Absence-epilepsy and Klinefelter syndrome

We describe a man with typical absence seizures and generalized tonic-clonic seizures (GTCS) in whom typical Klinefelter syndrome (KS) was diagnosed at the age of 36 years. Epileptic manifestations had onset at the age of 8 years. EEG and clinical data were consistent with juvenile absence epilepsy. This case underscores the little-studied association between epilepsy and KS, which is probably underestimated since KS is often diagnosed in adulthood. (C) 1997 by Elsevier Science Inc.