Joyce Visitacion NCM 104

BSN – 4AJ 1st Semester 2008-2009

CONGENITAL NEUROLOGICAL DISEASE

NEURAL TUBE DEFECTS

• Closure of the neural tube occurs during the 3rd and 4th week
AOG.
• RISK FACTORS:
- Maternal malnutrition
- Drug exposure (Teratogens)
- Low socio-economic group
- Older maternal age

CLINICAL MANIFESTATIONS:
• Anencephaly: large skull defect with no cortex; many are
still born and others die within days of birth.
• Encephaloceles: projections of the cranial content thru a
bony skull defect, usually occipital region.
- Severe mental retardations
- Seizure
- Movement disorder

NURSING INTERVENTIONS (SEIZURE):

1) Main function of the nurse: look for the safety of the
patient.
2) If the mouth is open (insert a tongue depressor to avoid
biting the tongue)
3) If the mouth is closed (do not insert a tongue depressor)
4) Medications:
• Phenytoin (Dilantin)
• Phenobarbital (Luminal)
• Diazepam (Valium)
5) Administer oxygen (hospital setting)

SPINA BIFIDA
• CNS defect that occurs as a result of incomplete closure of one
or more vertebrae.

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2 TYPES OF SPINA BIFIDA:
1. Spina Bifida Occulta
• Most common and less severe.
• Defect in the vertebral arches without herniation of any spinal
content.
• Birthmarks, dimples or hairy tuft at the base of the back.
• Meninges are not usually exposed on skin surface.

2. Spina Bifida Cystica

• Composed of Spina bifida with Meningocele and Spina bifida with
Meningomyelocele.

Spina bifida with meningocele

 Defect in vertebral arches with protrusion of meninges
only.
 Usually in the lumbosacral area.
 No involvement of spinal cord.

Spina bifida with meningomyelocele

 Defect of vertebral arches with protrusion of
meninges, spinal cord, CSF and nerve roots.
 Can have:
a) Knee contractures
b) Hydrocephalus
c) Hormone and Neurologic problems
d) Paralysis and bowel / bladder incontinence.

***Spina Bifida Occulta and Spina Bifida Meningocele can have

bowel / bladder dysfunction.

Diagnostic Factor:
• Alpha- Fetoprotein (AFP) at 16-18 weeks AOG as
screening factor for those high risk.

DIAGNOSTIC TESTS:
a) Amniocentesis presence of increase alpha-
fetoprotein
b) Maternal Blood Test

Trisomy 21 – decrease alpha-fetoprotein

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TREATMENT:
a) Spina bifida occulta – no treatment
b) Spina bifida meningocele – surgical closure
c) Spina bifida meningomyelocele – repair of the sac or cystic
lesion

MOST OF THESE CLINICAL MANIFESTATIONS ARE SEEN IN

SPINA BIFIDA MENINGOMYELOCELE:
1) Depends on the spinal cord involvement.
2) Visible spinal defect.
3) Flaccid paralysis of the legs.
4) Altered bladder and bowel function.
5) Hip and joint deformities.

NURSING MANAGEMENT:
1) Evaluate sac and measure lesion.
2) Perform neurological assessment.
3) Monitor increase in ICP.
4) Protect sac using aseptic technique (put dressing, cover with
sterile gauze soak in Normal Saline)
5) Place in prone position, head turned to one side for
feeding.
6) Assess for early signs of infection.
7) Administer medication as ordered (antibiotics,
anticholinergics, laxatives, anti-spasmodics)

COMMON NEUROLOGICAL HEALTH PROBLEMS AMONG

CHILDREN AND ADOLESCENTS:

CEREBRAL PALSY
• Main problem: absence of oxygen to the brain (Anoxia)
• Non- progressive disorder of movement and posture that
results from tension of the immature brain.
• Risk factors:
- Pre-maturity
- Birth asphyxia (suffocation / presence of
foreign substances in the airway)
- Early infection or trauma

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 -IUGR (Intra-Uterine Growth Retardation)
-Most cases occur in the absence of identifiable risk
factors.
TYPES OF CEREBRAL PALSY:
1) Pyramidal (spastic)
• Most common
• Tense, contracted muscles

2) Athetoid
• Constant, uncontrolled motion of limbs, head and
eyes.

3) Ataxic
• Poor sense of balance often causing falls and
stumbles.

4) Rigidity
• Tight muscles that resist effort to make them move.

5) Tremor
• Uncontrollable shaking, interfering with coordination

***50% of patients with Cerebral Palsy are average or above

intelligence.

CLINICAL MANIFESTATIONS:
• Abnormal muscle tone. Common clinical manifestations
• Abnormal coordination.
• Tones remain relatively constant regardless of activity and
level of arousal.
• Clasp knife rigidity (resistance to applied force with
sudden or give alternating with increased resistance)
• Significant hyperreflexia.
• Stiff and rigid arms or legs.
• Persistent of primitive and pathologic reflexes.
• Extreme irritability and crying.
• Feeding difficulties.
• Delayed gross development.
• Opisthotonus posture (abnormal arching of the back)

***If you carry a baby:

 Normal reaction: the baby does bicycling of the legs.
 Abnormal: the baby crosses his legs.

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 MANAGEMENT:
• Multidisciplinary approach.
• Therapeutic goal includes minimizing impairments,
maximizing function, preserving general health.

NURSING MANAGEMENT:
• Assess child’s development level and intelligence.
• Encourage early intervention and participation in school
programs.
• Prepare fro using mobilizing devices to prevent deformities.
• Communicate with the child on a functioning level, not
chronological age level.
• Provide safe environment.
• Provide safe, appropriate toys for age and developmental age.
• Position upright after meals.

REYE’S SYNDROME
• A syndrome of encephalopathy (brain disease) with associated
liver degeneration.
• Unknown cause, but can be associated with viral agents such as
influenza or varicella, toxins, aspirin and other salicylates and
metabolic defects.
• Reye’s Syndrome = infection + utilization of Aspirin
• Happens 1-3 days after infection
• Problem: infiltration of fats in the brain, heart and lungs.

CLINICAL MANIFESTATIONS:
Stage I: Sudden onset of persistent vomiting, fatigue.
Stage II: Behavior changes, disorientation, confusion,
hyperreflexia.
Stage III: Coma, decorticate posturing.
Stage IV: Deeper coma, decerebrate posturing.
Stage V: Seizures, absent deep tendon and respiratory
reflexes, flaccid paralysis.

***All of these stages have Hepatic Dysfunctions

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DIAGNOSTIC TESTS:
1. Elevated liver enzymes (indicative of Reye’s Syndrome)
Aspartate Aminotransferase (AST), Alanine Transaminase
(ALT): Normal = 8-20 unit/l

2. Elevated serum ammonia test.

GOAL OF TREATMENT:
1. Maintain effective cerebral perfusion and prevent increase
ICP.

SURGERY:
• Decompressive Craniotomy (surgery to decrease
ICP)

MEDICATION OF CHOICE:
• Acetaminophen (Tylenol)

NURSING MANAGEMENT:
1. Assess neurological status.
2. Monitor for altered level of consciousness and
increased ICP.
3. Monitor I and O.
4. Provide rest and decrease environmental
stimulation.
5. Monitor for signs of bleeding.