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Bing-wen Soong, MD, PhD; Ren-shyan Liu, MD; Liang-chih Wu, PhD; Yi-chun Lu, MS; Hsiang-ying Lee, MS

Arch Neurol. 2001;58:300-304.

Background  Spinocerebellar ataxia type 6 (SCA6) is a neurodegenerative disorder characterized by slowly progressive ataxia and dysarthria. The mutational basis is an expanded CAG repeat sequence within the coding regions of the CACNL1A4 gene. Basic clinical, neuroimaging, and pathological, and epidemiological features have been described in the literature. However, the metabolic features of SCA6 have not been elucidated.

Objective  To investigate the metabolic features of SCA6.

Patients and Methods  Seven patients with SCA6 and 7 healthy individuals underwent positron emission tomography using fluorodeoxyglucose F 18.

Results  Cerebral glucose utilization in the 7 patients with SCA6 was characterized by significant hypometabolism in widespread structures, including cortical regions and basal ganglia, as well as the cerebellar hemispheres and brainstem.

Conclusions  The results of the multiple-regional brain hypometabolism suggest that brain dysfunction associated with SCA6 may not be limited to the cerebellum and inferior olive, as previously suggested by the results of other pathologic studies.

From the Department of Neurology, National Yang-Ming University School of Medicine (Dr Soong), and the Neurological Institute (Dr Soong and Mss Lu and Lee) and PET/Cyclotron Center (Drs Liu and Wu), Veterans General Hospital–Taipei, Taipei, Taiwan, Republic of China.

Corresponding author and reprints: Bing-wen Soong, MD, PhD, Neurological Institute, Veterans General Hospital–Taipei, Taipei, Taiwan 112, Republic of China (e-mail: bwsoong{at}vghtpe.gov.tw).

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