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NEUROLOGY 1998;51:504-512
© 1998 American Academy of Neurology

Multilobar polymicrogyria, intractable drop attack seizures, and sleep-related electrical status epilepticus

R. Guerrini, MD, P. Genton, MD, M. Bureau, MD, A. Parmeggiani, MD, X. Salas-Puig, MD, M. Santucci, MD, P. Bonanni, MD, G. Ambrosetto, MD and C. Dravet, MD

From the Institute of Child Neurology and Psychiatry, University of Pisa, and Institute for Clinical Research Stella Maris Foundation (Drs. Guerrini and Bonanni), Pisa, Italy; the Centre Saint Paul (Drs. Genton, Bureau, and Dravet), Marseille, France; Institute of Neurology and Department of Child Neuropsychiatry (Drs. Parmeggiani, Santucci, and Ambrosetto), University of Bologna, Italy; and Department of Neurology (Dr. Salas-Puig), General Hospital of Asturias, Oviedo, Spain.

Address correspondence and reprint requests to Dr. Renzo Guerrini, Institute of Child Neurology and Psychiatry, University of Pisa, Via dei Giacinti 2, 56018 Calambrone, Pisa, Italy.

Background and Objective: Patients with cortical malformations often have intractable seizures and are candidates for epilepsy surgery. Within an unselected series of patients with various forms of cortical malformation, nine patients with multilobar polymicrogyria had electrical status epilepticus during sleep (ESES) accompanied by infrequent focal motor seizures. Eight patients also had intractable atonic drop attack seizures. Because ESES usually is accompanied by a good long-term seizure prognosis, the objective of this study was to examine ESES outcome among patients with a structural lesion that is usually highly epileptogenic and has a low seizure remission trend.

Methods: The nine patients had follow-up periods lasting 4 to 19 years. All underwent brain MRI, serial sleep EEG recordings, and cognitive testing during and after ESES.

Results: ESES and drop attack seizures appeared between the ages of 2 and 5 years(mean, 4 years) and ceased between the ages of 5 and 12 years (mean, 8 years). At the last visit patients were 8 to 23 years of age (mean, 14.5 years) and were either seizure free or had very infrequent focal motor seizures during sleep. Three patients were free from antiepileptic drugs. In no patient was definite cognitive deterioration apparent after ESES in comparison with earlier evaluations.

Conclusions: Age-related secondary bilateral synchrony underlying ESES may be facilitated in multilobar polymicrogyria. The good seizure outcome contrasts with that usually found in the presence of cortical malformations. For children with polymicrogyria and drop attack seizures, surgical treatment of the epilepsy should be considered cautiously, and sleep EEG recordings should be performed systematically.

Received January 22, 1998. Accepted in final form April 24, 1998.




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