Quantitative assessment of cerebral blood flow in genetically confirmed spinocerebellar ataxia type 6
Quantitative assessment of cerebral blood flow in genetically confirmed spinocerebellar ataxia type 6
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DOI：
10.1001/archneur.61.6.933
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发表时间：
2004-06-01
影响因子：
--
通讯作者：
Matsumoto, M
中科院分区：
文献类型：
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作者：
Honjo, K;Ohshita, T;Matsumoto, M
背景：脊髓小脑性共济失调6型（SCA 6）是一种由CAG三核苷酸扩增引起的常染色体显性遗传性小脑性共济失调。SCA 6患者的局部脑血流量（rCBF）特征尚未确定，而据报道，在其他小脑疾病中，大脑中的rCBF降低似乎是小脑损伤的远程影响。目的：为了阐明rCBF的特征，包括小脑-小脑的关系，通过脑单光子发射计算机断层扫描（SPECT）定量评估rCBF，并将其与遗传学证实的SCA 6患者的临床表现进行相关性分析。设计：使用Patlak图的锝Tc 99 m乙基半胱氨酸二聚体SPECT研究。患者：日本广岛，广岛大学医院。10例SCA 6和9名健康对照。主要结果测量：小脑蚓部，小脑半球和额叶的rCBF。结果：在SCA 6患者中，与健康对照组相比，小脑蚓部和半球的rCBF降低，这与病程呈负相关。额叶局部脑血流量与病程有轻度相关性，但无统计学意义。小脑蚓部的rCBF与构音障碍的严重程度呈负相关，但与CAG重复expansions.Conclusions无显著相关性：rCBF下降仅见于小脑，与病程、构音障碍和共济失调、小脑萎缩有关。SCA 6患者中未发现小脑低灌注的远期效应。
Background: Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant cerebellar ataxia caused by CAG trinucleotide expansion. The characteristics of regional cerebral blood flow (rCBF) in SCA6 patients have not been established, whereas it has been reported that decreased rCBF in the cerebrum seems to be a remote effect of cerebellar impairment in other cerebellar disorders.Objective: To clarify the characteristics of rCBF, including cerebro-cerebellar relationship, and its correlation with clinical manifestations in patients with genetically confirmed SCA6 using quantitative assessment of rCBF by brain single-photon emission computed tomography (SPECT).Design: Technetium Tc 99m ethyl cysteinate dimer SPECT study using a Patlak plot.Patients: Hiroshima University Hospital, Hiroshima, Japan. Ten patients with SCA6 and 9 healthy controls.Main Outcome Measure: The rCBF of the cerebellar vermis, cerebellar hemisphere, and frontal lobes.Results: In SCA6 patients, rCBF was decreased only in the cerebellar vermis and hemisphere compared with healthy controls, and this was inversely correlated with duration of illness. The rCBF in the frontal lobes was slightly correlated with duration of illness without statistical significance. The rCBF in the vermis was inversely correlated with severity of dysarthria, but there was no significant correlation with CAG repeated expansions.Conclusions: Decrease in rCBF was found only in the cerebellum and was associated with duration of illness, dysarthria and ataxia, and cerebellar atrophy. No remote effect of cerebellar hypoperfusion was found in the SCA6 patients.