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

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• 作者：Young Eun Huh ; Ji-Soo Kim ; Hyo-Jung Kim ; Seong-Ho Park ; Beom Seok Jeon…
• 关键词：Spinocerebellar ataxia ; Vertigo ; Cerebellum ; Vestibulo ; ocular reflex ; Head impulse test
• 刊名：The Cerebellum
• 出版年：2015
• 出版时间：June 2015
• 年：2015
• 卷：14
• 期：3
• 页码：284-291
• 全文大小：1,162 KB
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• 作者单位：Young Eun Huh (1) (3)
  Ji-Soo Kim (1)
  Hyo-Jung Kim (1)
  Seong-Ho Park (1)
  Beom Seok Jeon (2)
  Jong-Min Kim (1)
  Jin Whan Cho (3)
  David S. Zee (4)
  
  1. Department of Neurology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam-si, Gyeonggi-do, 463-707, Korea
  3. Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  2. Department of Neurology, Seoul National University College of Medicine, Seoul National University Hospital, Seoul, Korea
  4. Department of Neurology, Otolaryngology Head and Neck Surgery, Neuroscience, and Ophthalmology, The Johns Hopkins University School of Medicine, Baltimore, USA
  
• 刊物主题：Neurosciences; Neurology; Neurobiology;
• 出版者：Springer US
• ISSN：1473-4230

文摘

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=33-2?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=?.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, high-frequency head impulses may be a quantitative indicator of clinical decline in SCA6.