Wernekink连合综合征3例临床分析并文献复习  

The Clinical Analysis of 3 Cases of Wernekink Commissure Syndrome and Review of the Literature

作　　者：郝悦含[1] 伍博晰[2] 律青 赵丽宏 何志义[1] 

机构地区：[1]中国医科大学附属第一医院神经内科,110000 [2]沈阳市第四人民医院眼科,110000 [3]丹东市人民医院神经内科,118000

出　　处：《中国临床神经科学》2016年第5期514-518,共5页Chinese Journal of Clinical Neurosciences

摘　　要：目的探讨Wernekink连合综合征的临床表现、影像学特点及鉴别诊断,以提高临床医生对Wernekink连合综合征的认识。方法收集3例Wernekink连合综合征患者的临床资料,结合国外文献报道9例、国内文献报道6例病例资料,对共计18例患者的临床表现、影像学特点、鉴别诊断等进行复习、讨论。结果 18例Wernekink连合综合征患者均出现双侧小脑共济失调,其中表现为肢体、躯干共济失调18例,构音障碍17例;出现眼肌麻痹11例,其中动眼神经麻痹4例,核间性眼肌麻痹9例;出现腭肌阵挛3例。18例患者的影像学检查均表现为中脑下部导水管前方梗死灶。需要与Wernekink连合征相鉴别的疾病包括急性小脑炎、多发性硬化、Fisher综合征、热射病等。结论 Wernekink连合综合征非常少见,主要表现为双侧小脑性共济失调、眼球运动障碍、腭肌阵挛。共济失调是中脑梗死最常见的症状。Aim To investigate the clinical manifestations, radiological features and differential diagnosis of Wernekink commissure syndrome, in order to improve clinician awareness of the syndrome. Methods Three Wernekink commissure syndrome cases admitted to our department with clinical data were analyzed, the literature were reviewed, and the clinical manifestations, imaging features and differential diagnosis were discussed. Results At present, 9 cases reported abroad, 6 cases reported domestically, and 3 cases treated in our department, a total of 18 cases. Manifesting bilateral cerebellar ataxia were 18 cases, which showed the limbs and trunk ataxia 18 cases and dysarthria 17 cases. Manifesting ophthalmoplegia were 11 cases, including 4 cases of oculomotor nerve palsy and 9 cases of nuclear ophthalmoplegia. Manifesting palatal myoclonus were 3 cases. Imaging of the 18 cases all showed infarctions in front of the lower part of the midbrain aqueduct. Diseases distinguished with this syndrome include acute encephalitis, multiple sclerosis, Fisher syndrome, heat stroke, etc. Conclusion Wernekink commissure syndrome is very rare, mainly manifesting bilateral cerebellar ataxia, eye movement disorders, and palatal myoclonus. Ataxia is the most common symptom of midbrain infarction.

关 键 词：Wernekink连合综合征 中脑梗死 双侧小脑性共济失调 

分 类 号：R743[医药卫生—神经病学与精神病学]