Department of Neurology, Kyoto University Graduate School of Medicine Faculty of Medicine, Kyoto, Japan
Department of Neurology, Kobe City Medical Center General Hospital, Osaka, Japan.
BMJ Case Rep. 2024 Apr 30;17(4):e257805. doi: 10.1136/bcr-2023-257805.
A middle-aged man with progressive multifocal leukoencephalopathy (PML) in a human T-cell lymphotropic virus type-1 (HTLV-1) carrier on haemodialysis presented with mild dysarthria and ataxia. Brain MRI revealed asymmetric T-hyperintense lesions in the cerebral white matter, cerebellum and brainstem. A small amount of JC virus (JCV) genome in cerebrospinal fluid was detected by PCR and cerebellar biopsy demonstrated JCV-DNA presence. Pathological findings showed demyelinating lesions and glial cells with mildly enlarged nuclei, accompanied by T-lymphocytes, neutrophils and plasma cell infiltration. The CD4+/CD8+ratio was 0.83. High-dose corticosteroid therapy was effective for inflammatory PML lesions, and the administration of mefloquine combined with mirtazapine led to favourable outcome. The encephalitis in this case is considered to have occurred secondarily to JCV infection in the presence of HTLV-1 infection. Therefore, it is crucial to investigate the presence of HTLV-1 in order to understand the aetiology of this brain inflammation.
一名中年男性,为人类 T 细胞嗜淋巴细胞病毒 1 型(HTLV-1)携带者,正在接受血液透析,患有进行性多灶性脑白质病(PML),表现为轻度构音障碍和共济失调。脑部 MRI 显示大脑白质、小脑和脑干存在非对称 T 高信号病变。PCR 检测到少量脑脊液中的 JC 病毒(JCV)基因组,小脑活检显示 JCV-DNA 存在。病理发现脱髓鞘病变和细胞核轻度增大的神经胶质细胞,伴有 T 淋巴细胞、中性粒细胞和浆细胞浸润。CD4+/CD8+比值为 0.83。大剂量皮质类固醇治疗对炎症性 PML 病变有效,联合使用甲氟喹和米氮平治疗取得了良好的效果。本例脑炎被认为是在存在 HTLV-1 感染的情况下继发于 JCV 感染。因此,调查 HTLV-1 的存在对于了解这种脑炎症的病因至关重要。