摘要
Wernekink连合综合征(Wernekink commissure syndrome,WCS)临床少见,经典表现为双侧小脑性共济失调、显著的构音障碍及核间性眼肌麻痹。2018年4至5月蚌埠医学院第一附属医院收治4例WCS患者,现结合国内外文献报道,对其病因、临床表现和影像学特征等进行探讨。WCS均有双侧小脑性共济失调表现,主要为躯干及对称性肢体共济失调,但在程度上可呈左右非对称性分布,笔者基于解剖学提出了分型假说;构音障碍是WCS主要而恒定的临床表现;眼肌麻痹的临床表现变异性较大,出现动眼神经麻痹时提示病变超出经典WCS范畴,可认为是非典型性WCS;下橄榄核变性及腭肌阵挛发生率较低,可能与病例报道距发病的时间长短有关;意识改变及情绪变化可能是被忽略的WCS特征性表现,应加以重视。脑梗死是WCS的主要病因,但本组有1例系统性红斑狼疮引发脑梗死且首发症状为WCS的患者,这提示在WCS的诊断和治疗中应重视特殊病因。
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.
作者
许力
李玉玉
谢静
胡明洁
屈洪党
刘晓林
XU Li;LI Yuyu;XIE Jing;HU Mingjie;QU Hongdang;LIU Xiaolin(Department of Neurology,First Affiliated Hospital,Bengbu Medical College,Bengbu Anhui 233020,China;School of Life Sciences,Bengbu Medical College,Bengbu Anhui 233020,China)
出处
《中南大学学报（医学版）》
CAS
CSCD
北大核心
2020年第4期469-475,共7页
Journal of Central South University ：Medical Science
基金
安徽省高校人文社会科学研究重点项目(SK2018A0181)
蚌埠医学院科技发展基金(BYKF1797)。
