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NEUROLOGY 1984;34:285
© 1984 American Academy of Neurology

Juvenile myoclonic epilepsy of Janz

A. V. Delgado-Escueta, MD and Fe Enrile-Bacsal, MD

From the Comprehensive Epilepsy Program (Dr. Delgado-Escueta), Department of Neurology, UCLA School of Medicine, Los Angeles, CA, the Southwest Regional Epilepsy Center (Dr. Delgado-Escueta), Neurology Service, West Los Angeles VA Medical Center, Wadsworth Division, Los Angeles, CA, and Public Health Service Fellow (Fogarty International) (Dr. Enrile-Bacsal), Section of Neurology, University of the Philippines College of Medicine, Taft Avenue, Manila, Philippines.

We studied 43 patients, aged 15 to 69 years, whose convulsive seizures were uncontrolled because the syndrome of juvenile myoclonic epilepsy was not recognized. Awakening myoclonic jerks appeared with tonic-clonic (18 patients), clonic-tonic-clonic (24 patients), and absence seizures (17 patients), with a mean age at onset of 13.6 years. Generalized seizures were present in relatives of 17 patients. All patients had diffuse 3 1/2- to 6-Hz multispike-wave complexes. Valproic acid stopped convulsions in 86% of patients. After being free of seizures for 2 years, withdrawal of valproic acid was followed by relapse of convulsions in 12 patients.

Address correspondence and reprint requests to Dr. Delgado-Escueta, Comprehensive Epilepsy Program, Department of Neurology. UCLA School of Medicine, Los Angeles, CA 90024.

Supported in part by Department of Health and Human Services contract No. N01-NS-0-2332.

Accepted for publication June 17, 1983.




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