Differences in Dysphagia Between Spinocerebellar Ataxia Type 3 and Type 6
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ORIGINAL ARTICLE
Differences in Dysphagia Between Spinocerebellar Ataxia Type 3 and Type 6 Chiharu Isono • Makito Hirano • Hikaru Sakamoto • Shuichi Ueno • Susumu Kusunoki • Yusaku Nakamura
Received: 28 June 2012 / Accepted: 23 January 2013 / Published online: 21 March 2013 Ó Springer Science+Business Media New York 2013
Abstract Spinocerebellar ataxias (SCAs) are a group of neurodegenerative disorders frequently associated with autosomal dominant inheritance. SCA type 3 (SCA3) and SCA type 6 (SCA6) are the most common forms in Japan as well as the rest of the world. SCA3 affects multiple nervous systems while SCA6 affects mainly the cerebellar system. Dysphagia is clinically important since aspiration pneumonia is the most common cause of death in patients with SCA. We retrospectively studied dysphagia in 7 patients with SCA3 and 13 with SCA6 by videofluoroscopic examination of swallowing (VF). This is a larger series of patients with SCA6 than in previous studies, which had inconsistent results. Dysphagia was evaluated according to the scale established by the Japanese Society of Dysphagia Rehabilitation and the dysphagia outcome severity scale, an internationally used scale. The former separately evaluates oral and pharyngeal phases, while the latter concurrently grades both phases. Dysphagia according to the Japanese scale was mild but statistically significant in SCA6 and severe in SCA3. DOSS indicated abnormalities in SCA3 but not in SCA6. The swallowing abnormalities in SCA3 or SCA6 did not parallel the duration of disease or physical
Electronic supplementary material The online version of this article (doi:10.1007/s00455-013-9450-4) contains supplementary material, which is available to authorized users. C. Isono M. Hirano (&) H. Sakamoto S. Ueno Y. Nakamura Department of Neurology, Faculty of Medicine, Sakai Hospital Kinki University, 2-7-1 Harayamadai Minami-ku, Sakai, Osaka 590-0132, Japan e-mail: [email protected] S. Kusunoki Department of Neurology, Faculty of Medicine, Kinki University, Osakasayama, Osaka 577-0818, Japan
disability, suggesting that even patients with early disease or with well-preserved physical functions were at risk for aspiration. Our patients with dysphagia received percutaneous endoscopic gastrostomy-tube feeding at an appropriate time and underwent rehabilitation of swallowing. No patient had aspiration pneumonia. In conclusion, evaluation of swallowing ability by VF is essential for preventing aspiration in patients with SCA. Keywords Dysphagia SCA3 SCA6 DOSS Pharyngeal phase Deglutition Deglutition disorders VF evaluation
Spinocerebellar ataxias (SCAs) are a group of neurodegenerative disorders frequently associated with autosomal dominant inheritance. Recent molecular advances have shown that SCA can be classified into more than 30 diseases. Of these, SCA type 3 (SCA3) and type 6 (SCA6) are the most common forms in Japan as well as the rest of the world [1, 2]. SCA3 typically develops in middle age and affects multiple systems, including the
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