Chapter 40

The Child with Neuromuscular or

Muscular Dysfunction
Neurological Dysfunction

 Neurologic alterations seen in infants and children

related to structural causes, infections, or injuries
 Either acquired or congenital
 Several factors predispose a child to alterations in
neurological function

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Cerebral Palsy (CP)

 Characterized by early onset and impaired movement

and posture
 Incidence 2.4 to 3.6 per 1000 live births
 Most common permanent physical disability in childhood

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CP—Etiology

 Any perinatal or neonatal brain lesion or brain

maldevelopment, regardless of the cause, may be
linked to as many as 80% of the total cases of CP
 Prenatal/postnatal Infection
 Prenatal/postnatal hypoxia/ asphyxia
 Often no identifiable immediate cause

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Possible Motor Signs of CP

 Poor head control after age 3 months

 Stiff or rigid limbs
 Arching back/pushing away
 Floppy tone
 Unable to sit without support at age 8 months
 Clenched fists after age 3 months
 Excessive irritability
 No smiling by age 3 months
 Feeding difficulties
 Persistent tongue thrusting
 Frequent gagging or choking with feeds

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Cerebral Palsy and IQ

 Wide variation
 50%-60% of CP patients have normal IQ
 Difficult to assess
 Rigid, atonic, and quadriparetic CP have highest
incidence of profound impairment

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Goals of Therapy for CP

 Establish locomotion, communication, and self-help

 Gain optimum integration of motor functions
 Correct associated defects as early and effectively as
possible
 Provide educational opportunities
 Promote socialization experiences

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Pharmacologic to Decrease
Spasticity in CP
 Botulinum toxin type A (Botox) to control spasticity
 Baclofen
 Oral
 Implanted pump for intrathecal administration
 Dantrolene sodium (Dantrium)
 Diazepam (Valium)
 Zantac

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Botulism

 Food poisoning resulting from ingestion of toxin

produced by the anerobic bacillus Clostridium
botulinum
 Sources
 Improperly sterilized home-canned foods for older children
 Infant sources—honey and light or dark corn syrup

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Clinical Manifestations of
Botulism
 CNS symptoms appear abruptly 12 to 36 hours after
ingestion
 General signs
 Weakness, dizziness, headache, diplopia, speech
difficulties
 Vomiting
 Progressive, life-threatening respiratory paralysis

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Treatment of Botulism

 IV botulism immune globulin (BIG-IV [BabyBIG]) to treat

infants
 Trivalent equine botulinum antitoxin and bivalent
antitoxin to treat older children
 Supportive measures—respiratory support
 Continue therapy until paralysis abates

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Infant Botulism

 Caused by ingestion of spores or vegetative cells of C.

botulinum and subsequent release of toxin
 Source of botulism in infants—honey, and light or dark
corn syrup fed to them
 Wide variation in severity of disease—mild constipation
to respiratory failure

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Infant Botulism—cont’d

 Constipation is often the presenting symptom

 Generalized weakness, decreased movement,
diminished deep tendon reflexes
 Loss of head control, feeding difficulty, weak cry,
diminished gag reflex, hypotonia
 Do not treat infants with botulism antitoxin
 Prognosis generally good but very slow (weeks to
months)

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Spina Bifida
Neural Tube Defects (NTDs)

 Failed closure of neural tube

 May involve the entire length of the neural tube or a small portion
 Incidence
 Affects more girls than boys
 Occurs three times more often in Caucasians and Hispanics than in African
Americans
 One of the most common birth defects in the United States

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Prenatal Management of NTD
 Treatment and prevention
 Supplementation with folic acid 0.4 mg/day
 If there is a history of NTD, 4 mg/day
 In 1998, the Food and Drug Administration (FDA) fortified cereal grains with folic
acid
 Begin preconception
 Prenatal and antenatal assessment
 Antenatal surgical intervention

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Antenatal Diagnosis of NTD
 Elevated α-fetoprotein (AFP) in amniotic fluid; tested at 6 to 18 weeks of
gestation
 Uterine ultrasound
 Genetic counseling

 Why do we want to know?

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NTDs

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Spina Bifida
 Failure of the osseous spine to close
 Two types
 Spina bifida occulta
 Not visible externally

 Spina bifida cystica

 Visible defect
 Saclike protrusion

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Spina Bifida Occulta
 Usually lumbosacral, at L5 to S1
 Skin indicators (absent, singly or combination)
 Sacral dimple
 Sacral angioma or port wine nevus
 Sacral tufts of dark hair
 Sacral lipoma

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Spina Bifida Occulta (cont’d)
 Tethered cord
 Abnormal adhesion to a bony or fixed structure
 Puts traction on the cord
 Altered gait
 Bowel and bladder problems
 Foot deformities
 May not be seen in early infancy

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Diagnostic Tests for Spina
Bifida Occulta
 X-ray
 MRI
 CT
 Ultrasonography

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Spina Bifida Cystica
 Visible defect with external saclike protrusion
 Two types
 Meningocele
 Myelomeningocele

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Meningocele
 Sac contains meninges and spinal fluid but no neural elements
 No neurologic deficits

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Myelomeningocele
 Neural tube fails to close
 May be anywhere along the spinal column
 Lumbar and lumbosacral areas are the most common
 May be diagnosed prenatally or at birth
 Sac contains meninges, spinal fluid, and nerves
 Varying and serious degrees of neurologic deficit
 Sac prone to CS fluid leakage

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Myelomeningocele

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Myelomeningocele (cont’d)
 Location and magnitude of the defect determine the nature and extent of
impairment
 If the defect is below the second lumbar vertebra
 Flaccid paralysis of lower extremities
 Sensory deficit
 Not necessarily uniform on both sides of the defect

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Initial Management of
Myelomeningocele
 Multidisciplinary team approach
 Intensive nursing care
 Prevent infection
 Assess neurologic and associated anomalies
 DDH, clubfoot, genitourinary deformity
 Early closure in 12 to 72 hours after birth
 Prevent stretching of other nerve roots and further damage
 Promote family bonding

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Ongoing management of NTD
 Postoperative care and follow-up
 Orthopedic considerations
 Genitourinary function
 Bowel function and control
 Family support and education
 Plan for home care routine
 Prevent complications
 Assess for latex allergy

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Latex Allergy
 Identified as a serious health hazard to children with spina bifida
 Spina bifida patients are at high risk for latex allergy due to repeated
exposure to latex products from multiple surgeries and repeated urinary
catheterizations and multiple surgeries

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Allergic Reactions to Latex
 Range from urticaria, wheezing, and rash to anaphylaxis
 Reactions tend to increase in severity when latex comes in contact with the
mucous membranes, wet skin, bloodstream, or airway
 Cross-reactions with foods: Banana, avocado, kiwi, chestnuts

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Goals Related to Latex Allergy
 Reduce exposure to latex for spina bifida patients who are at risk of
developing the allergy
 Remain alert for development of the allergy
 Create a latex-safe environment for allergic individuals

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