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炎症性小脑进行性多灶性白质脑病,CD4/CD8 比值为 2.9,在一名类风湿关节炎患者中显示出良好预后。

Inflammatory Cerebellar PML with a CD4/CD8 Ratio of 2.9 Showed a Favorable Prognosis in a Patient with Rheumatoid Arthritis.

作者信息

Nishigori Ryusei, Warabi Yoko, Shishido-Hara Yukiko, Nakamichi Kazuo, Nakata Yasuhiro, Komori Takashi, Isozaki Eiji

机构信息

Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.

Department of Neurology, Kyoto University Graduate School of Medicine, Japan.

出版信息

Intern Med. 2019 Nov 15;58(22):3323-3329. doi: 10.2169/internalmedicine.3038-19. Epub 2019 Jul 31.

Abstract

The patient was a 74-year-old woman with rheumatoid arthritis who developed ataxia. MRI revealed T2-hyperintense lesions predominantly in the left middle cerebellar peduncle. Punctate or linear Gd enhancement was also observed on T1-weighted images. A brain biopsy was conducted and the pathology revealed a mild demyelinated lesion. Polymerase chain reaction (PCR) of biopsied brain tissues revealed the presence of JC virus (JCV) DNA, but JCV-infected oligodendroglia-like cells were not apparent on immunohistochemistry. Sensitive in-situ hybridization, however, detected three JCV-positive cells and the infiltration of CD4 and CD8 T cells and plasma cells was also observed. Immunosuppressants were tapered off and mirtazapine and mefloquine administered, resulting in a favorable outcome.

摘要

该患者为一名74岁患类风湿性关节炎的女性,出现共济失调。磁共振成像(MRI)显示T2高信号病变主要位于左侧小脑中脚。在T1加权图像上也观察到点状或线状钆增强。进行了脑活检,病理显示为轻度脱髓鞘病变。对活检脑组织进行聚合酶链反应(PCR)检测发现存在JC病毒(JCV)DNA,但免疫组织化学检查未发现JCV感染的少突胶质细胞样细胞。然而,敏感的原位杂交检测到三个JCV阳性细胞,并且还观察到CD4和CD8 T细胞以及浆细胞的浸润。逐渐减少免疫抑制剂的用量,并给予米氮平和甲氟喹,结果良好。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/626c/6911754/a7a6c0ea211a/1349-7235-58-3323-g001.jpg

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