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The wide clinical spectrum and nigrostriatal dopaminergic damage in spinocerebellar ataxia type 6

Cited 24 time in Web of Science Cited 29 time in Scopus
Authors
Kim, Jong-Min; Lee, Jee-Young; Kim, Ji Seon; Kim, Hee Jin; Kim, Yu Kyeong; Kim, Sang Eun; Jeon, Beom S.; Park, Sung Sup
Issue Date
2010-05
Publisher
B M J PUBLISHING GROUP
Citation
JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY; Vol.81 5; 529-532
Abstract
Spinocerebellar ataxia type 6 (SCA6) manifests a wide spectrum of non-cerebellar system involvements. The objective of this study was to examine the presence of nigrostriatal dopaminergic system derangement in SCA6. Eight patients with SCA6 who underwent a regular follow-up for at least 2 years participated in this study. A detailed neurological examination was performed and striatal dopamine transporter (DAT) was evaluated using [(99m)Tc]-eTRODAT-1 SPECT. The main clinical feature of SCA6 was cerebellar ataxia with impaired eye movements. However, a wide spectrum of non-cerebellar system involvements, such as autonomic dysfunction, and pyramidal and extrapyramidal signs, was also observed. Two patients had bradykinesia. L-dopa was tried in one patient without benefit. Of the two patients with bradykinesia, DAT density was reduced to the Parkinson`s disease (PD) range with a rostrocaudal gradient typical of PD in one patient (CAG repeats 13/22) and was mildly decreased in the other patient (12/25). Of the four patients without extrapyramidal signs, three (12/22, 11/25, 17/22) showed mild to severe reduction of DAT density and one (13/22) had a normal density. This study shows that SCA6 has a varying degree of nigrostriatal dopaminergic derangement. Two patients manifested mild bradykinesia, emphasising the need to screen for SCA6, even in patients with progressive ataxia and parkinsonism. Further histopathological studies would be helpful to determine the nigrostriatal dopaminergic damage in SCA6.
ISSN
0022-3050
Language
English
URI
https://hdl.handle.net/10371/76309
DOI
https://doi.org/10.1136/jnnp.2008.166728
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College of Medicine/School of Medicine (의과대학/대학원)Laboratory Medicine (검사의학전공)Journal Papers (저널논문_검사의학전공)
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