Ped Neuro Rehab Exam 2- Lecture Six (Myelodysplasia)

Front 1

Spina bifida

Back 1

Common name for a number of neural tube defects caused by problems with early development of the spine.

Abnormal opening exists along the vertebral column leaving the SC unprotected and vulnerable to mechanical injury or infection.

Front 2

Incidience of SB

Back 2

1500 babies born annually in US.

Hispanic women have higher rate compared to non-hispanic caucasion compared to non-Hispanic black

Front 3

SB causes:

Back 3

Cause unknown, genetics may play a role.
Nutritional and environmental factors contribute.
Certain drugs increase risk, like valproic acid.
Mothers who are obese before becoming pregnant are at an increased risk.

Front 4

Risk of recurrence after having one child with SB increases __%; after having 2 with SB, __%.

Back 4

2-3; 10

Front 5

Screening for SB

Back 5

Blood test: Maternal Serum Alpha Protein (MSAP) if increased in blood suggests a neural tube defect. Will then do an US.

Front 6

Maternal use of vitamin B-folic acid supplement reduces risk of neural tube defect by __-__%

Back 6

50-70

Front 7

When must folic acid intake be initiated and when can it be terminated?

Back 7

Initiated before conception, terminated 12 weeks gestation.

Front 8

Where can you find folic acid

Back 8

vitamins, greens, eggs, citrus fruit, spinach, etc.

Front 9

How much folic acid should women of childbearing age capable of becoming pregnant take?

Back 9

0.4mg

Front 10

How much folic acid should a woman that has a child with neural tube defect take daily?

Back 10

4 mg

Front 11

Aencephaly

Back 11

didn't fuse closer to the brain

Front 12

Types of Myelodysplasia

Back 12

Spina bifida occulta
Spina bifida aperta/cystica

Front 13

Spina bifida occulta

Back 13

Hidden.
May be an opening in the vertebra, but not a protruding sac. Neural tube is closed.
Don't have neurologic problems.
Entire defect may be covered with skin.
Skin depression or dimple, dark tufts of hair, birthmark.

Front 14

Problems that may occur in spina bifida occulta:

Back 14

Abnormal adhesion of spinal cord to area of malformation; may have problems with gait, foot weakness, and possibly bowel and bladder control.

May have tethered SC neural tracts at end adhere to area of malformation/surrounding structures and may stretch during periods of growth and be painful.

Front 15

Spina bifida aperta/cystica

Back 15

Visible or open fluid-filled sac protruding out of an opening.

Front 16

Types of spina bifida aperta/cystica

Back 16

Meningocele
Myelomeningocele

Front 17

Menigocele

Back 17

Skin covered
Initially associated with no paralysis
Meninges are involved, but the sac doesn't include neural tissue.
Depending on location, easily repaired by surgery.
Risk of rupture and infection.
Possible bowel/bladder problems.

Front 18

Myelomeningocele

Back 18

Open spinal cord defect protrudes dorsally, spans 3-6 verebrae; usually not skin covered.
This is usually more severe.

Whatever is outside the vertebrae is no longer functioning and lose function of everything below.

Associated with spinal N paralysis.
Meninges and some part of actual SC and/0r nerves are involved.

Easily ruptured increasing the risk of meningeal infection.

Severity related to amount of neural tissue involved.

usually all have bowel/bladder problems.

Accompanied by degrees of paralysis of legs, clubfoot, joint deformities, hip dysplasia.

Front 19

Related medical conditions:

Back 19

Arnold-Chiari malformation
Hydrocephalus
Orthopedic problems like clubfoot and scoliosis.
Upper limb dyscoordination/overuse injuries
Bladder problems- repeated infections can cause kidney damage.
Sensory deficits related to abnormal neurological control.
Allergic reactions to latex.

Front 20

Arnold-Chiari malformation

Back 20

part of cerebellum protruding through foramen magnum and causes a blockage and don't have CSF flowing through.
This occurs with hydrocephalus.
Typically treated via surgery to increase room.

Front 21

Hydrocephalus

Back 21

Water on the brain.
Myelomeningocele- 80-85% chance a shunt will be needed.

Untreated- pressure on the brain. May have downward gaze, separation of the sutures with possible bulging.

Usually non-progressive.

Need shunt revision as they grow.

Front 22

Problem with the shunt presents as:

Back 22

nausea, vomiting, headache, sleepiness, coma, irritability, mood changes, changes in eyes, and/or trouble walking or any neurologic sign.

Front 23

Vision and myelodysplasia

Back 23

Problems with visual perception.

May need eye patching for muscle imbalances.

Front 24

Language and myelodysplasia

Back 24

May have high frequency of irrelevant utterances.

Poorer performance with abstract language.

Better with concrete language skills.

Front 25

Gross motor ability in myelodysplasia

Back 25

Depends on level of lesion.
Lowest intact neuromuscular segment.
Left may be different than right.

Front 26

Complete myelodysplasia

Back 26

normal function down to a particular level with flaccid paralysis, sensation loss, and absent reflexes below.

Front 27

Incomplete myelodysplasia

Back 27

Mixed level of involvement with some stiffness and some volitional control.

Front 28

Fine motor effects of myelodysplasia

Back 28

Upper limb dyscoordination (hydrocephalus).
Use visual feedback rather than kinesthetic.
Often use arms as primary mobility.
Often have delayed development of arm dominance.

Front 29

Fewer than __% develop voluntary bowel/bladder control.

Back 29

5

Front 30

Social emotional factors in myelodysplasia

Back 30

Child may have poorly developed self-concept and low self-esteem.
Child may have poor coping skills and social skills.

Front 31

What cognitive difficulties are associated with myelodysplasia?

Back 31

Children without hydrocephalus or with uncomplicated hydrocephalus will have normal intelligence.

Articulate with good verbal skills most of the time.

Learning disabilities

Cocktail Party personality- associated behavior disorder. Superficial chit chat with everyone all the time. Often seen in children with hydrocephalus.

Front 32

Fitness and Health performance and myelodysplasia

Back 32

Obesity
Skin breakdown- especially around orthotics
Osteoporosis
Check the scar occasionally.

Front 33

Parapodium

Back 33

Base that stands them and they're strapped in. Can put a walker in front of them and they swivel their body to help them walk. Used with preschool age.

Front 34

Prognosis in myelodysplasia

Back 34

Dependent on location and severity of lesion and presence of associated problems. The more proximal, the more severe.

Front 35

Poorer prognosis:

Back 35

Complete paralysis
Serious meningitis
Hydrocephalus

Front 36

Lifespan

Back 36

Most children live into adulthood with medical care.

Front 37

Spina bifida occulta prognosis

Back 37

Most asymptomatic and lack neurologic signs.
Usually no consequences.

Front 38

Myelomeningocele prognosis

Back 38

Mortality rate 10-15%
70% have no cognitive impairments.
Seizures are more common.
Learning disabilities are more common.

Front 39

Walking (def)

Back 39

complete ambulatory function with or without braces or assistive devices.

Front 40

Walking time: High lumbar (L1-2)

Back 40

5 y 2 mo

Front 41

Walking time Mid-lumbar (L3)

Back 41

5 y

Front 42

Walking time Low lumbar (L4-5)

Back 42

3 y 10 mo

Front 43

Walking time sacral

Back 43

2 y 2 mo

Front 44

Non-ambulation is correlated with

Back 44

Increased time and energy expenditure it takes.
Weight gain
Rapid growth requiring frequent fitting of braces and equipment.
SC tethering

Front 45

Adapted activities and environment

Back 45

Oral vs. written assignments
Hitting vs. kicking a ball
Visual memory schedules
Objects placed lower for reach

Front 46

Typical interventions

Back 46

Monitor health
Activities to maintain strength, endurance, and mobility
Promote standing and sitting balance
Gait training with assistive devices as necessary.
Transfer training
Adapt activities and environments to facilitate child's participation.
Promote independent self-care skills with adaptive equipment if necessary.
Work on development of keyboarding skills.
Activities to increase self-esteem, confidence, coping skills and decrease depression.
Toilet training

Front 47

Ground reaction force AFO

Back 47

plastic part of AFO on anterior side instead so they lean forward

Front 48

RGO parapodium

Back 48

child has some hip flexion and the parapodium will swing your leg forward for you. Can lock them into flexion if use with TLHKFO.

Front 49

T10 Musculature

Back 49

Lower trunk musculature is weak.

Front 50

T10 Appearance

Back 50

Strong upper limbs and thoracic and neck motions.
Difficult with supported sitting balance and may have decreased respiratory function.

Front 51

T12 Musculature

Back 51

Abs and paraspinals may provide some pelvic control.
Quadratus lumborum.

Front 52

T12 Appearance

Back 52

Good sitting balance
Weak hip hiking
Ambulation may be possible with parapodium.

Front 53

L1-2 Musculature

Back 53

L1- Weak iliopsoas (gr 2)
L2- Iliopsoas, sartorius, hip adductors (gr 3 or better)

Front 54

L1-2 Appearance

Back 54

Weak hip movements.
Short-distance household walking possible using KAFOs or RGOs and upper limb support.
Wheelchair for community distances.

Front 55

L3 Musculature

Back 55

Add quads (gr 3 or better)

Front 56

L3 Appearance

Back 56

Usually require KAFOs and forearm crutches to ambulate.
Wheelchair for community distances.
60% achieve independent living

Front 57

L4 Musculature

Back 57

Medial hamstrings or tibialis anterior (gr 3 or better)
May have a weak peroneus tertius.

Front 58

L4 Appearance

Back 58

Calcaneal foot deformities are common.
Usually functional ambulators with AFOs and forearm crutches.
20% continue to ambulate as adults.

Front 59

L5 Musculature

Back 59

Lateral hamstrings gr three or better plus one of the following:
Glut med gr 2 or better, tibialis posterior gr 3 or better, or peroneus tertius gr 4 or better

Front 60

L5 Appearance

Back 60

Hindfoot valgus deformities
Able to ambulate w/o orthoses, but requires them to correct foot position and substitute for lack of push-off.
Gluteal lurch present without upper limb support.
80% achieve independent living

Front 61

S1 Musculature

Back 61

L5 plus at least 2 of the following: Triceps surae gr 2, glut max gr 2, glut med gr3.

Front 62

S1 Appearance

Back 62

Improved hip stability
Can ambulate without orthoses or upper limb support.
Weak push-off when running or stair climbing.
Mild-moderate gluteal lurch.
Toe musculature strong, foot deformities less common.

Front 63

S2 Musculature

Back 63

Triceps surae gr 3, gluts gr 4

Front 64

S2 Appearance

Back 64

Decreased push-off and stride length.

Front 65

No loss

Back 65

Bowel and bladder function normally and lower limb strength is normal.