SEIZURES IN KLINEFELTERS-SYNDROME

This study describes the clinical spectrum of patients with Klinefelte r's syndrome and seizures. Klinefelter's syndrome is a sex chromosomal abnormality and the most common cause of male hypogonadism, It is cha racterized by cognitive dysfunction, hypogonadism, and abnormalities o f physical maturation. Neurologic impairment has been recognized, but seizures have received little attention. The authors describe three Am erican patients and discuss nine additional patients from two European centers previously reported with Klinefelter's syndrome and seizures, The most common profile of patients with Klinefelter's syndrome and s eizures includes mental retardation, behavior problems, epileptiform e lectroencephalograms (EEGs), and generalized tonic-clonic seizures. Th e seizures of six of 11 patients with epilepsy were well controlled wi th antiepileptic drugs. One patient had a single seizure and was not t reated with medication. In patients with Klinefelter's syndrome and re current seizures, the electroclinical spectrum is heterogenous and out come with antiepileptic drug treatment is favorable. (C) 1998 by Elsev ier Science Inc. All rights reserved.