Neuro

Cerebral Palsy

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Cerebral Palsy

  • Epidemiology
  1. Incidence: Up to 2.5 per 1000 U.S. born children
  • Pathophysiology
  1. Brain injury occurring before age 3 to 5 years
  2. Results in non-progressive disorder of movement and Posture
  3. Wide variability in disease involvement and in degree of intellectual capacity
  • Causes
  1. Idiopathic in 25% of cases
  2. Prenatal causes or risk factors: Up to 80% of cases
    1. Delivery complications (i.e. asphyxia): 6% of cases
    2. Preterm birth before 32 weeks or <2500 grams
    3. Intrauterine Growth Retardation
    4. Intracranial Hemorrhage
    5. Teratogen Exposure
  3. Postnatal causes (<20%)
    1. CNS Infection
    2. Head Trauma
    3. Hyperbilirubinemia
  • Signs
  1. Limb spasticity (80% of cases)
    1. Hypertonic muscle movement
    2. Hyperreflexia
    3. Scissors gait
    4. Toe-walking
  2. Diskinesia (10-20% of cases)
    1. Slow, writhing limb movements worse during stress
  3. Ataxic Cerebral Palsy (5-10% of cases)
    1. Wide-based gait
    2. Intention Tremor
  • Associated Findings
  1. Cognitive Impairment (66% of cases)
  2. Motor Impairment
    1. Diplegia
    2. Hemiplegia
    3. Quadriplegia
  3. Seizure disorder (50-66% of cases, often spastic type)
  4. Growth Delay
  5. Hearing Impairment
  6. Vision Impairment
    1. Screen for Strabismus and hemianopia
  7. Gastrointestinal disorders
    1. Vomiting (Delayed Gastric Emptying)
    2. Constipation (GI motility, dehydration, mobility)
    3. Risk of Sigmoid Volvulus
  8. Swallowing difficulty
    1. Aspiration risk
    2. Drooling
    3. Inadequate oral intake
  9. Osteoporosis
  10. Urinary Incontinence
  11. Altered sensory perception (pain on light touch)
  • Diagnosis
  1. Assessment Tools
    1. Gross Motor Function Classification System for CP
      1. Palisano (1997) Dev Med Child Neurol 39:214-23 [PubMed]
  2. Findings suggestive of Cerebral Palsy
    1. Slow motor development
    2. Altered muscle tone and Posture
    3. Moro Reflex persists beyond 6 months of age
    4. Dominant hand preference established under 12 months
  3. Findings of alternative diagnosis (neurodegenerative)
    1. Loss of acquired skills
    2. Atypical body odor (as seen in metabolic disorders)
    3. Loss of Deep Tendon Reflexes
  • Differential diagnosis
  1. Consider neurodegenerative disorders
  2. Examples
    1. Arginase deficiency
    2. Glutaric aciduria
    3. Niemann-Pick Disease
    4. Lesch-Nyhan Syndrome
    5. Rett Syndrome
  • Management
  1. Global therapies with variable efficacy
    1. Neurodevelopmental treatment (Bobath method)
    2. Conductive education with rehabilitation program
  2. Physical therapy
    1. Balance benefit with stress of frequent visits
    2. Resistive Exercise is controversial
      1. Has been avoided due to increased spasticity risk
      2. Recent studies suggest strengthening is beneficial
        1. Dodd (2002) Arch Phys Med Rehabil 83:1157-64 [PubMed]
  3. Medications: Spasticity
    1. Botulinum Toxin (Botox) injections for leg spasticity
      1. Reddigough (2002) Dev Med Child Neurol 44:820-7 [PubMed]
    2. Baclofen (Lioresal) intrathecally
      1. See Baclofen Pump
      2. Campbell (2002) Dev Med Child Neurol 44:660-5 [PubMed]
  4. Medications: Pain (especially Hip Pain)
    1. Assess pain and treat adequately
  5. Seizures
    1. Emergent Seizure management is the same as for non-Cerebral Palsy patients
      1. Administer Benzodiazepines and Anticonvulsant loading
      2. Check Serum Glucose and anticonvulsant levels
      3. Other labs and diagnostic evaluation only as indicated by exam and history
    2. Seizures may be refractory to standard measures
      1. Combination antiepileptics are often required for Seizure control
      2. Consider ketogenic diet (risk of Drug Interactions)
  6. Surgery
    1. Leg spasticity
      1. Selective dorsal rhizotomy
        1. Selective cutting of L1-S2 dorsal rootlets
    2. Muscle imbalance with hip subluxation, dislocation
      1. Abduction bracing
      2. Soft tissue release
      3. Femoral or pelvic osteotomy
    3. Implantable stimulator to superior-medial Cerebellum
      1. Davis (2000) Arch Med Res 31:290-9 [PubMed]
    4. Gastrostomy for swallowing and eating difficulties
      1. Samson-Fang (2003) Dev Med Child Neurol 45:415-26 [PubMed]
  7. Adjuncts
    1. Lower limb orthoses (variable evidence)
    2. Elastic body suits (difficult compliance)
  8. General measures
    1. Multidisciplinary approach (care team)
    2. Assistive Devices for Activities of Daily Living
    3. Enable mobility
    4. Address Mood Disorders
  • Prevention
  • Secondary conditions
  1. Observe for cancers of higher risk in Cerebral Palsy
    1. Brain cancer
    2. Breast Cancer
    3. Routine Health Maintenance
      1. Left lateral position more comfortable for pelvic
  2. Educate about injury risks
    1. Drowning risk
    2. Motor Vehicle Accidents
  • References
  1. Majoewsky (2012) EM:Rap 2(9): 4
  2. Krigger (2006) Am Fam Physician 73:91-102 [PubMed]