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一名患有小脑共济失调的成人发生伴有可逆性胼胝体压部病变的轻度脑炎/脑病:病例报告

Mild Encephalitis/Encephalopathy with a Reversible Splenial Lesion in an Adult with Cerebellar Ataxia: A Case Report.

作者信息

Nakajima Masataka, Suda Satoshi, Kimura Kazumi

机构信息

Department of Neurology, Graduate School of Medicine, Nippon Medical School.

出版信息

J Nippon Med Sch. 2020;87(3):153-156. doi: 10.1272/jnms.JNMS.2020_87-305.

Abstract

Mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) is a clinicoradiological syndrome characterized by transient mild encephalopathy and magnetic resonance imaging (MRI) findings of a reversible lesion in the splenium of the corpus callosum (SCC). Patients with MERS generally present with central nervous system symptoms such as consciousness disturbance, headache, and seizure; adult-onset MERS with cerebellar ataxia is rare. A 53-year-old man was admitted to our hospital with fever of 1 week's duration, headache, neck stiffness, and gait disturbance. Neurological examination revealed bilateral intention tremor (predominantly affecting the right hand) and gait ataxia. Diffusion-weighted brain MRI showed a focal hyperintense lesion in the SCC. Cerebrospinal fluid analysis revealed elevated levels of mononuclear cells and proteins. Brain imaging with I-iofetamine single-photon emission computed tomography showed reduced cerebral blood flow in the left thalamus and right cerebellum. Several diseases, including cerebellar stroke and acute cerebellitis, develop as comorbidities in patients with acute cerebellar ataxia. This case suggests that MERS should be suspected in adults with cerebellar ataxia.

摘要

伴可逆性胼胝体压部病变的轻度脑炎/脑病(MERS)是一种临床放射学综合征,其特征为短暂性轻度脑病以及磁共振成像(MRI)显示胼胝体压部(SCC)存在可逆性病变。MERS患者通常表现出中枢神经系统症状,如意识障碍、头痛和癫痫发作;成人起病且伴有小脑共济失调的MERS较为罕见。一名53岁男性因持续1周的发热、头痛、颈部僵硬和步态障碍入院。神经系统检查发现双侧意向性震颤(主要影响右手)和步态共济失调。脑部弥散加权MRI显示SCC有局灶性高信号病变。脑脊液分析显示单核细胞和蛋白质水平升高。使用碘[I]异氟烷单光子发射计算机断层扫描进行脑成像显示左丘脑和右小脑的脑血流量减少。包括小脑卒中及急性小脑炎在内的几种疾病在急性小脑共济失调患者中作为合并症出现。该病例提示,对于患有小脑共济失调的成人应怀疑MERS。

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