Department of Neurology, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Ten'nudai 1-1-1, Tsukuba, Ibaraki 305-8575, Japan.
Department of Neurosurgery, Division of Clinical Medicine, Faculty of Medicine, University of Tsukuba, Ten'nudai 1-1-1, Tsukuba, Ibaraki 305-8575, Japan.
Mult Scler Relat Disord. 2018 Feb;20:159-163. doi: 10.1016/j.msard.2018.01.017. Epub 2018 Jan 31.
CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory central nervous system disorder that gives rise to brainstem symptoms such as diplopia and articulatory disorders. It shows characteristic "salt-and-pepper" enhancing brainstem lesions on magnetic resonance imaging (MRI) scans. We present an unusual case of CLIPPERS, in which a 29-year-old man initially presented with a seizure. Brain MRI revealed faint open-ring enhancements in the right frontal lobe, and a brain biopsy was performed to make a definitive diagnosis. Immunohistological tests showed perivascular infiltration by primarily CD3- and CD20-positive cells, albeit including a few CD68-positive cells, which confirmed a diagnosis of CLIPPERS. Both the symptoms and the MRI findings were markedly improved by 3 courses of steroid (methylprednisolone) pulse therapy, followed by oral steroid (prednisolone) administration. This case shows that CLIPPERS can present as a phenotype of cortical lesions and seizures as the first signs. A brain biopsy should therefore be actively carried out for differential diagnosis, especially when other, cortically based inflammatory disorders are suspected.
CLIPPERS（伴有桥脑血管周围强化的慢性淋巴细胞炎症对类固醇有反应）是一种炎症性中枢神经系统疾病，可引起脑干症状，如复视和构音障碍。它在磁共振成像（MRI）扫描上显示出特征性的“椒盐”增强脑干病变。我们报告了一例不典型的 CLIPPERS 病例，一名 29 岁男性最初表现为癫痫发作。脑部 MRI 显示右额叶有微弱的开环增强，为明确诊断进行了脑部活检。免疫组织化学测试显示主要由 CD3 和 CD20 阳性细胞浸润，但也包括一些 CD68 阳性细胞，这证实了 CLIPPERS 的诊断。3 个疗程的类固醇（甲基强的松龙）脉冲治疗后，症状和 MRI 表现明显改善，随后口服类固醇（泼尼松龙）治疗。该病例表明，CLIPPERS 可表现为皮质病变和癫痫发作的表型作为首发症状。因此，对于鉴别诊断，特别是怀疑存在其他皮质炎症性疾病时，应积极进行脑部活检。
