Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 17, 2009
Schizencephaly
- Schizencephaly consists of unilateral or bilateral cleft(s) in the cerebral hemisphere(s) causing communication between the ventricles and subaracnoid spaces.
- Clefts are often found in perisilvian areas but can be found all along the cerebral cortex and are lined with gray matter (usually polymicrogyria).
- Two variations: the first is open lip where the walls of the cleft are widely separated. The second is closed lip where the walls are in close approximation.
- There is often associated septo-optic dysplasia with agenesis of the septum pellucidum and optic nerve hypoplasia.
- The ventricle wall may be tented and "point" to the defect.
- The etiology of schizencephaly is unclear and likely multifactorial.
- Clinical findings include focal seizures with early onset and developmental delay and contra-lateral paraplegia.
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