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特发性和常染色体显性遗传性小脑共济失调中的扫视速度

Saccade velocity in idiopathic and autosomal dominant cerebellar ataxia.

作者信息

Bürk K, Fetter M, Skalej M, Laccone F, Stevanin G, Dichgans J, Klockgether T

机构信息

Department of Neurology, University of Tübingen, Germany.

出版信息

J Neurol Neurosurg Psychiatry. 1997 Jun;62(6):662-4. doi: 10.1136/jnnp.62.6.662.

Abstract

Slow saccades are often found in degenerative ataxia. Experimental studies have shown that horizontal saccades are generated in the paramedian pontine reticular formation and that lesions in this area produce slow saccades. Based on these findings, saccade slowing should be a frequent feature of olivopontocerebellar atrophy, a type of cerebellar degeneration with prominent involvement of the pons. To test this hypothesis, saccade velocity was measured in 31 patients with autosomal dominant cerebellar ataxia (ADCA) and 17 patients with idiopathic cerebellar ataxia (IDCA). Saccade velocity was reduced in most patients with ADCA whereas it was normal in IDCA although olivopontocerebellar atrophy occurred in both groups. Saccade velocities correlated with pontine size in ADCA but not in IDCA. The data disprove the hypothesis that saccadic slowing is a clinical hallmark of olivopontocerebellar atrophy. Instead, only patients with ADCA and morphological features of olivopontocerebellar atrophy have slow saccades.

摘要

慢扫视运动常见于退行性共济失调。实验研究表明,水平扫视运动由脑桥旁正中网状结构产生,该区域的损伤会导致慢扫视运动。基于这些发现,扫视减慢应是橄榄脑桥小脑萎缩的常见特征,橄榄脑桥小脑萎缩是一种小脑变性,脑桥受累明显。为验证这一假设,对31例常染色体显性小脑共济失调(ADCA)患者和17例特发性小脑共济失调(IDCA)患者的扫视速度进行了测量。大多数ADCA患者的扫视速度降低,而IDCA患者的扫视速度正常,尽管两组均出现了橄榄脑桥小脑萎缩。ADCA患者的扫视速度与脑桥大小相关,而IDCA患者则不然。这些数据反驳了扫视减慢是橄榄脑桥小脑萎缩临床标志的假设。相反,只有具有橄榄脑桥小脑萎缩形态特征的ADCA患者存在慢扫视运动。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/37ae/1074160/4908cd83942f/jnnpsyc00006-0116-a.jpg

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