Peds Neuromuscular

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Peds Neuromuscular
2012-12-01 13:33:47

Pediatric Neuromuscular Disorders
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  1. What is the most common permanent physical disability in childhood?
    • Cerebral Palsy (CP)
    • Nonspecific term
    • Nonprogressive. 
  2. What is the etiology of CP?
    • Whether before or after birth, some kind of sudden interuption of oxygen supply (anoxia). 
    • Preterm birth or ELBW or VLBW most important factors
  3. What is the most common form of CP
    Spastic. Misleading because it presents as hypotonia, ie "floppy babies." 
  4. What are some motor SnSs of CP?
  5. What are some possible behavior SnSs of CP?
    • No smiling by 3mo.
    • Feeding diff, ie excessive tongue thrusting and frequent gagging during feeding. 
  6. T/F CP kids usually have v IQ.
    False. 70% have normal IQ
  7. What is the most common cause of neural tube defects?
    Maternal folic acid deficency during pregnancy-->GET ON THOSE PRENATALS! 4mg/day if Hx of NTD
  8. How do you Dx NTD during pregers?
    ^ AFP in amniotic fluid
  9. In children with anencephaly, what is usually the cause of death?
    Respiratory failure.
  10. What are the two types of Spina Bifida?
    • Occulta: Not externally visible, usually at L5-S1
    • --Can present as port wine nevi, sacral dimple, tuft of hair, or lipoma (lump under skin.)
    • Cystica: Externally visible as sac-like protrusion from small of back.
    • Menigocele: Menegis + SF but not neural elements. Most benign.
    • Myelomeningocele: If at or lower than L2, ^ risk of of paralysis of lower extremities and sensory defecits. Not necessarily bilat. 
  11. How does SB Occulta with "tethered cord" present?
  12. Tx of Myelomeningocele?
    • Prevent nfxn!
    • Early closure within 12-72hrs after birth.
    • Be aware that due to multiple Sx and catheterization, child will be at ^ risk of latex allergy. 
  13. What is Duchenne's Muscular Dystrophy? (DMD)
    • Most common and most severe MD in childhood.
    • X-linked (1:3500 males)
  14. SnSs of DMD?
    • Onset between age 3 and 5 years
    • Progressive, ascending muscle weakness, wasting, and contractures. 
    • Calf muscles hypertrophy in most patients to compensate for lost fnxn of feet.
    • Progressive generalized weakness in adolescence
    • Death from respiratory or cardiac failure as disease ascends.
    • Gower's Sign (roll over and push with arms to stand).
    • Mental deficiencies common. 
  15. Dx of DMD?
    EMG, muscle biopsy, and serum CPK (high in first 2 yrs, then decline as muscles atrophy.)
  16. Tx for DMD?
    • Supportive
    • Maintain mobility and fxn as long as possible.
    • Alternate forms for ADLs
    • Possible Sx for release of contractures
    • Genetic counseling for parents. 
  17. How is GBS different thatn DMD?
    • GBS is:
    • Aquired (usually following nfxn or admin of vaccine), not genetic
    • autoimmune in nature. Inflammation/edema in cranium and spine-->impared nerve fxn--> muscle paralysis in affected areas. 
    • progressive paralysis, not atrophy of muscles
    • Dx based on:
    • EMG
    • LP
    • Symptoms (ie symmetric paralysis)
  18. Tx of BGS?
    • Steroids
    • IV Ig
    • Plasmapheresis
    • RT
  19. Prognosis of GBS?
    • Younger age = better chance of total recovery
    • Most kids have total fnx restored in 2 days - 2 weeks. 
  20. Pathology of Infant Botulism?
    • C.  botulinism
    • As the toxin is absorbed, it irreversibly binds to acetylcholine receptors on motor nerve terminals at neuromuscular junctions-->muscles remain contracted.
    • Prognosis is good with Tx, but generally slow recovery. 
  21. SnSs of Infant Botulism?
    • 12-36hrs after ingestion:
    • --Constipation (usually first symptom and reason for child being brought in)
    • – Weakness (hypotonia), dizziness, headache, diplopia, speech/feeding difficulties, loss of head control
    • --Weak cry
    • --diminished gag rflx
    • – Vomiting
    • – Loss of reflexes
    • – Progressive, life-threatening respiratory paralysis
  22. Tx for infant botulism?
    • antitoxin (Cx'd for infants)
    • C. botulinism Ig
    • RT support until paralysis gone.