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Journal of Child Neurology
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Infantile Spasms: An Early Epileptic Manifestation in Some Patients With the Congenital Bilateral Perisylvian Syndrome

Ruben Kuzniecky, MD

UAB Epilepsy Center University of Alabama at Birmingham, Birmingham, AL

Frederick Andermann, MD, FRCPC

Montreal Neurological Institute McGill University, Montreal, Quebec

Renzo Guerrini, MD

INPE University of Pisa, IRCC Stella Maris, Pisa, Italy

We report four patients with infantile spasms and the congenital bilateral perisylvian syndrome. Onset of spasms occurred during the first 6 months of life. Response to corticotropin treatment was prompt and resulted in resolution of seizures in all patients. Epilepsy developed in the four children after an interval of 2 to 12 years. Developmental outcome was variable; three were severely restricted and one was married and lived independently. Imaging studies revealed bilateral perisylvian lesions characteristic of polymicrogyria. Infantile spasms may be the presenting seizure type in some patients with the congenital bilateral perisylvian syndrome. (J Child Neurol 1994;9:420-423).

Journal of Child Neurology, Vol. 9, No. 4, 420-423 (1994)
DOI: 10.1177/088307389400900418
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