SCHIZENCEPHALY
 
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SCHIZENCEPHALY

 

DEFINITION:

A disorder of neuronal migration characterized by unilateral or bilateral clefts within the cerebral hemispheres.

EPIDEMIOLOGY:

  • incidence: ?
  • age of onset:
    • infancy to childhood
  • risk factors:
    • no genetic or chromosomal basis has been established
  • associated anomalies:
    • disorders of induction
      • myelomeningocele
    • other migration disorders
      • grey matter heterotopias
      • microgyria

PATHOGENESIS:

1. Background

  • clinical features correlate with the size of the lesion
  • pathologically may present as a fluid-filled brain cavity lined by normal dura or a pia-arachnoid membrane

CLINICAL FEATURES:

1. Neurologic Manifestations

  • infantile hypotonia
  • seizure disorder
  • developmetnal delay
  • mental retardation
  • microcephaly
  • motor dysfunction (spastic quadriplegia, hemiparesis, hypotonia)

INVESTIGATIONS:

1. Imaging Studies

1. CT/MRI

  • the clefts within the cerebral hemispheres can extend from the cortical surface or the subarachnoid space to the underlying ventricular cavity
  • clefts are almost invariably located in the area of the sylvian fissure
  • symmetrical clefts usually align parallel to the normal primary fissures
  • hypoplastic cerebrum in region of the cleft

MANAGEMENT:

1. Team Approach

  • periodic multidisciplinary follow-up
    • Paediatrics, Neurology, Orthopedics, OT, PT
  • genetic counselling
    • as no genetic or chromosomal etiology has been identified, there is a low risk for recurrence

 

 

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