CLINICAL AND ELECTROENCEPHALOGRAPHIC FINDINGS IN JUVENILE TYPE DRPLA

We present five different types of dentatorubral-pallidoluysian atroph y in one Japanese family. Two siblings and their paternal uncle manife sted the juvenile type dentatorubral-pallidoluysian atrophy, the sibli ngs' father had the late-adult type, and another paternal uncle had th e early-adult type, Gene analysis confirmed the diagnosis for the prob and and her sibling. By following the clinical courses and electroence phalographic changes, we found that the types of epileptic seizures an d the electroencephalograms of the juvenile dentatorubral-pallidoluysi an atrophy patients changed as the illness progressed. The siblings ex hibited different levels of clinical severity despite the similar deox yribonucleic acid expansion detected in their lymphocytes. (C) 1998 by Elsevier Science Inc. All rights reserved.