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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。
Objective To investigate the clinical manifestations, imaging features and differential diagnosis of Wernekink’s conjunctival syndrome in order to improve clinicians’ understanding of Wernekink’s conjunctival syndrome. Methods The clinical data of 3 patients with Wernekink’s conjunctive syndrome were collected. The data of 9 cases reported in foreign literature and 6 cases reported in domestic literature were collected. The clinical manifestations, imaging features and differential diagnosis of 18 patients were reviewed and discussed. Results 18 patients with Wernekink’s conjunctival syndrome both showed bilateral cerebellar ataxia, which manifested as limbs, trunk ataxia in 18 cases, dysarthria in 17 cases; ophthalmoplegia in 11 cases, including oculomotor nerve palsy in 4 cases , 9 cases of intraocular ophthalmoplegia; 3 cases of palatal myoclonus. Eighteen patients underwent imaging studies showing infarcts in front of the midbrain lower aqueduct. Diseases that require identification with Wernekink include acute cerebritis, multiple sclerosis, Fisher’s syndrome, thermal disease and the like. Conclusions Wernekink’s conjunctival syndrome is very rare and is mainly manifested as bilateral cerebellar ataxia, eye movement disorders and palatal myoclonus. Ataxia is the most common symptom of cerebral infarction.