Abstract:

Objective  To summarize the clinical manifestations, laboratory and MRI examinations, treatment and prognosis of 4 patients with human immunodeficiency virus (HIV)-associated progressive multifocal leukoencephalopathy (PML).  Methods  The clinical data of 4 patients with HIV-associated PML was retrospectively analyzed. Results  All of the 4 cases presented with progressive neurological function deficit and limb weakness. One case was accompanied by dysphasia, and one by dizziness. These symptoms got worsened as time went on. Cranial MRI revealed demyelination, hypointense signal in T₁WI, hyperintense signal in T₂WI and FLAIR. DWI revealed central hypointense and peripheral hyperintense signal. Enhanced scan revealed no contrast enhancement. Two cases were treated with highly active antiretroviral therapy (HAART), and the longest survival was 20 months. Patients who did not take standard anti-HIV therapies had a poor long-term prognosis. Conclusions  Patients with HIV-associated PML mainly present progressively worsened neurological function deficit. The imaging manifestations are typical. Early and timely HAART may play a role in the treatment.

Key words: HIV, Leukoencephalopathy, progressive multifocal, Antiretroviral therapy, highly active, Magnetic resonance imaging

摘要：

目的 总结人类免疫缺陷病毒（HIV）相关进行性多灶性白质脑病的临床表现、实验室和影像学特点、治疗及预后。方法 回顾分析4 例拟诊HIV 相关进行性多灶性白质脑病患者的临床资料。结果 4 例患者临床主要表现为逐渐加重的神经功能缺损症状、肢体无力，1 例伴言语困难、1 例伴头晕，病程中症状逐渐加重。头部MRI均表现为脱髓鞘改变，T₁WI呈低信号，T₂WI和FLAIR 成像呈高信号，DWI 呈中心低信号、周围高信号，增强扫描病灶未见明显强化。2 例行高效抗逆转录病毒疗法，最长生存期达20 个月；未行规范抗HIV 治疗者远期预后不佳。结论 HIV 相关进行性多灶性白质脑病临床以进行性加重的神经功能缺损症状为主，影像学表现典型，早期并及时予高效抗逆转录病毒疗法可以部分恢复。

关键词: HIV, 脑白质病, 进行性多灶性, 抗逆转录病毒治疗, 高效, 磁共振成像