CP

• **Lesch-Nyhan can mimic CP, so it is important to screen children with an initial diagnosis of CP for Lesch-Nyhan, especially if the prenatal and perinatal courses were normal.**
• a static motor encephalopathy that arises during the perinatal period from a lesion or defect in the developing brain.
• Seizures, impaired vision, developmental delay, or speech problems may accompany these motor deficits. 
• 5 types...

Spastic diplegia

• is a form of cerebral palsy that is marked by bilateral spasticity of the lower extremities, which may be mild or severe,
• little deficit in the upper extremities.
• Scissoring of the lower extremities may be present when the child is suspended vertically.
• In mild or moderate cases, walking is delayed, and the child may walk on tiptoes.
• In some instances, the feet are in an equinovarus posture.
• Pathophys:
  • This injury pattern arises commonly in preterm infants from ischemia to the vascular border zone around the cerebral ventricles and resultant periventricular leukomalacia, as reported for the infant in the vignette.
• The prognosis for normal cognitive development and no seizure activity is good among children who have spastic diplegia.
• Required supportive care may be limited to orthopedic interventions and physical therapy to reduce spasticity.

Other cerebral palsies can be more variable and severe in prognosis.

Spastic hemiplegia is accompanied by

• more upper than lower extremity spasticity
• walking often is delayed until 2 years of age.
• This pattern frequently is due to an atrophic cerebral hemisphere; intrauterine stroke is one possible cause.
• At least 25% of children who have spastic hemiplegia are cognitively impaired, sometimes with mental retardation, and they may have seizures.

Spastic quadriplegia

• is more severe
• increased tone in all extremities, often more in the lower
• strong tendency toward mental retardation, seizures, feeding difficulties because of dysfunctional swallow, speech problems, and cortical visual impairment.

Athetoid cerebral palsy

• can be similar in presentation to spastic quadriplegia
• although the basal ganglia are more involved, resulting in more dystonia and extraneous movement.
• Rarely, this pattern follows kernicterus
• Currently it is seen more often with hypomyelination of the basal ganglia following hypoxic-ischemic injury in an infant who is born at term.

Ataxic cerebral palsy is rare. A child who presents with ataxia and developmental delay should be evaluated for underlying disorders such as juvenile Huntington disease or ataxia-telangiectasia.

Management

Caring for the child who has cerebral palsy requires a multidisciplinary approach that involves physicians, physical and occupational therapists, speech pathologists, social workers, and educators. Adaptive equipment such as braces, motorized chairs, and communication boards may be necessary. Nutritional guidance is important, and feeding devices may be necessary because of dysfunctional swallowing and aspiration risk. Spasticity may be managed with drug treatment (benzodiazepines, dantrolene, and baclofen), intrathecal baclofen pumps, dorsal root rhizotomy, or intramuscular botulinum toxin injections. Parental support, including visiting health nurse, financial counseling, and respite care, can be extremely helpful.

References:
Haslam RH. Encephalopathies: cerebral palsy. In: Behrman RE, Kliegman
RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed.
Philadelphia, Pa: WB Saunders Co; 2000:1843-1845
Liptak GS. The pediatrician's role in caring for the developmentally
disabled child. Pediatr Rev. 1996;17:203-210