should be avoided? Why?

Back 526

- exercise for cardiovascular and muscular endurance plays a major role in keeping these individuals fit and may slow deterioration of lung function
- exercise improves airway clearance, delays decline in pulmonary function, delays onset of dyspnea, and prevents decreases in bone density
- best reason for them to exercise is to improve aerobic fitness because it correlates with increased survival
- low-impact endurance training is ideal

- exercise program should be based on results of exercise test performed by a PT
- as endurance decreases with disease progression, activities like table tennis may be suggested

- avoid skiing, bungee jumping, parachuting, and scuba diving because they have inherent risks due to altitude, increasing vascular pressure, or air trapping

Front 527

For what must children with CF be monitored during exercise?

Back 527

- desaturation – if their oxygen saturation drops below 90%, exercise must be terminated and the supervising therapist notified before additional exercise is attempted

- monitor with a pulse oximeter

Front 528

How should nutritional needs be adjusted for children with CF engaged in an exercise program?

Back 528

- good nutrition (and pulmonary function) must always be considered
- caloric intake may need to be increased to avoid weight loss because individuals with CF expend more energy to perform exercises than individuals without CF
- fluid replacement is also crucial, and must include electrolytes, not just water; especially important in hot weather

Front 529

What is SMA?

Back 529

- spinal muscular atropy
- progressive disease of nervous system inherited as autosomal recessive trait; genetic mutation on chromosome 5
- second most common fatal recessive genetic disorder seen in children after CF
- 1 of 10,000 live births; prevalence in population is 1 in 6,000, 1 in 40 are carriers of the gene
- degeneration anterior horn cells
- hypotonia of peripheral origin
- damage to LMNs produces low muscle tone or flaccidity, depending on whether some or all of the anterior horn cells degenerate
- muscle fibers have little or no innervation from the spinal nerve if the anterior horn cell is damaged, and the result is weakness
- no intellectual deficits

Front 530

What are the three types of SMA discussed in our text?

Back 530

- Type I SMA (acute infantile SMA or Werdnig-Hoffman disease) – most severe form
- manifests in first months of life, usually quickly and unexpectedly
- limp, frog-legged LE posture evident at birth, along with a weak cry
- deep tendon reflexes are absent, and tongue may fasciculate (quiver) due to weakness
- rapid motor neuron death causes inefficiency of major body organs, especially of the respiratory system, and pneumonia-induced respiratory failure is the most frequent cause of death
- death usually before age 2-3, sometimes within weeks

- Type II SMA (chronic or intermediate form of Werdnig-Hoffman; Dubowitz disease) – onset sometime between (2) 6-18 mos
- affects children who are never able to stand/walk, but maintained a sitting position at some time
- progress varies greatly; some patients avoid progression altogether
- scoliosis is a pervasive problem and may require surgical intervention (if life expectancy is good)
- 12-15% fracture rate; weight bearing recommended to prevent fractures
- most patients live well into adulthood; respiratory system is a major concern

- Type III SMA (Kugelberg-Welander) – onset after 18 months
- patients able to walk without support at some time, but many lose this ability
- progression of the disease is slow
- respiratory involvement is less noticeable, and life expectancy is normal or near normal
- proximal weakness, especially in hips, knees, trunk
- developmental progress is slow; independent sitting by 1 year, independent walking by 3 years
- focus on mobility, including walking; also access, positioning, and pulmonary hygiene
- gait is slow and waddling, often with bilateral Trendelenberg signs; safety an issue as child weakens
- ambulation lost in 50% of patients by age 10; wheelchair dependent by midadulthood
- good UE strength

Front 531

What physical therapy support is provided to infants with Type I SMA (acute infantile SMA, Werdnig-Hoffman disease)?

Back 531

- positioning and family support are most important interventions
- focus is on fostering normal developmental activities and providing access to the environment
- poor head control and need for diaphragmatic respiration may make positioning in prone difficult
- possibly position in side-lying to play
- listening to family concerns is integral part of the therapy

Front 532

What is DMD?

Back 532

- Duchenne’s muscular dystrophy
- X-linked recessive trait—manifests only in boys; girls can be carriers
- 1 in 3,500 male births
- 2/3 of cases inherited, 1/3 from spontaneous mutation
- initially develop motor skills normally
- between ages 3-5 may begin to fall more, have trouble navigating stairs, or use Gower maneuver to rise from the floor, indicating muscle weakness
- elevated levels of creatine kinase in blood as result of muscle breakdown
- definitive diagnosis by muscle biopsy
- intellectual function is below normal, with 25-30% exhibiting some degree of MR

Front 533

Pathophysiology of DMD

Back 533

- children lack gene that produces muscle protein dystrophin
- absence of this protein weakens cell membrane, leads to destruction of muscle fibers
- lack of protein nebulin prevents proper alignment of the contractile filaments during muscle contraction
- muscle fibers break down and are replaced by fat and connective tissue
- fiber necrosis, degeneration, and regeneration are seen on muscle biopsy
- replacement of muscle fiber with fat and connective tissue results in pseudohypertrophy, most apparent in the calves

- smooth muscle is also affected by the lack of dystrophin
- 84% of boys with DMD exhibit cardiomyopathy (heart muscle weakness)
- cardiac failure results either from this weakness or from respiratory insufficiency
- as muscles of respiration become involved, pulmonary function is compromised
- death from respiratory or cardiac failure usually occurs before age 25
- at individual’s/family’s wishes, life can be prolonged by mechanical ventilation

Front 534

How does loss of muscle tissue in DMD affect the child?

Back 534

- with progressive loss of muscle, weakness ensues, followed by loss of AROM and PROM
- limitations in range and ADLs begin around age 5
- inability to climb stairs seen between ages 7 and 13
- ability to ambulate usually lost between 9 and 16 years of age

Front 535

What problems are addressed in a physical therapy intervention for DMD?

Back 535

- impaired strength
- impaired AROM and PROM
- impaired gait
- impaired functional abilities
- impaired respiratory function
- spinal deformities—apparent or potential
- potential need for adaptive equipment, orthoses, and wheelchair
- emotional trauma of the individual and family

Front 536

What is the ultimate goal of the physical therapy program for children with DMD?

Back 536

- provide education and support for the family while
- managing the child’s impairments

Front 537

What types of physical therapy goals are set to manage the impairments of a child with DMD?

Back 537

- prevent deformity
- prolong function by maintaining capacity for ADLs
- facilitate movement
- control discomfort
- movement in some form must be an integral part of a physical therapy POC for a child with DMD

Front 538

How does the weakness manifest in DMD?

Back 538

- proximal muscle weakness is one of the major clinical features of DMD; most clearly present in shoulder and pelvic girdles
- loss of strength eventually progresses distally to encompass all the musculature
- whether exercise can be used to counter the weakness is subject to debate; it has not been shown to hasten progression of the disease, however

Front 539

What types of exercise are beneficial to patients with DMD?

Back 539

- some therapists do not encourage active resistance, instead focusing on preserving functional levels of strength by performing ADLs
- other therapists recommend submaximal exercise as beneficial but only if not burdensome to the family
- theoretically, exercise should be able to assist intact muscle fibers to increase in strength to make up for lost fibers
- exercise capacity is probably best determined by stage and rate of disease progression
- exercise may be more beneficial early as opposed to later in the disease process
- walking should be done for at least 2-3 hours per day; the longer a child can remain ambulatory, the better

Front 540

What key muscles should be targeted to treat weakness in patients with DMD?

Back 540

- abdominals
- hip extensors
- hip abductors
- knee extensors
- triceps
- scapular stabilizers

Front 541

What exercise activities are generally perceived as detrimental to children with DMD?

Back 541

- overexertion
- exercising at maximal levels
- immobility
- high resistance
- eccentric training

Front 542

What factors can help assess or predict future mobility status in children with DMD?

Back 542

- mobility status is related to knee extension strength and gait velocity

- boys with less than antigravity (3/5) quadriceps strength lose ability to ambulate

- speed of walking has been used to predict length of time that will pass before a child with DMD is wheelchair bound
--- a high percentage who could walk 10 meters in less than 6 seconds were more than 2 years away from requiring a wheelchair
--- all the boys who took 12 seconds or longer were in wheelchairs in less than a year

Front 543

What is the potential for contractures in children with DMD? What actions are taken to counter contractures?

Back 543

- potential for muscle contractures is high, and although they cannot be prevented, their progression can be slowed

- every effort should be made to maintain ROM at all joints
- specifically, attention should be paid to the gastrocnemius-soleus complex and TFL
--- tightness in these muscle groups results in gait deviations and a widened BOS

- a prone positioning program (as long as it does not compromise respiratory function) is crucial for managing the detrimental effects of gravity
--- time in prone counteracts the potential formation of hip and knee flexion contractures that develop from too much sitting

- prolonged sitting can all too quickly lead to LE flexion deformities that hinder ambulation
--- alternatives to sitting should be scheduled several times a day

Front 544

Describe the gait abnormalities seen in children with DMD.

Back 544

- ambulate with characteristic waddle because pelvic girdle muscles weaken
- hip extensor weakness can lead to compensatory lordosis, which keeps center of mass posterior to the hip joint
- excessive lateral trunk lean during gait may be seen in response to bilateral Trendelenberg signs indicative of hip abductor weakness
- knee hyperextension may be substituted for quadriceps muscle strength, further increasing lumbar lordosis
- failure to keep body weight in front of knee joint or behind hip joint can result in a loss of the ability to stand
- plantar flexion contractures can compromise toe clearance, lead to toe walking, and make balance even more precarious

Front 545

What medical treatments have been used to try to combat the progression of DMD?

Back 545

- no known treatment can stop the progression of DMD
- steroid therapy – prednisolone has been shown to improve muscle strength and decrease deterioration of muscle function

- two other promising approaches: myoblast transplantation and gene therapy
--- both approaches have met with difficulties, mostly immune reactions
--- a myoblast transfer in subjects with Becker MD had limited success

Front 546

What types of surgical and orthotic management are used to prevent loss of range or ambulation?

Back 546

- as functional gait and range declines, surgical and orthotic management broadens
- Achilles tendon lengthening
- TFL fasciotomy
- tendon transfers
- tenotomies
- myoblast transfers

- these procedures must be followed by vigorous physical therapy to achieve the best gains
- AFOs often prescribed following heel cord lengthening

- orthoses to maintain heel cord length during ambulation
- night splint to incorporate the knees, because knee flexion contractures can be problematic

- in most cases, however, as the quadriceps lose strength, severe lordosis develops to compensate
- this change keeps body weight in front of the knee joints and allows gravity to control knee extension
- child’s gait becomes lurching, and if the ankles do not have sufficient range to keep feet plantigrade, dynamic balance is impaired

Front 547

How is respiratory function treated with respect to children with DMD?

Back 547

- respiratory function must be aggressively managed--breathing exercises and ROM
- diaphragmatic breathing emphasized along with lateral basal chest expansion
- chest wall tightness discouraged by active trunk rotation, passive counterrotation, and manual stretching
- postural drainage with percussion may be needed to clear the lungs
- parents should be taught appropriate airway clearance techniques
- exercise program needs to include aerobic component because the respiratory system ultimately causes the child to die from the effects of the disease

Front 548

Describe Becker MD.

Back 548

- onset between 5 and 10 years of age
- X-linked recessive gene
- 3-6 per 100,000 males—rarer than DMD
- dystrophin present, but in lesser amounts than normal
- less elevation of creatine kinase, less destruction of muscle fibers
- lower incidence of MR
- progression of disease much slower than that of DMD
- likely to ambulate until late teens, live until 40s
- needs vocational assistance

Front 549

Describe Fragile X syndrome.

Back 549

- a.k.a., Martin-Bell syndrome
- leading inherited cause of mental retardation (remember DS is the leading chromosomal cause)
- 1 per 2,000 males; 1 per 4,000 females
- fragile site on the X chromosome at a cellular level

- clinical manifestations vary depending on completeness of the mutation
- disease appears to worsen in successive generations
- symptoms in girls are not as severe as in boys
- girls do not generally have dysmorphic features (structural differences often seen in the face) or connective tissue abnormalities
- children of female carriers have a greater risk of the disorder than those of male carriers

- mental retardation ranges from severe to borderline normal
- average IQ between 20 and 60, with a mean of 30-45
- additional cognitive deficits may include ADHD, learning disability, and autistic-like mannerisms
- those mildly affected may have language delays and behavioral problems

Front 550

What are the characteristics of Fragile X syndrome?

Back 550

- mental retardation
- poor coordination
- general decrease in muscle tone
- enlarged testes after puberty

- long, narrow face
- prominent forehead, jaw and ears

- connective tissue involvement can include
--- joint hypermobility
--- flatfoot
--- inguinal hernia
--- pectus excavatum
--- mitral valve prolapse

Front 551

Describe the motor development in a child with Fragile X syndrome.

Back 551

- both gross- and fine-motor skills are delayed
- average age of walking is 2 years
- 75% of boys exhibit flatfooted, waddling gait
- child’s motor skills are the same developmental age as the child’s mental ability

- maintaining balance in any developmental posture is a challenge due to
--- low tone
--- joint hypermobility
--- gravitational insecurity

Front 552

Describe the tactile defensiveness displayed by children with Fragile X syndrome.

Back 552

regardless of severity of the disorder:
- 90% avoid eye contact
- 80% display tactile defensiveness

- touch can be perceived as aversive, and light touch may elicit withdrawal response rather than an orienting response
- treatment involves use of different-textured surfaces on equipment the child can touch
- vestibular stimulation, firm pressure, and increasing proprioceptive input through weight bearing and movement are helpful

Front 553

How do sensory integration issues manifest in children with Fragile X syndrome?

Back 553

- decreased ability to tolerate being exposed to multiple sensory inputs at one time
- become easily overwhelmed because they cannot filter out environmental stimuli
- when gaze aversion occurs, it is thought to be related to the child’s high degree of anxiety, rather than to autism or social dysfunction
- low tolerance for frustration often leads to tantrums—be alert to child’s losing control and institute appropriate behavior modification responses

Front 554

How should children with Fragile X syndrome be taught motor skills or tasks?

Back 554

- visual learning is a strength—use a visual cue with a verbal request
- teaching a skill or task must be done in the context in which it is expected to be performed (e.g., do not teach tooth brushing in the cafeteria)

- physical, social, and emotional surroundings in which learning takes place are significant for the activity to make sense to the child
- teaching a task in its entirety, rather than breaking it down into its component steps, may help lessen the child’s difficulty with
--- sequential learning and
--- tendency to perseverate

Front 555

Describe the etiology of Rett syndrome

Back 555

- neurodevelopmental disorder that almost exclusively affects females (1 in 12,000)
- presentation suggests X-linked dominant means of inheritance, with supposition that it is lethal to males
- alternatively, could mean males are spared because the mutation comes from the father—daughters would receive the mutated X-chromosome, but sons would receive the Y-chromosome
- however, Rett syndrome has been reported in males

Front 556

What are the characteristics of Rett syndrome?

Back 556

- mental retardation (major cause of MR in females)
--- despite the MR, it is not a neurodegenerative disorder; it represents a failure of postnatal development due to a mutation in an X-linked binding protein
- reduced brain growth
- brain’s ability to form new synapses is severely impaired
- ataxia
- growth retardation
- expressions of the syndrome vary in severity

- early development in affected infants is normal, with symptoms usually appearing during the first year of life
- hypotonia present in infancy is replaced by increasing hypertonia and loss of acquired skills
- develop stereotypical hand motions (flapping, wringing, slapping) as well as mouthing

- decline in function during childhood includes:
--- a decreased ability to communicate
--- seizure activity
--- later scoliosis

Front 557

Motor control occurs because of:

Back 557

physiologic processes that happen at
- cellular
- tissue and
- organ levels
- and that happen quickly to produce timely and efficient movement
- impaired timing or sequencing are deficits in motor control

Front 558

What is the role of sensation in motor control?

Back 558

- initially sensory information cues reflexive movements/reflexive motor response

- as voluntary movement emerges during motor development, sensation provides feedback accuracy
- sensory information is crucial to the mover when interacting with objects and maneuvering within an environment

Front 559

What is the ultimate level of motor control?

Back 559

- voluntary movement

- achieved by maturation of the cortex

Front 560

What is a reflex?

Back 560

- pairing of a sensory stimulus with a motor response

- some are simple, some complex

Front 561

Where do the simplest (primitive) reflexes occur?

Back 561

- spinal cord level (e.g., flexor withdrawal)

- referred to as primitive reflexes because they occur early in the life span of the infant

Front 562

What level is above simple/primitive reflexes? Where do they occur

Back 562

- tonic reflexes
- brain stem
- produce changes in muscle tone and posture
- e.g., ATNR, TLR, etc.

Front 563

By what time frame are infantile reflexes (sucking and rooting), primitive spinal cord reflexes, and tonic reflexes integrated?

Back 563

- by 4-6 months

Front 564

What is integration in the hierarchic theory?

Back 564

- mechanism by which less mature responses are incorporated into voluntary movement
- e.g., adults no longer have a rooting reflex

Front 565

What are the ultimate balance reactions?

Back 565

- righting (postural response – e.g., head/trunk righting)
- protective (protective reaction – e.g., safeguarding of balance—catching oneself with hands/arms when LOB while sitting)
- equilibrium (postural response - )
(develop in this order)

- postural responses continue to be present in adulthood; involve head and trunk; provide body with automatic way to respond to movement of the COG within and outside body’s BOS

- protective reactions are extremity movements in response to quick displacements of COG out of BOS; also considered postural reactions and serve as a backup system should righting or equilibrium reaction fail to compensate for loss of balance

Front 566

With what portion of the brain are automatic postural responses associated?

Back 566

- midbrain (righting reactions)

- cortex (equilibrium reactions)

Front 567

What is the tonic labyrinthine reflex?

Back 567

- produces increased extensor tone when infant is supine

- produces increased flexor tone when infant is prone

Front 568

What is the ATNR?

Back 568

- when infant turns head to right, right arm extends and left arm flexes

- seen to some extent in LE as well

Front 569

Motor control emerges from interaction between _______, _______, and ________.

Back 569

- task
- individual
- environment

Front 570

Because motor abilities of a person change over time, the ______ _____ to a given problem may also change. What other factor may change over time to affect the intricacy of the movement solution?

Back 570

- motor solutions

- motivation

Front 571

What two deficits may be apparent in motor control of individuals with nervous system disease?

Back 571

- impaired timing

- impaired sequencing

Front 572

Initially, sensory information cues reflexive movements, later it provides:

Back 572

- feedback for accuracy

Front 573

The farther up one goes in the hierarchy (spinal cord, brain stem, midbrain, cortex) the more ______ there is of lower structures and the movements they produce (reflexes.)

Back 573

inhibition

Front 574

Inhibition allows previously demonstrated stimulus-response patterns of movements to be integrated or modified into more ________ movements.

Back 574

volitional

Front 575

Levels of reflex in the CNS

Back 575

- spinal cord – primitive reflexes (e.g., rooting, flexor withdrawal)
- brain stem – tonic reflexes (e.g., TLR, ATNR)
- midbrain – righting reactions (e.g., neck righting, optical righting)
- cortex – equilibrium reactions (e.g., ultimate balance reactions—incorporation of limbs)

Front 576

Development of motor control can be described by the relationship of _____ and _____ of body postures, and by the acquisition of ______ ______ _______.

Back 576

- mobility (initial random movements)
- stability (maintenance of a posture)
- automatic postural responses

Front 577

Name the four aspects of development of motor control.

Back 577

- mobility (initial random movements)
- stability (weight-bearing, antigravity maintenance of a posture—tonic holding and cocontraction)
- controlled mobility (weight shifting/movement within a posture)
- skill (movement from one posture to another)

- acquire posture, then develop the control

- mobility combines with stability, then the distal body parts are free to move
- also, proximal mobility combines with distal stability (e.g., weight-shifting in quadruped)

Front 578

- mobility (initial random movements)
- stability (weight-bearing, antigravity maintenance of a posture—tonic holding and cocontraction)
- controlled mobility (weight shifting/movement within a posture)
- skill (movement from one posture to another)

- acquire posture, then develop the control

- mobility combines with stability, then the distal body parts are free to move
- also, proximal mobility combines with distal stability (e.g., weight-shifting in quadruped)

Back 578

- tonic holding (at the end of shortened range of movement, isometric movements)

- cocontraction (simultaneous static contraction of antagonistic muscles around a joint for stability)

Front 579

Differentiate controlled mobility and skill.

Back 579

- controlled mobility – mobility superimposed on stability in WB and movement (proximal mobile, distal stable)

- skill – mobility superimposed on stability in NWB (proximal stable, distal mobile)

(e.g., in walking—the stance limb performs controlled mobility, the swing leg performs skilled work)

Front 580

Postural control develops in a _______ direction, and is demonstrated by the ability to maintain the ___________.

Back 580

- cephalocaudal

- alignment of the body

Front 581

The functions that maintain or preserve alignment have been called _______ or ______.

Back 581

- equilibrium

- balance

Front 582

Righting reactions are responsible for….

Back 582

- orienting head in space

- keeping eyes and mouth horizontal

Front 583

Protective reactions are extremity movements that occur in response to….

Back 583

- rapid displacement of the body by diagonal or horizontal forces

(by extending one or both extremities, the individual prepares for a fall or prepares to catch himself)

Front 584

When do the protective extension reflexes show up in the UEs? In the LEs?

Back 584

- forward protective extension – 6 months
- lateral protective extension – 7-8 months
- backward protective extension – 9 months

- protective staggering of the LEs – 15-17 months

Front 585

________ reactions are the most advanced postural reactions and are the last to develop. What do they do?

Back 585

- equilibrium

- allow the body as a whole to adapt to slow changes in the relationship of the COG with the BOS

Front 586

Trunk rotation as an equilibrium response is only evident during _______ displacements.

Back 586

lateral

Front 587

What are the three expected responses to a lateral displacement of the COG toward the periphery of the BOS in standing?

Back 587

- lateral head and trunk righting away from the weight shift
- arm and leg opposite the direction of the weight shift abduct
- trunk rotation away from the weight shift may occur

- if the trunk rotation does not occur, the other two responses can provide only brief postponement of the inevitable fall

Front 588

Systems model of motor control differs from hierarchal model of motor control chiefly by:

Back 588

- the view that motor control is accomplished by the complex interactions of many systems, not just the nervous system

- the view that posture and movement are thought to be self-organizing by maturation of the nervous system (thus increased speed) and the changing relationships among body systems produces different motor responses

- the characteristic of feedback loops
--- closed-loop model – sensory information used as feedback to assist nervous system with next action
--- open-loop model – movement performed without feedback; error detection and feedback are ex post facto; for fast movements

Front 589

What is the basic functional unit in the systems theories of motor control?

Back 589

movement pattern—movement emerges from complex interaction of changing body systems
(rolling, creeping, etc. are movement patterns)

Front 590

The relationship between posture and movement is called _______ ______.

Back 590

postural control

(postural control system has 7 components)

Front 591

What are limits of stability (part of the postural control system)?

Back 591

- boundaries of BOS of any given posture
- as long as COG is inside BOS, person is stable
- CNS perceives the body’s limits of stability through various sensory cues

Front 592

What is environmental adaptation (part of the postural control system)?

Back 592

body’s sensory systems provide input that allows the generation of a movement pattern that dynamically adapts to current conditions

Front 593

What is the role of the musculoskeletal system as part of the postural control system?

Back 593

- provides the mechanical structure for any postural response

- includes postural alignment and musculoskeletal flexibility of all body segments

Front 594

What is a predictive central set (part of the postural control system)?

Back 594

- postural readiness
- sensation and cognition cue movement and anticipate what movement is to come

- anticipatory preparation = feedforward processing – sensory information sent ahead to prepare for the movement to follow (in contrast to feedback, which is sent back after the fact for comparison and error detection)

- many adults with neurologic deficits lack this anticipatory preparation, so postural preparedness is often a beginning point for treatment

Front 595

What is motor coordination (part of the postural control system)?

Back 595

ability to sequence muscle response in a timely manner to respond to displacement of COG within BOS

Front 596

What is the cone of stability?

Back 596

in standing, the area in which a person can move within the limits of stability or BOS

Front 597

What is postural control?

Back 597

- relationship between posture and movement
- readiness to react to threats to balance
- ability to anticipate postural needs to support a motor plan (plan to move)

Front 598

What is eye-head stabilization (part of the postural control system)?

Back 598

- visual and vestibular systems coordinate
- provide accurate information about surrounding environment during movement and gait
- keep eyes and head properly oriented for stable visual image of the environment

Front 599

What is sensory organization (part of the postural control system)?

Back 599

- three sensory systems used for posture and balance
---visual – critical for development of balance and head control in first 3 years
--- vestibular – head lifting related to maintaining correct orientation of head to gravity and maintaining stable visual image, even when body or head is moving; eyes must dissociate from head
--- somatosensory- combination of touch and proprioception body receives from being in contact with support surface; used for postural response; not used for balance until middle to late childhood

Front 600

Describe Nashner’s model of postural control.

Back 600

three common sway strategies seen in quiet standing:
- ankle strategy –sways backward, anterior tibialis fires; sways forward, gastrocnemius fires; seen as early as 18 mos, but response time is longer than that of adults
- hip strategy – narrower BOS (like standing crosswise on balance beam); ankle strategy not effective because whole foot is not in contact with support surface; muscles fire proximal to distal
- stepping strategy – (a.k.a., LE protective reaction) balance disturbance strong and/or fast enough to force individual to take a step to avoid LOB or fall

adult sway strategies expected to be present in 7-10 year old children (likely because nervous system commonly appears to be mature in myelination of major tracts by age 10)

Front 601

Which system appears to be the dominant sensory system for posture and balance during the first three years of life?

Back 601

visual system

Front 602

Ambulation potential for children with MMC: thoracic

Back 602

- preambulation orthosis – standing frame
- ambulation orthosis – parapodium,/swivel rocker, RGO
- age – 12-18 months, 24-36 months
- assistive device – walker/no device needed, walker crutches
- ambulation potential – children: therapeutic or household ambulation; adolescents/adults: W/C independent

Front 603

Ambulation potential for children with MMC: L1-L2

Back 603

- preambulation orthosis – standing frame
- ambulation orthosis – parapodium, RGO
- age – 12-18 months, 24-36 months
- assistive device – walker, walker crutches
- ambulation potential – children: household or community ambulation; adolescents/adults: W/C independent

Front 604

Ambulation potential for children with MMC: L3-L4

Back 604

- preambulation orthosis – None
- ambulation orthosis – RGO/HKAFO, KAFO/AFO
- age – 12-18 months, 24-36 months
- assistive device – walker, walker crutches
- ambulation potential – children: household or community, adolescents/adults: variable; household or community if quadriceps grade 4 or 5

Front 605

Ambulation potential for children with MMC: L5

Back 605

- preambulation orthosis – none
- ambulation orthosis – KAFO, AFO
- age – 12-18 months, 24-36 months
- assistive device – none
- ambulation potential – children: household or community; adolescents/adults: household or community

Front 606

Ambulation potential for children with MMC: S1-S3

Back 606

- preambulation orthosis – none
- ambulation orthosis – AFO/FO, none
- age – 12-18 months
- assistive device – none
- ambulation potential – children: community; adolescents/adults: community