National Institute of Mental Health and Neurosciences, Bangalore, Karnataka, India.
J Neurovirol. 2013 Jun;19(3):198-208. doi: 10.1007/s13365-013-0168-8. Epub 2013 May 23.
Progressive multifocal leukoencephalopathy (PML) is a rare, subacute, demyelinating disease of the central nervous system caused by JC virus. Studies of PML from HIV Clade C prevalent countries are scarce. We sought to study the clinical, neuroimaging, and pathological features of PML in HIV Clade C patients from India. This is a prospective cum retrospective study, conducted in a tertiary care Neurological referral center in India from Jan 2001 to May 2012. Diagnosis was considered "definite" (confirmed by histopathology or JCV PCR in CSF) or "probable" (confirmed by MRI brain). Fifty-five patients of PML were diagnosed between January 2001 and May 2012. Complete data was available in 38 patients [mean age 39 ± 8.9 years; duration of illness-82.1 ± 74.7 days). PML was prevalent in 2.8 % of the HIV cohort seen in our Institute. Hemiparesis was the commonest symptom (44.7 %), followed by ataxia (36.8 %). Definitive diagnosis was possible in 20 cases. Eighteen remained "probable" wherein MRI revealed multifocal, symmetric lesions, hypointense on T1, and hyperintense on T2/FLAIR. Stereotactic biopsy (n = 11) revealed demyelination, enlarged oligodendrocytes with intranuclear inclusions and astrocytosis. Immunohistochemistry revelaed the presence of JC viral antigen within oligodendroglial nuclei and astrocytic cytoplasm. No differences in clinical, radiological, or pathological features were evident from PML associated with HIV Clade B. Clinical suspicion of PML was entertained in only half of the patients. Hence, a high index of suspicion is essential for diagnosis. There are no significant differences between clinical, radiological, and pathological picture of PML between Indian and Western countries.
进行性多灶性白质脑病（PML）是一种罕见的亚急性中枢神经系统脱髓鞘疾病，由 JC 病毒引起。关于 HIV Clade C 流行国家 PML 的研究很少。我们旨在研究来自印度的 HIV Clade C 患者的 PML 的临床、神经影像学和病理学特征。这是一项在印度一家三级神经转诊中心进行的前瞻性和回顾性研究，时间为 2001 年 1 月至 2012 年 5 月。诊断被认为是“明确”（通过组织病理学或 CSF 中的 JCV PCR 证实）或“可能”（通过 MRI 脑证实）。在 2001 年 1 月至 2012 年 5 月期间，诊断出 55 例 PML 患者。38 例患者有完整的数据[平均年龄 39±8.9 岁；疾病持续时间 82.1±74.7 天]。我们研究所看到的 HIV 队列中，PML 的患病率为 2.8%。偏瘫是最常见的症状（44.7%），其次是共济失调（36.8%）。20 例可明确诊断。18 例仍为“可能”，MRI 显示多灶性、对称病变，T1 低信号，T2/FLAIR 高信号。立体定向活检（n=11）显示脱髓鞘，大的少突胶质细胞，核内包涵体和星形胶质细胞增生。免疫组化显示寡突胶质细胞核和星形胶质细胞质内存在 JC 病毒抗原。与 HIV Clade B 相关的 PML 在临床、放射学或病理学特征上没有明显差异。只有一半的患者有 PML 的临床怀疑。因此，高度怀疑是诊断所必需的。印度和西方国家的 PML 临床、放射学和病理学表现没有显著差异。
