Congenital unilateral perisylvian syndrome: radiological basis and clinical correlations.
METHODS
Advances in neuroimaging have allowed correlations between radiological patterns and clinical features of brain malformations. This paper reports clinical, prognosis, and electroencephalographic features of six children with a previously unrecognised neuroimaging picture of unilateral widening and verticalisation of the sylvian fossa associated with an abnormal ipsilateral perisylvian cortex.


RESULTS
All children had reduced hemisphere size and thalamostriatal hypoplasia ipsilateral to the cleft and hemiplegia. Cognitive development was mostly impaired. Epilepsy occurred in two patients and was mainly characterised by partial seizures. Studies with EEG showed hemispheric slowing of background activity homolateral to the perisylvian dysplasia. Occurrence of the malformation among their siblings was not found.


CONCLUSIONS
Similar brain malformations occasionally reported in older patients confirm the clinical picture, sporadic occurrence, and prognosis found, allowing the validation of a unilateral perisylvian syndrome.
Sébire
G
G
Département de Pédiatric, Hôpital Bicetre, Le Kremlin Bicetre, France.
Husson
B
B
Dusser
A
A
Navelet
Y
Y
Tardieu
M
M
Landrieu
P
P
D001921:Brain / Q000002:abnormalities* / Q000503:physiopathology*; D002547:Cerebral Palsy / Q000151:congenital* / Q000503:physiopathology*; D002675:Child, Preschool; D002880:Chromosomes, Human, Pair 11; D003072:Cognition Disorders / Q000503:physiopathology*; D004569:Electroencephalography; D004827:Epilepsy / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D007223:Infant; D007621:Karyotyping; D008279:Magnetic Resonance Imaging; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D013577:Syndrome