Premium
Electroencephalographic Findings of Hereditary Dentatorubral‐Pallidoluysian Atrophy (DRPLA)
Author(s) -
Inazuki Gen,
Baba Kansaku,
Naito Haruhiko
Publication year - 1989
Publication title -
psychiatry and clinical neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.609
H-Index - 74
eISSN - 1440-1819
pISSN - 1323-1316
DOI - 10.1111/j.1440-1819.1989.tb02572.x
Subject(s) - myoclonus , ataxia , progressive myoclonus epilepsy , epilepsy , atrophy , medicine , electroencephalography , pediatrics , audiology , pathology , psychiatry
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.