Troppmann Martina, Büttner Roland, Boewer Michael, Salzberger Bernd
Department of Internal Medicine I, University of Regensburg, Regensburg, Germany.
J Med Case Rep. 2012 Jul 2;6:177. doi: 10.1186/1752-1947-6-177.
Progressive multifocal leukoencephalopathy is an opportunistic infection occurring in patients with severe cellular immunodeficiency. This case highlights the role of cellular immunodeficiency in the reactivation of John Cunningham virus in a case of an early stage plasmacytoma.
A 76-year-old Caucasian woman presented with progressive left-sided hemiparesis, accompanied by hypoesthesia, hypoalgesia and neuropsychological symptoms. Magnetic resonance imaging demonstrated new hyperattenuating lesions in the right thalamus and left-sided subcortically. A polymerase chain reaction test revealed 4500 copies of John Cunningham virus-deoxyribonucleic acid/ml in cerebrospinal fluid. Human immunodeficiency virus infection was ruled out. A bone marrow biopsy showed an early stage immunoglobulin G-kappa plasmacytoma. Cidofovir (5mg/kg) weekly for three weeks was started. A significant improvement of her neuropsychological symptoms was achieved, but motor system and sensory symptoms did not change.
This case shows a rapid course of progressive multifocal leukoencephalopathy with severe residual deficits. In the diagnostic workup of all patients with atypical neurologic symptoms or immunodeficiency, progressive multifocal leukoencephalopathy should be included as a differential diagnosis.
进行性多灶性白质脑病是一种发生于严重细胞免疫缺陷患者的机会性感染。本病例强调了细胞免疫缺陷在一例早期浆细胞瘤患者中导致约翰·坎宁安病毒再激活中的作用。
一名76岁的白人女性出现进行性左侧偏瘫，伴有感觉减退、痛觉减退和神经心理症状。磁共振成像显示右侧丘脑和左侧皮质下有新的高密度病变。聚合酶链反应检测显示脑脊液中约翰·坎宁安病毒脱氧核糖核酸含量为4500拷贝/毫升。排除了人类免疫缺陷病毒感染。骨髓活检显示为早期免疫球蛋白G-κ浆细胞瘤。开始每周给予西多福韦（5毫克/千克），共三周。其神经心理症状有显著改善，但运动系统和感觉症状未改变。
本病例显示进行性多灶性白质脑病病程进展迅速，遗留严重功能缺损。在所有有非典型神经症状或免疫缺陷患者的诊断检查中，应将进行性多灶性白质脑病纳入鉴别诊断。
