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32 Cards in this Set

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ETIOLOGY

___ deficiency
Teratogens
Genetic component
Folic acid
DIAGNOSIS

Alpha-fetoprotein
–___ used test
–MSAFP blood test
–___weeks gestation

Fetal Ultrasonography
–Primary screening tool for NTD
–18 weeks gestation

Amniocentesis
–20 weeks

MRI
Most commonly

16 weeks gestation
EMBRYOLOGY

NEURAL TUBE CLOSURE
FAILURE TO CLOSE
ok
CATEGORIES (3)

SPINA BIFIDA ___
___CELE
___MENINGOCELE
SPINA BIFIDA OCCULTA - Most common

MENINGOCELE - Least common

MYELOMENINGOCELE - Second most common
SPINA BIFIDA OCCULTA

___ common, least severe
___ do not fuse
Lumbar or sacral spine
Spinal cord, nerves not damaged
Dimpling or ___
Most common

Vertebral arches do not fuse

tuft of hair
MENINGOCELE

___ common, second in severity
Vertebral arches do not fuse
Spinal cord intact
___ in skin covered sac
Paresis, bowel/ bladder ___ possible
Least common

Neural elements

Paresis, bowel/ bladder incontinence possible
MYELOMENINGOCELE

Second most common, ___

Vertebral arches do not fuse

Open defect or membranous sac

Sac contains CSF, meninges, spinal cord

Spinal cord ___ below sac

___, ___ loss, bowel/ bladder dysfunction

Usually lumbar

Usually bilateral but may be unilateral or asymmetrical
most severe

Spinal cord damaged below sac

Sensory, motor loss, bowel/ bladder dysfunction
LEVEL IDENTIFICATION

Motor level is assigned according to last intact ___ found
nerve root
ASSOCIATED CNS ABNORMALITIES

Arnold Chiari Malformation
Chiari malformations (CMs) are structural defects in the cerebellum, the part of the brain that controls balance. When the indented bony space at the lower rear of the skull is smaller than normal, the cerebellum and brainstem can be pushed downward. The resulting pressure on the cerebellum can block the flow of cerebrospinal fluid (the liquid that surrounds and protects the brain and spinal cord) and can cause a range of symptoms including dizziness, muscle weakness, numbness, vision problems, headache, and problems with balance and coordination. There are three primary types of CM. The most common is Type I, which may not cause symptoms and is often found by accident during an examination for another condition. Type II (also called Arnold-Chiari malformation) is usually accompanied by a myelomeningocele-a form of spina bifida that occurs when the spinal canal and backbone do not close before birth, causing the spinal cord to protrude through an opening in the back. This can cause partial or complete paralysis below the spinal opening. Type III is the most serious form of CM, and causes severe neurological defects. Other conditions sometimes associated with CM include hydrocephalus, syringomyelia, and spinal curvature.

NINDS Chiari Malformation Information Page
CLINICAL SYMPTOMS

Stridor

Apnea

GERD

Vocal cord paralysis
–Gargled voice

Dysphagia

Seizures

Nystagmus

Problems with feeding
–aspiration

Ataxia

Hypotonia

UE weakness
ok
SECONDARY DYSFUNCTION

___ deficit
Decreased coordination
Decreased motor planning
Postural insecurity
Balance
ASSOCIATED CNS ABNORMALITIES

Hydrocephalus
Hydrocephalus – head gets enlarged, shunt in 4th ventricle to peritoneum and gets absorbed into the body – furl it up, and as child growths it unfolds.
CLINICAL SIGNS

Head enlargement
Irritability
___ sign of eyes; downward deviation
Projectile vomiting
Sunset sign of eyes
SECONDARY DYSFUNCTIONS

Visual motor perceptual problems
Cognitive deficits
“Cocktail party” conversation; ___ IQ > performance IQ
verbal IQ > performance IQ
SIGNS OF SHUNT MALFUNCTION

___
Bulging fontanelle - an outward curving of an infant's soft spot (fontanelle). In an infant, the space where two sutures join forms a membrane-covered "soft spot" called a fontanelle (fontanel). The fontanelles allow for growth of the skull during an infant's first year.

Vomiting

Change in appetite

Sunset sign of eyes - Setting-sun sign refers to the downward deviation of an infant’s or a young child’s eyes as a result of pressure on cranial nerves III, IV, and VI. With this late and ominous sign of increased intracranial pressure (ICP), both eyes are rotated downward, typically revealing an area of sclera above the irises; occasionally, the irises appear to be forced outward. Pupils are sluggish, responding to light unequally.

Edema, redness along shunt tract
INFANTS
SIGNS OF SHUNT MALFUNCTION

___
–Vomiting
–Irritability
–Headaches
–Edema, redness along shunt tract
TODDLERS
SIGNS OF SHUNT MALFUNCTION
SCHOOL –AGED CHILDREN

Headaches
Lethargy
Irritability
Edema, redness along shunt tract
Handwriting changes
High –pitched cry
Seizures
Memory changes
Rapid growth of ___ circumference
___ of skin over scalp
___ not previously noted
Vomiting
Personality changes
Changes in school performance
head

Thinning of skin over scalp

Nystagmus not previously noted
SKELETAL ABNORMALITIES

___–congenital or acquired
Deformity from muscle imbalance
___–talipes equinovarus
Hip ___
Scoliosis

Clubfoot

Hip dislocation
NEUROLOGICAL DETERIORATION

1. ___ - refers to an abnormal widening of the central canal of the spinal cord that creates a cavity in which cerebrospinal fluid can accumulate. As spinal fluid builds up, it may put abnormal pressure on the spinal cord and damage nerve cells and their connections. ___ is sometimes used interchangeably with syringomyelia, the name for a condition that also involves cavitation in the spinal cord. In ___, the cavity that forms is connected to the fourth ventricle in the brain, and is almost always associated with infants and children who have hydrocephalus or birth defects such as Chiari Malformation II and Dandy-Walker Malformation. Symptoms, which may occur over time, include weakness of the hands, arms, and legs; and sensory loss in the neck and arms. Some individuals have severe pain in the neck and arms. Diagnosis is made by MRI, which reveals abnormalities in the anatomy of the spinal cord; and electromyography
2. ___ - a neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column. These attachments cause an abnormal stretching of the spinal cord. The course of the disorder is progressive. In children, symptoms may include lesions, hairy patches, dimples, or fatty tumors on the lower back; foot and spinal deformities; weakness in the legs; low back pain; scoliosis; and incontinence. ___ syndrome may go undiagnosed until adulthood, when sensory and motor problems and loss of bowel and bladder control emerge. This delayed presentation of symptoms is related to the degree of strain placed on the spinal cord over time. ___ syndrome appears to be the result of improper growth of the neural tube during fetal development, and is closely linked to spina bifida. Tethering may also develop after spinal cord injury and scar tissue can block the flow of fluids around the spinal cord. Fluid pressure may cause cysts to form in the spinal cord, a condition called syringomyelia. This can lead to additional loss of movement, feeling or the onset of pain or autonomic symptoms.
1. Hydromyelia
2. Tethered cord
SIGNS OF HYDROMYELIA

Progressive ___
___ weakness
Increased tone
scoliosis

UE weakness
SIGNS OF TETHERED CORD

Progressive scoliosis
___ weakness
Increased ___
UTI or incontinence
Change in sensation
Pain/ irritability
Foot ___
LE weakness

Increased tone

Foot deformity
RELATED PROBLEMS

Skin breakdown
___ allergy
Obesity
Sensory integration problems
Neurogenic bladder/bowel
Frequent hospitalizations
Decreased:
–Motor planning
–Kinesthetic awareness
–Attention
–Short term memory
–Eye/hand coordination
Latex allergy
RED FLAG

Latex allergy
–___% present
–Watery eyes, itching, hives, rash, anaphylactic shock

Examples:
–Balloons, pacifiers, beach toys, koosh balls, reflex hammers, stethoscope tubing, crutch axillary pads and tips, swim goggles, wheelchair tires

DO NOT USE LATEX PRODUCTS WITH THESE CHILDREN
75% present
CLINICAL PRESENTATION

LEVEL: T6-T12

MUSCLE FUNCTION: Upper trunk, abdominals, back, intercostal

SECONDARY IMPAIRMENTS: Kyphoscoliosis contractures, hip abd/ER, clubfeet

PROGNOSIS FOR WALKING:
TLSO
WHEELCHAIR
STANDING FRAME
WHEELED STANDER
PARAPODIUM
RGO/WALKER
___ AMBULATION
SHORT DISTANCE AMBULATION
CLINICAL PRESENTATION

LEVEL: L1 - L3

MUSCLE FUNCTION: Hip flexors, Hip Adductors, possible quadriceps

SECONDARY IMPAIRMENTS: hip flexion contractures, hip dislocation, wind swept, scoliosis

PROGNOSIS FOR WALKING:
___ AMBULATION
OR EXERCISE AMBULATION ONLY
STANDER
PARAPODIUM
RGO/WALKER
HKAFO, KAFO, AFO
FOREARM CRUTCHES
WHEELCHAIR
HOUSEHOLD AMBULATION
CLINICAL PRESENTATION

LEVEL: L4 - L5

MUSCLE FUNCTION: Quadriceps, gluteus medius, gluteus minimus, hamstrings, AT, PT, peroneals

SECONDARY IMPAIRMENTS: HFC, hip dislocation, lumbar lordosis, calcaneovarus

PROGNOSIS FOR WALKING:
___ OR ___ AMBULATION
HKAFO, KAFO, AFO
CRUTCHES, CANE
WHEELCHAIR
HOUSEHOLD OR COMMUNITY AMBULATION
CLINICAL PRESENTATION

LEVEL: S1-S2

MUSCLE FUNCTION: Quadriceps, gluteus maximus, peroneals, gastroc, toe flexors

SECONDARY IMPAIRMENTS: calcaneovarus, toe clawing, heel ulcers

PROGNOSIS FOR WALKING
___ AMBULATION
AFO, SMO, FO
COMMUNITY AMBULATION
GOALS OF PT

Establish motor level by ___

Provide information to medical team about available ___

Perform periodic MMT for comparison purposes

Provide family instruction regarding positioning, handling to prevent deformity

Provide family with HEP for motor development

Input regarding orthotic and mobility aid

Monitor patient for ___
MMT

LE movement

CNS deterioration
ASSESSMENT

ROM
Postural alignment
Sensory
Muscle tone
Muscle strength
Motor function
Balance, postural control
Skin integrity
ok
FOCUS OF TREATMENT
Infant

Age appropriate developmental skills
Head / trunk control
Postural reactions (protective, equilibrium)
Mobility training
___ for upper extremity strengthening
Adaptive seating or standing (parapodium)
Goal is ___
Prone

upright standing
FOCUS OF TREATMENT
Preschool

Functional upright mobility
___ management
Orthopedic intervention
Skin care
Strengthening
Adaptive training
Orthotic

Preschool – socialize and interact w/ peers – eye level w/ them so stand up
FOCUS OF TREATMENT
School –aged Child

Energy expenditure
Orthopedic intervention
Orthotic management
Home, school, community ___
Skin care and pressure relief
negotiation

School-aged – get around high school, get to the lunch room, manage the lunch tray, go to bathroom. Must be educationally relevant. Usually don’t get pt privately anymore.