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韦尼克连合综合征4例临床分析

Clinical analysis of 4 cases of Wernekink commissure syndrome.

作者信息

Xu Li, Li Yuyu, Xie Jing, Hu Mingjie, Qu Hongdang, Liu Xiaolin

机构信息

Department of Neurology, First Affiliated Hospital, Bengbu Medical College, Bengbu Anhui 233020, China.

School of Life Sciences, Bengbu Medical College, Bengbu Anhui 233020, China.

出版信息

Zhong Nan Da Xue Xue Bao Yi Xue Ban. 2020 Apr 28;45(4):469-475. doi: 10.11817/j.issn.1672-7347.2020.190030.

Abstract

Wernekink commissure syndrome (WCS) is very rare. Four patients with WCS, admitted to our hospital from April to May 2018, were chosen for this study, and their clinical manifestations, imaging features, and etiology were retrospectively analyzed based on the literatures. All patients with WCS manifested as bilateral cerebellar ataxia such as symmetrical limb and trunk ataxia, but the degree of ataxia was asymmetrical distribution based on the anatomy. Dysarthria was the main and constant clinical manifestation of the syndrome. Ophthalmoplegia had great variability, and WCS with oculomotor nerve palsy may be considered as atypical WCS. The incidence of olivary degeneration and palatine myoclonus is relatively low, which may be related to the difference in the reported time intervals of cases. Changes in consciousness and emotion may be the characteristic of neglected WCS, which should be paid more attention. Cerebral infarction is the main etiology of WCS. We reported that cerebral infarction and WCS was the first symptom in a patient with systemic lupus erythematosus (SLE). We should pay more attention to special etiology in diagnosis and treatment of WCS.

摘要

韦尼克连合综合征(WCS)非常罕见。选取2018年4月至5月我院收治的4例WCS患者进行本研究,并结合文献对其临床表现、影像学特征及病因进行回顾性分析。所有WCS患者均表现为双侧小脑性共济失调,如肢体和躯干对称性共济失调,但共济失调程度基于解剖结构呈不对称分布。构音障碍是该综合征主要且持续存在的临床表现。眼肌麻痹差异较大,伴有动眼神经麻痹的WCS可被视为非典型WCS。橄榄体变性和腭肌阵挛的发生率相对较低,这可能与病例报告的时间间隔差异有关。意识和情绪变化可能是被忽视的WCS的特征,应予以更多关注。脑梗死是WCS的主要病因。我们报告了1例系统性红斑狼疮(SLE)患者以脑梗死和WCS为首发症状。在WCS的诊断和治疗中,我们应更多关注特殊病因。

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