Electroencephalographic findings of hereditary dentatorubral-pallidoluysian atrophy (DRPLA).
The electroencephalograms (EEGs) of 22 patients suffering from hereditary DRPLA were studied. Epileptoform patterns were observed in 14 patients (63.6%) with epileptic seizures. The epileptoform patterns most frequently observed were those atypical spike-wave complexes. Slow wave bursts were seen in 18 patients (81.8%). Photosensitivity was revealed in six (27.3%) patients, all of whom presented progressive myoclonus epilepsy (PME) syndrome. Abnormal background activity was evident in 17 (77.3%) patients. These abnormalities in EEG were more severe in patients in the PME type than those of the A (ataxia) and AE (ataxia and epilepsy) types.
Inazuki
G
G
Department of Psychiatry, Niigata University School of Medicine, Japan.
Baba
K
K
Naito
H
H
D000293:Adolescent; D000328:Adult; D001264:Athetosis / Q000175:diagnosis / Q000235:genetics*; D002524:Cerebellar Ataxia / Q000175:diagnosis / Q000235:genetics*; D002529:Cerebellar Nuclei / Q000002:abnormalities; D002648:Child; D004569:Electroencephalography; D004831:Epilepsies, Myoclonic / Q000175:diagnosis / Q000235:genetics*; D005071:Evoked Potentials; D005260:Female; D005917:Globus Pallidus / Q000002:abnormalities*; D006801:Humans; D006816:Huntington Disease / Q000175:diagnosis / Q000235:genetics*; D008297:Male; D008875:Middle Aged; D009849:Olivopontocerebellar Atrophies / Q000175:diagnosis / Q000235:genetics*; D012012:Red Nucleus / Q000002:abnormalities*; D013132:Spinocerebellar Degenerations / Q000235:genetics*