Tsuchida Takumi, Ura Shigehisa, Yabe Ichiro
Department of Emergency Medicine, Hokkaido University Hospital, Sapporo, Japan.
Department of Neurology, Asahikawa Red Cross Hospital, Asahikawa, Japan.
Case Rep Neurol. 2023 Mar 15;15(1):48-53. doi: 10.1159/000529121. eCollection 2023 Jan-Dec.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly affects the brainstem. Apart from corticosteroids, there are few reported treatment options for CLIPPERS, and there is no standard therapy. A 77-year-old man presented with diplopia that had persisted for 5 months. Dysarthria and numbness of the distal right upper extremity and right lips were also observed. Brain magnetic resonance imaging (MRI) revealed a hyperintense area around the brainstem. Symptoms were relieved immediately following intravenous methylprednisolone (IVMP) administration. However, after gradual tapering of oral prednisolone to 5 mg/day, the symptoms relapsed, and brain imaging revealed that the condition had worsened. Intravenous immunoglobulins (IVIg) were administered for recurrence, with no clinical improvement. After each IVMP treatment, the patient recovered promptly. Based on the patient's symptoms and characteristic MRI findings, exclusion of other diseases, and the significant efficacy of corticosteroids, he was diagnosed with CLIPPERS. There was no recurrence at a maintenance prednisolone dose of 8 mg/day. IVIg had a poor effect on the acute phase of CLIPPERS symptoms. Compared with other immunosuppressants, IVIg is less effective in suppressing the relapse of CLIPPERS.
类固醇反应性桥脑周围血管强化的慢性淋巴细胞性炎症（CLIPPERS）是一种主要影响脑干的中枢神经系统炎性疾病。除皮质类固醇外，关于CLIPPERS的报道的治疗选择很少，且尚无标准疗法。一名77岁男性出现复视，持续5个月。还观察到构音障碍以及右上肢远端和右唇部麻木。脑部磁共振成像（MRI）显示脑干周围有高信号区。静脉注射甲泼尼龙（IVMP）后症状立即缓解。然而，口服泼尼松龙逐渐减量至5毫克/天后，症状复发，脑部影像学检查显示病情恶化。复发时给予静脉注射免疫球蛋白（IVIg），但临床症状未改善。每次IVMP治疗后，患者均迅速康复。根据患者的症状、特征性MRI表现、排除其他疾病以及皮质类固醇的显著疗效，他被诊断为CLIPPERS。泼尼松龙维持剂量为8毫克/天时未复发。IVIg对CLIPPERS症状急性期效果不佳。与其他免疫抑制剂相比，IVIg在抑制CLIPPERS复发方面效果较差。
