title: Two cases of spinocerebellar ataxia accompanied by involvement of the skeletal motor neuron system and bulbar palsy.
abstract: We report two patients with spinocerebellar ataxia (SCA) with cranial and spinal motor neuron involvement. They initially presented with cerebellar ataxia, followed by bulbar palsy and limb motor neuron sign. One of the patients had a brother with allied disorder. SCA type 1 (SCA1), SCA3 and SCA6 have been reported to involve the motor neuron system, but they were excluded by DNA analyses in the present two patients. These two patients may form a distinct disease entity among SCAs.
authors.affiliation: Department of Neurology, Graduate School of Medicine, Dentistry and Pharmacy, Okayama University. yasuyuki@cc.okayama-u.ac.jp
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Ohta
authors.forename: Takeshi
authors.initials: T
authors.lastname: Hayashi
authors.forename: Makiko
authors.initials: M
authors.lastname: Nagai
authors.forename: Miyuki
authors.initials: M
authors.lastname: Okamoto
authors.forename: Shoko
authors.initials: S
authors.lastname: Nagotani
authors.forename: Isao
authors.initials: I
authors.lastname: Nagano
authors.forename: Nobuhiko
authors.initials: N
authors.lastname: Ohmori
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Takehisa
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Murakami
authors.forename: Mikio
authors.initials: M
authors.lastname: Shoji
authors.forename: Tatsushi
authors.initials: T
authors.lastname: Kamiya
authors.forename: Koji
authors.initials: K
authors.lastname: Abe
mesh_terms: D010244:Bulbar Palsy, Progressive / Q000150:complications* / Q000175:diagnosis; D004247:DNA / Q000235:genetics; D004576:Electromyography; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000175:diagnosis; D009133:Muscular Atrophy / Q000150:complications / Q000175:diagnosis / Q000473:pathology; D010375:Pedigree; D020754:Spinocerebellar Ataxias / Q000150:complications* / Q000175:diagnosis / Q000235:genetics; D014059:Tongue / Q000473:pathology
chemical_list: D004247:DNA