Harvey's Garden

Cerebral palsy

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2 min read

Epidemiology

  • Most common motor disability in children

Etiology

  • Prematurity (< 32 wks): Single greatest risk factor.
  • Very low birth weight (< 1500 g).
  • Periventricular leukomalacia (PVL), IVH, chorioamnionitis, neonatal kernicterus.

Pathophysiology

Spastic cerebral palsy may be caused by white matter necrosis (seen in this patient as periventricular leukomalacia), which leads to a loss of descending inhibitory control from the upper motor neurons. This results in muscle overactivity, leading to increased tone and hyperreflexia.

Clinical features

  • Core Hallmark: Static, non-progressive motor and tone dysfunction.
  • Early signs: Hand preference < 1 yo, delayed motor milestones, persistent primitive reflexes (e.g., Moro > 6 mo).
  • Spastic Diplegia (most common):
    • Bilateral LE > UE; “scissor gait”, toe-walking.
    • UMN signs: Hyperreflexia, sustained clonus, (+) Babinski.
  • Dyskinetic: Choreoathetosis and dystonia secondary to basal ganglia injury (kernicterus).
  • Associated: Seizures (~50%), intellectual disability (~50%), strabismus, dysphagia.

Warning

Definite hand preference before 1 year of age suggests one-sided muscle weakness and is a red flag for hemiplegia.

Diagnostics

  • Initial / Screening:
    • Clinical evaluation: Serial neurodevelopmental examinations demonstrating non-progressive motor deficits, tone abnormalities, and persistent primitive reflexes.
  • Confirmatory / Neuroimaging:
    • Brain MRI (neuroimaging of choice): Identifies underlying structural etiology (e.g., periventricular leukomalacia, focal ischemic stroke, cerebral dysgenesis).
  • Key Labs & Adjuncts:
    • Metabolic/Genetic testing: Indicated if MRI is normal, atypical features are present, or signs of disease progression/neuroregression appear.
    • EEG: If clinical seizures or altered sensorium suspected.
    • Vision and Audiologic Screening: Indicated in all newly diagnosed pts.

Differential Diagnostics

  • Spinal Muscular Atrophy (SMA):
    • Diff by progressive Lower Motor Neuron (LMN) signs (hypotonia, diffuse muscle weakness, tongue fasciculations, absent deep tendon reflexes [DTRs]); CP displays UMN signs/hyperreflexia.
  • Inborn Errors of Metabolism / Leukodystrophies (e.g., Krabbe, Metachromatic Leukodystrophy):
    • Diff by neuroregression (loss of previously acquired milestones) and progressive neurodegeneration; CP is static and non-progressive.
  • Hereditary Spastic Paraplegia:
    • Diff by positive family history, later onset (childhood/adolescence), and progressive bilateral LE spasticity.
  • Duchenne Muscular Dystrophy (DMD):
    • Diff by elevated serum CK, calf pseudohypertrophy, Gowers sign, and progressive proximal muscle weakness without spasticity.

Treatment

  • First-line: Multidisciplinary PT/OT, ankle-foot orthoses (AFOs).
  • Spasticity Pharmacotherapy:
    • Oral: Baclofen or Tizanidine (generalized).
    • Local: Botulinum toxin injections (focal dynamic contractures).
    • Refractory: Intrathecal baclofen pump, Selective Dorsal Rhizotomy.
  • Orthopedic Surgery: Tendon lengthening, hip reconstruction for fixed contractures.
  • Supportive: Anticonvulsants for epilepsy, G-tube for severe dysphagia/FTT.

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