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八又二分之一综合征作为一种临床孤立综合征的罕见病例。

Rare occurrence of eight-and-a-half syndrome as a clinically isolated syndrome.

作者信息

Jacob Sneha, Murray Ann

机构信息

Department of Neurology, West Virginia University Health Sciences Center, Morgantown, West Virginia, USA.

出版信息

BMJ Case Rep. 2018 Jan 26;2018:bcr-2017-222057. doi: 10.1136/bcr-2017-222057.

Abstract

Eight-and-a-half syndrome is a rare condition that is described as a combination of one-and-a-half syndrome and an ipsilateral facial nucleus lesion. We present a clinical case of occurrence of eight-and-a-half syndrome that was caused by a demyelinating lesion in the dorsal pontine tegmentum. A 44-year-old man presented to the hospital with a subacute onset of horizontal diplopia and left-sided facial weakness. MRI revealed a T2 hyperintense lesion in his dorsal pons, which was consistent with a demyelinating pathology. Treatment with intravenous steroids showed significant improvement in his symptoms. In our case, it occurred due to a suspected demyelinating lesion that was this patient's first and only demyelinating event, leaving him with a diagnosis of clinically isolated syndrome. His responsiveness to steroids represents the first case report of an adult patient presenting with an eight-and-a-half syndrome secondary to a suspected demyelinating pathology.

摘要

八又二分之一综合征是一种罕见疾病,被描述为一个半综合征与同侧面神经核损害的组合。我们报告一例由脑桥背侧被盖区脱髓鞘病变引起的八又二分之一综合征临床病例。一名44岁男性因亚急性起病的水平性复视和左侧面部无力入院。MRI显示其脑桥背侧T2高信号病变,符合脱髓鞘病理改变。静脉注射类固醇治疗后其症状显著改善。在我们的病例中,该综合征是由一个疑似脱髓鞘病变引起的,这是该患者首次也是唯一的脱髓鞘事件,最终他被诊断为临床孤立综合征。他对类固醇的反应代表了首例报告的成年患者因疑似脱髓鞘病理改变而出现八又二分之一综合征。

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