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Vestibular Performance During High-Acceleration Stimuli Correlates with Clinical Decline in SCA6

Cited 15 time in Web of Science Cited 16 time in Scopus
Authors
Huh, Young Eun; Kim, Ji-Soo; Kim, Hyo-Jung; Park, Seong-Ho; Jeon, Beom Seok; Kim, Jong-Min; Cho, Jin Whan; Zee, David S.
Issue Date
2015
Publisher
Springer
Citation
Cerebellum, vol.14, pp. 284-291
Keywords
Spinocerebellar ataxiaVertigoCerebellumVestibulo-ocular reflexHead impulse test
Abstract
In spinocerebellar ataxia type 6 (SCA6), the vestibular dysfunction and its correlation with other clinical parameters require further exploration. We determined vestibular responses over a broad range of stimulus acceleration in 11
patients with SCA6 (six men, age range=33–72 years, mean age±SD=59±12 years) using bithermal caloric irrigations, rotary chair, and head impulse tests. Correlations were also pursued among disability scores, as measured using the International Cooperative Ataxia Rating Scale, disease duration, age at onset, cytosine-adenine-guanine (CAG) repeat length, and the gain of the vestibulo-ocular reflex (VOR). In response to relatively low-acceleration, low-frequency rotational and bithermal caloric stimuli, the VOR gains were normal or increased regardless of the severity of disease. On the other hand, with relatively high-acceleration, high-frequency head impulses, there was a relative increase in gain in the mildly affected patients and a decrease in gain in the more severely affected patients and gains were negatively correlated with the severity of disease (Spearman correlation, R=−0.927, p<0.001). Selective decrease of the vestibular responses during high-acceleration, high-frequency stimuli may be ascribed to degeneration of either the flocculus or vestibular nuclei. The performance of the VOR during high-acceleration, highfrequency head impulses may be a quantitative indicator of clinical decline in SCA6.
ISSN
1473-4222
Language
English
URI
http://hdl.handle.net/10371/95208
DOI
https://doi.org/10.1007/s12311-015-0650-3
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Neurology (신경과학교실)Journal Papers (저널논문_신경과학교실)
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