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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March 9, 2009
Congenital Unilateral Perisylvian Syndrome
- Congenital perisylvian syndromes are late migration/cortical organization disorders associated with distinctive clinical and imaging features.
- Bilateral abnormalities are more common than unilateral. Bilateral cases present with pseudobulbar palsy while unilateral cases present with contralateral hemiparesis. Cognitive impairment and seizures are often present in both forms.
- Imaging finding consists of a vertically oriented sylvian fissure continuous with the central/postcentral sulcus associated with variable extension of perisylvian polymicrogyric cortex. Septo-optic dysplasia may be associated with the unilateral form.
- Axial MR images can be confused with schizencephaly, but sagittal images show typical findings.
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