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[Schizencephaly: clinical and MRI features].

T Morioka1, S Nishio, K Hisada

  • 1Department of Neurosurgery, Graduate School of Medicine, Kyushu University, Fukuoka, Japan.

No to Shinkei = Brain and Nerve
|December 10, 1999
PubMed
Summary

Schizencephaly, a brain malformation, presents variably even with bilateral lesions. This study highlights diverse clinical and neuroimaging findings in four cases, showing generally good neurodevelopmental outcomes and controlled epilepsy.

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Area of Science:

  • Neurology
  • Developmental Neuroscience
  • Neuroimaging

Context:

  • Schizencephaly is a rare congenital brain malformation characterized by a cleft in the cerebral hemisphere.
  • Understanding its diverse clinical and neuroimaging spectrum is crucial for accurate diagnosis and management.

Purpose:

  • To describe the clinical and neuroimaging features of four patients with schizencephaly.
  • To illustrate the variability in presentation and outcome, even in cases with bilateral lesions.

Summary:

  • Four cases of schizencephaly were analyzed, including variations in lesion laterality (unilateral/bilateral) and lip type (open/closed).
  • Despite bilateral lesions in most cases, neurodevelopmental outcomes were generally favorable, with mild hemiparesis noted in two patients.
  • All patients experienced epilepsy, which was effectively managed with antiepileptic drugs.

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Impact:

  • This case series underscores the significant variability in schizencephaly's clinical presentation and neurodevelopmental trajectory.
  • Findings emphasize that bilateral schizencephaly does not invariably lead to severe impairment, suggesting a complex interplay of factors influencing outcome.