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[经反复脑脊液 JC 病毒检测确诊的双侧小脑桥脑臂病变的进行性多灶性白质脑病及 JC 病毒颗粒细胞神经元病共存。1 例报告]

[Progressive multifocal leukoencephalopathy with bilateral middle cerebellar peduncle lesions confirmed by repeated CSF-JC virus tests and coexistence of JC virus granule cell neuronopathy. Report of a case].

作者信息

Ito Daisuke, Yasui Keizo, Hasegawa Yasuhiro, Nakamichi Kazuo, Katsuno Masahisa, Takahashi Akira

机构信息

Department of Neurology, Japan Red Cross Nagoya Daini Hospital.

出版信息

Rinsho Shinkeigaku. 2016 Jul 28;56(7):481-5. doi: 10.5692/clinicalneurol.cn-000873. Epub 2016 Jun 30.

DOI:10.5692/clinicalneurol.cn-000873
PMID:27356732
Abstract

A 65 year-old woman with small lymphocytic leukemia presented with subacute cerebellar ataxia. Six months after rituximab chemotherapy, a cranial MRI revealed lesions in the bilateral middle cerebellar peduncles. Both cerebrospinal fluid (CSF) JC virus (JCV)-DNA PCR test on three occasions and brain biopsy were negative. CSF tests were repeated. The fourth test performed 6 months after the onset showed positive JCV-DNA, and a definite diagnosis of progressive multifocal leukoencephalopathy (PML) was made. Neuroimaging of cerebellar atrophy was considered to be coexistence of granule cell neuronopathy. Medication with mirtazapine and mefloquine was temporarily effective for several months. Little are known solitary bilateral MRI lesions of the middle cerebellar peduncle in PML. JCV-PCR test of CSF may be negative at an earlier stage of PML. Repeated CSF tests should be essential to confirming the diagnosis in such cases.

摘要

一名65岁的小淋巴细胞白血病女性患者出现亚急性小脑共济失调。利妥昔单抗化疗6个月后,头颅磁共振成像(MRI)显示双侧小脑中脚有病变。三次脑脊液(CSF)JC病毒(JCV)-DNA聚合酶链反应(PCR)检测及脑活检均为阴性。重复进行脑脊液检测。发病6个月后进行的第四次检测显示JCV-DNA呈阳性,从而明确诊断为进行性多灶性白质脑病(PML)。小脑萎缩的神经影像学表现被认为是颗粒细胞神经元病的共存。米氮平和甲氟喹治疗数月有暂时疗效。PML中孤立的双侧小脑中脚MRI病变鲜为人知。PML早期脑脊液JCV-PCR检测可能为阴性。对于此类病例,重复进行脑脊液检测对于确诊至关重要。

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