title: Benign adult familial myoclonus epilepsy is a progressive disorder: no longer idiopathic generalized epilepsy.
abstract: Brain dysfunction in Japanese benign adult familial myoclonus epilepsy (BAFME) has not been elucidated. To clarify diffuse brain dysfunction as indicated by posterior dominant rhythm (PDR) slowing in patients with BAFME. The frequency of PDR on EEG was studied in 19 BAFME patients (50.6±15.7 years) and 38 age-matched control subjects (50.1±14.5 years). We investigated the relationship between age and PDR in both groups. PDR frequency in the patient group (9.1±0.7 Hz) was significantly slower than that of age-matched control subjects (10.4±1.1 Hz; p<0.0001), regardless of the use of anticonvulsants. There was no significant difference in PDR slowing with age between groups. These findings suggest that Japanese patients with BAFME have mild diffuse brain dysfunction with minimal progression.
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Department of Clinical Laboratory Medicine, Kyoto University Graduate School of Medicine.
authors.forename: Takefumi
authors.initials: T
authors.lastname: Hitomi
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine.
authors.forename: Katsuya
authors.initials: K
authors.lastname: Kobayashi
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine.
authors.forename: Takeyo
authors.initials: T
authors.lastname: Sakurai
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine.
authors.forename: Sakiho
authors.initials: S
authors.lastname: Ueda
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine.
authors.forename: Naoto
authors.initials: N
authors.lastname: Jingami
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine, Department of Neurology, Takeda General Hospital.
authors.forename: Kyoko
authors.initials: K
authors.lastname: Kanazawa
authors.affiliation: Department of Epilepsy, Movement Disorders and Physiology Graduate School of Medicine, Kyoto, Japan.
authors.forename: Riki
authors.initials: R
authors.lastname: Matsumoto
authors.affiliation: Department of Neurology, Kyoto University Graduate School of Medicine.
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Takahashi
authors.affiliation: Department of Epilepsy, Movement Disorders and Physiology Graduate School of Medicine, Kyoto, Japan.
authors.forename: Akio
authors.initials: A
authors.lastname: Ikeda
mesh_terms: D000328:Adult; D000368:Aged; D044466:Asian People; D001921:Brain / Q000503:physiopathology*; D018450:Disease Progression; D004569:Electroencephalography / Q000379:methods; D004831:Epilepsies, Myoclonic / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology*; D004829:Epilepsy, Generalized / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree
keywords: benign adult familial myoclonus epilepsy (BAFME); diffuse brain dysfunction; posterior dominant rhythm