Cerebral palsy

Background

• Leading cause of childhood disability effecting function and development.
• Defined as a group of disorders involving motor or postural abnormalities that are noted during early development
  • Injury occurs during fetal or neonatal period up to 3 yrs. Official diagnosis may not be made until 3 years of age
• Associated with
  • Birth: hypoxia, placental abruption, prolonged labor, uterine rupture
  • Child: jaundice/kernicterus, low-birth weight, prematurity
  • Mother: infection (prenatal), infertility treatment, multiple births, thyroid disorder, seizure disorder

Classification

• Spastic cerebral palsy is the most common type, ~80% of cases

Spastic diplegia (30-40%)

• Bilateral lower extremities effected more than upper extremities or sole involvement of lower extremities.

Spastic hemiplegia (20-30%)

• One side of the body effected, including an upper and lower extremity
• Upper extremity spasticity effected more than lower extremity
  • Double hemiplegia: when both arms are more involved than the legs

Spastic quadriplegia (10-15%)

• Cerebral palsy affecting all 4 extremities and the trunk (full body)

Dyskinetic cerebral palsy

• Athetoid, choreoathetoid, and dystonic
• Extrapyramidal signs characterized by abnormal movements; hypertonicity

Mixed cerebral palsy

• Mixture of spastic and dyskinetic components without predominant area

Hypotonic cerebral palsy

• Rare: Truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes

Monoplegia

• Rare: One limb involved, either an arm or a leg
• Must rule out other causes.

Clinical Features

• Abnormal muscle tone (most common)
  • Hypertonia > hypotonia
• Mental retardation (30-50%)
• Speech and language impairment
• Ophthalmologic impairment
• Hearing impairment
• Oromotor dysfunction
• Epilepsy
• Disease classified according to resting tone and limb involvement

Differential Diagnosis

Weakness

• Neuromuscular weakness
  • UMN:
    • CVA (Main)
    • Hemorrhagic stroke
  • Spinal cord disease:
    • Infection (Epidural Abscess (Spinal))
    • Infarction/ischemia
    • Trauma (Spinal Cord Syndromes)
    • Inflammation (Transverse Myelitis)
    • Tumor
  • Peripheral nerve disease:
    • Guillain-Barre syndrome
    • Tick paralysis
  • NMJ disease:
    • Botulism
    • Organophosphate toxicity
    • Lambert-Eaton myasthenic syndrome
  • Muscle disease:
    • Rhabdomyolysis
• Non-neuromuscular weakness
  • Can't miss diagnoses:
    • severe infection/Sepsis (Main)
    • Hypoglycemia
    • Periodic paralysis (electrolyte disturbance, K, Mg, Ca)
      • Hypokalemic periodic paralysis
      • Thyrotoxic periodic paralysis
    • Respiratory failure
  • Emergent Diagnoses:
    • Symptomatic Anemia
    • Severe dehydration
    • Hypothyroidism
    • Malignancy

Diagnosis

• R/o Baclofen withdrawal in patient being treated for spasticity with Baclofen pump.