Cerebral Palsy Overview Quiz

Cerebral Palsy (Little's Disease)

• Definition: A group of permanent movement and posture disorders causing activity limitations, resulting from non-progressive lesions in the developing fetal or infant brain. Primarily affecting motor centers (cerebral cortex, cerebellum, and basal ganglia). Often accompanied by neurological issues like mental retardation, epilepsy, hearing/vision impairments, and emotional/behavioral disturbances.

Causes

• Prenatal (80%): Factors including antenatal infections, congenital malformations, and fetal asphyxia.
• Natal (10%): Birth asphyxia and birth trauma.
• Postnatal (10%): Unknown factors.

Topographic Classification (Distribution of Motor Defect)

• Monoplegia: One limb affected.
• Hemiplegia: Upper and lower limbs on one side affected.
• Diplegia: All limbs affected, lower limbs more affected than upper limbs.
• Paraplegia: Only both lower limbs affected.
• Quadriplegia: All four limbs affected.

Clinical Types

• Spastic Cerebral Palsy: The most common type, characterized by pyramidal tract lesion (UMNL) signs like hypertonia, hyperreflexia, positive Babinski sign, and possible clonus. Often includes persistent primitive reflexes and pseudobulbar palsy. Often due to pre- or perinatal stroke.

  • Spastic diplegia: Primarily affects legs (more than arms).
  • Spastic hemiplegia: Due to prenatal or neonatal stroke, shows decreased movements on the affected side, and delayed walking.
  • Spastic quadriplegia: Most severe form, affecting all extremities, often associated with mental retardation and seizures.

• Ataxic Cerebral Palsy: Characterized by hypotonia and hyporeflexia, cerebellar ataxia (incoordination of voluntary movements), nystagmus, and intention tremors.

• Extrapyramidal (Dyskinetic/Athetoid) Cerebral Palsy: Commonest causes include asphyxia and kernicterus. Characterized by hypotonia, then subsequently rigidity and abnormal movements (choreic, athetoid).

• Atonic Cerebral Palsy: Deep hypotonia, meaning a floppy infant, contrasting with spasticity.

• Mixed Cerebral Palsy: Combination of different types of cerebral palsy.

Diagnosis

• Clinical evaluation through thorough history and physical examination. Ruling out progressive CNS disorders, metabolic disorders, spinal cord tumors, and muscular dystrophies.
• Investigations such as CT and MRI scans to identify the cause.

Associated Conditions

• Brain malformations, spinal cord lesions, and congenital infections are possible causes. TORCH screen, genetic evaluation, metabolic screen, and assessments for hearing, visual function, and seizures are used to rule out complications.
• Other conditions that can mimic cerebral palsy may include spinal cord tumors, channelopathies, Sandifer syndrome, MECP2 duplication, congenital dopa-responsive disorders, genetic spastic paraplegia, specific metabolic disturbances (e.g. GLUT1 deficiency), and ataxia telangiectasia.

Treatment

• Multidisciplinary approach involving specialists like occupational therapists, physical therapists, speech pathologists, social workers, educators, and developmental psychologists.
• Medications include anti-spastic drugs aiming to reduce spasticity.
• Possible surgical interventions like adductor tenotomy or psoas transfer and rhizotomy for marked lower extremity spasticity. Botox injections for drooling and spastic muscles, and Levodopa for dystonia or rigidity.