PMID: 9830997Nov 27, 1998Paper

Seizures in Klinefelter's syndrome

Pediatric Neurology
W O TatumP Genton

Abstract

This study describes the clinical spectrum of patients with Klinefelter's syndrome and seizures. Klinefelter's syndrome is a sex chromosomal abnormality and the most common cause of male hypogonadism. It is characterized by cognitive dysfunction, hypogonadism, and abnormalities of physical maturation. Neurologic impairment has been recognized, but seizures have received little attention. The authors describe three American 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 seizures includes mental retardation, behavior problems, epileptiform electroencephalograms (EEGs), and generalized tonic-clonic seizures. The seizures of six of 11 patients with epilepsy were well controlled with antiepileptic drugs. One patient had a single seizure and was not treated with medication. In patients with Klinefelter's syndrome and recurrent seizures, the electroclinical spectrum is heterogenous and outcome with antiepileptic drug treatment is favorable.

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Citations

Aug 24, 2006·Journal of Autism and Developmental Disorders·S L Merhar, P Manning-Courtney
Oct 29, 2004·Journal of Clinical Neurophysiology : Official Publication of the American Electroencephalographic Society·Salvatore GrossoPaolo Balestri
May 5, 2010·Italian Journal of Pediatrics·Giovanni Sorge, Anna Sorge
Apr 8, 2011·Brain & Development·Hirofumi InoueTakashi Ichiyama
Dec 15, 2015·Indian Journal of Psychological Medicine·Anu Rita JayaramanNambi Siva
Jan 24, 2009·American Journal of Medical Genetics. Part a·Stefano SartoriAlessandra Murgia
Dec 29, 2005·Journal of the Chinese Medical Association : JCMA·Chwen-Yi YangHon-Mei Cheng
Dec 17, 2010·Journal of Pediatric Endocrinology & Metabolism : JPEM·Inas MazenHala T El-Bassyouni
Feb 23, 2018·Frontiers in Genetics·Paul DunnLyn R Griffiths

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