Lee Ming-Hsing, Chen Yan-Zhong, Wang Lih-Shinn, Yen Pao-Sheng, Hsu Yung-Hsiang
Department of Pathology, Tzu-Chi University and Buddhist Tzu-Chi General Hospital, Hualien, Taiwan.
J Formos Med Assoc. 2007 Mar;106(3 Suppl):S24-8. doi: 10.1016/s0929-6646(09)60362-4.
Progressive multifocal leukoencephalopathy (PML) is a demyelinating brain disease caused by Jamestown Canyon virus (JCV). This disease is an important cause of morbidity and mortality in acquired immunodeficiency syndrome (AIDS) patients. We report a 34-year-old man infected with HIV-1 who presented with frequent general tonic clonic seizure and left side weakness for 2 months. Clinical features and magnetic resonance imaging (MRI) findings with hyperintensity on T2-weighted imaging and low density on T2 fluid attenuated inversion recovery involving multiple white matter were compatible with PML. He died of sepsis 2 months after diagnosis. Autopsy demonstrated progressive multifocal leukoencephalopathy according to characteristic histopathologic picture with multifocal demyelination, bizarre astrocytes formation and basophilic intranuclear inclusion bodies in the oligodendrocytes. JCV genome was demonstrated in the nucleus of oligodendrocytes using in situ hybridization. In conclusion, in AIDS patients with neurologic signs and typical MRI findings who present with multifocal demyelination lesions, PML should be diagnosed clinically.
进行性多灶性白质脑病(PML)是一种由詹姆斯敦峡谷病毒(JCV)引起的脱髓鞘性脑病。该疾病是获得性免疫缺陷综合征(AIDS)患者发病和死亡的重要原因。我们报告了一名34岁感染HIV-1的男性,他出现频繁的全身强直阵挛发作和左侧肢体无力2个月。临床特征以及磁共振成像(MRI)表现为T2加权像高信号、T2液体衰减反转恢复序列低密度,累及多个白质区域,这些表现与PML相符。诊断后2个月,他死于败血症。尸检根据特征性组织病理学表现证实为进行性多灶性白质脑病,表现为多灶性脱髓鞘、奇异星形胶质细胞形成以及少突胶质细胞中的嗜碱性核内包涵体。使用原位杂交在少突胶质细胞核中检测到JCV基因组。总之,对于出现多灶性脱髓鞘病变、有神经系统症状且MRI表现典型的AIDS患者,临床上应诊断为PML。