Metabolic characterization of spinocerebellar ataxia type 6

被引：30
作者：
Soong, BW [1 ]
Liu, RS
Wu, LC
Lu, YC
Lee, HY
机构：
[1] Vet Gen Hosp Taipei, Neurol Inst, Taipei 112, Taiwan
[2] Natl Yang Ming Univ, Sch Med, Dept Neurol, Taipei 112, Taiwan
[3] Vet Gen Hosp Taipei, PET Cyclotron Ctr, Taipei 112, Taiwan
来源：
ARCHIVES OF NEUROLOGY | 2001年 / 58卷 / 02期
关键词：
D O I：
10.1001/archneur.58.2.300
中图分类号：
R74 [神经病学与精神病学];
学科分类号：
摘要：
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.
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页码：300 / 304
页数：5
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