1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-8-29-a 10.15844/pedneurbriefs-8-4-7 Seizure Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Perisylvian Polymicrogyria Epileptic Syndrome http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 04 1994 01 06 2016 8 4 29 29 Copyright: © 1994 The Author(s) 1994 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The epileptic spectrum in the congenital bilateral perisylvian syndrome

The epileptic spectrum and EEG findings in 31 patients with a congenital bilateral perisylvian syndrome are reported from the University of Alabama at Birmingham and the CBPS Multicenter Collaborative Study Group.

 Congenital Bilateral Perisylvian Syndrome Pseudobulbar Palsy Perisylvian Polymicrogyria

The epileptic spectrum and EEG findings in 31 patients with a congenital bilateral perisylvian syndrome are reported from the University of Alabama at Birmingham and the CBPS Multicenter Collaborative Study Group. The syndrome was characterized by pseudobulbar palsy, cognitive deficits, polymicrogyria and seizures. Associated malformations (eg. arthrogryposis, club feet) were present in 30% patients. Seizures present in 27 (87%) began between 1 month and 14 years of age (mean, 7.9 years). Seizure patterns were varied and mainly consistent with secondary generalized epilepsy; infantile spasms occurred in 4, and partial seizures in 7 (26%). EEG abnormalities were generalized spike and wave and multifocal discharges in 7, and multifocal patterns in 10 (39%). CT and MRI showed symmetric bilateral perisylvian cortical thickening. Seizures were unresponsive to AEDs in 65%. Callosotomy in 7 with intractable epilepsy and drop attacks was partially effective; drop attacks ceased in 4. [1]

COMMENT. The authors and study group report a developmental syndrome characterized by congenital pseudobulbar palsy, epilepsy, mental retardation, and perisylvian polymicrogyria. Patients with drop attacks may respond to callosotomy when antiepileptic drugs are ineffective.

 Kuzniecky R Andermann F Guerrini R The epileptic spectrum in the congenital bilateral perisylvian syndrome Neurology 1994 Mar 44 3 Pt 1 379 385 10.1212/WNL.44.3_Part_1.379 8145902