Abstract：
:Positron emission tomography studies on the regional cerebral glucose metabolism (rCMRglc) and 18F-fluorodopa (18F-Dopa) uptake were performed in 3 patients with Machado-Joseph disease (MJD), a dominantly inherited degenerative disease in the cerebellum, brainstem and basal ganglia. The rCMRglc in MJD was found to be significantly decreased in the cerebellum, brainstem, striatum and whole cerebral cortex in comparison to that in normal subjects. These results of rCMRglc were different from those for dominantly inherited olivopontocerebellar atrophy (dOPCA) or cerebellar cortical degeneration (CCD), however they were similar to those for sporadic olivopontocerebellar atrophy (sOPCA) and multiple system atrophy (MSA). The 18F-Dopa uptake in MJD was found to be significantly decreased in the putamen and relatively spared in the caudate, which was different from that of MSA. In addition, these results indicate that MJD showed a dysfunction, not only in the regions with apparent pathological involvement such as cerebellum, brainstem and nigro-striatal dopaminergic system, but also in the cerebral cortex and the striatum where no pathology could be observed using conventional morphological techniques.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Taniwaki T,Sakai T,Kobayashi T,Kuwabara Y,Otsuka M,Ichiya Y,Masuda K,Goto Idoi
10.1016/s0022-510x(96)00242-0subject
Has Abstractpub_date
1997-01-01 00:00:00pages
63-7issue
1eissn
0022-510Xissn
1878-5883pii
S0022510X96002420journal_volume
145pub_type
杂志文章abstract：:We report eight patients with adult-onset subacute sclerosing panencephalitis (SSPE), of which, four were treated with oral isoprinosine and four with intraventricular alpha-interferon plus oral isoprinosine. One of the four patients treated with oral isoprinosine died within two months, and the disease progressed in ...
journal_title：Journal of the neurological sciences
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pub_type： 杂志文章,评审
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pub_type： 杂志文章,多中心研究
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pub_type： 杂志文章
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
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pub_type： 杂志文章
doi：10.1016/j.jns.2003.09.014
更新日期：2004-02-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
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pub_type： 杂志文章
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更新日期：2000-06-15 00:00:00
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