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局限于小脑和脑干的进行性多灶性白质脑病伴特发性CD4(+) T淋巴细胞减少症

Progressive Multifocal Leukoencephalopathy Localized in the Cerebellum and Brainstem Associated with Idiopathic CD4(+) T Lymphocytopenia.

作者信息

Aotsuka Yuya, Uzawa Akiyuki, Nishimura Kazutaka, Kojima Kazuho, Yamaguchi Mika, Makino Takahiro, Nakamichi Kazuo, Saijo Masayuki, Kuwabara Satoshi

机构信息

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

出版信息

Intern Med. 2016;55(12):1645-7. doi: 10.2169/internalmedicine.55.6649. Epub 2016 Jun 15.

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease that favors the cerebrum and typically occurs in immunosuppressed patients. We herein report the case of a 66-year-old man with PML, idiopathic CD4(+) T lymphocytopenia (ICL), and chronic renal failure. Cranial magnetic resonance imaging (MRI) showed a crescent-shaped lesion in the left cerebellum, brainstem, and middle cerebellar peduncle. Although the patient did not present with HIV infection, collagen diseases, or tumors, JC virus DNA was detected in the cerebrospinal fluid. Clinicians should consider PML and ICL in the differential diagnosis if the patient develops progressive ataxia and a crescent-shaped cerebellar lesion on MRI.

摘要

进行性多灶性白质脑病(PML)是一种脱髓鞘疾病,好发于大脑,通常发生在免疫抑制患者中。我们在此报告一例66岁患有PML、特发性CD4(+) T淋巴细胞减少症(ICL)和慢性肾衰竭的男性病例。头颅磁共振成像(MRI)显示左侧小脑、脑干和小脑中脚有新月形病变。尽管该患者未出现HIV感染、胶原病或肿瘤,但在脑脊液中检测到了JC病毒DNA。如果患者出现进行性共济失调且MRI显示有新月形小脑病变,临床医生在鉴别诊断时应考虑PML和ICL。

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