Positional vertigo and macroscopic downbeat positioning nystagmus in spinocerebellar ataxia type 6 (SCA6).
To investigate the frequency of positioning nystagmus in degenerative ataxic disorders, we examined downbeat positioning nystagmus (DPN) in 25 patients with spinocerebellar ataxia type 6 (SCA6) and 58 patients with other types of degenerative ataxia. DPN was observed in 21 of the 25 patients with SCA6 (84 %) versus only 3 of the 58 patients (5.2 %) with other types of degenerative ataxia, including multiple system atrophy, SCA1, SCA2, SCA3/Machado-Joseph disease, and non-SCA6 late-onset pure cerebellar ataxia. Our findings indicated that DPN is a distinct part of the clinical presentation of SCA6, showing that vestibular cerebellum is more affected in SCA6 than other types of degenerative ataxia.
Yabe
Ichiro
I
Department of Neurology, Hokkaido University Graduate School of Medicine, N15 W7, Kita-ku, Sapporo 060-8638, Japan. hsasaki@med.hokudai.ac.jp
Sasaki
Hidenao
H
Takeichi
Norihito
N
Takei
Asako
A
Hamada
Takeshi
T
Fukushima
Kikuro
K
Tashiro
Kunio
K
D000328:Adult; D000368:Aged; D002531:Cerebellum / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009760:Nystagmus, Physiologic; D020754:Spinocerebellar Ataxias / Q000150:complications*; D014717:Vertigo / Q000209:etiology* / Q000473:pathology