Study of clinical profile and outcomes in progressive multifocal leukoencephalopathy in acquired immunodeficiency syndrome patients in the highly active antiretroviral therapy era - An observational study

Background and Objectives: Progressive multifocal leukoencephalopathy (PML) is a viral infection affecting the central nervous system (CNS) seen mostly in advanced human immunodeficiency virus (HIV) infection. There is limited data on the epidemiology and disease course of these patients from India. This study was aimed to determine the frequency of PML in patients with HIV/acquired immunodeficiency syndrome (AIDS) and the clinical presentation and prognosis of these patients. Materials and Methods: The study was conducted at a tertiary care HIV center in New Delhi. Data of 765 patients from our anti-retroviral therapy (ART) clinic during a span of 4 years were retrospectively analyzed and reviewed. The diagnosis was based on the clinical and radiological picture and exclusion of other differential diagnosis by cerebrospinal fluid and serological studies. Results: Of 765 patients with HIV/AIDS, 12 (1.56%) were diagnosed with PML on the basis of consistent clinical and radiological features after ruling out other differential diagnosis. PML was the initial presentation of HIV infection in 8 (55.5%) patients. 11 (89%) patients had CD4 count <200/mu l. Insidious onset focal limb weakness (50%) and dysarthria (50%) were common symptoms. Magnetic resonance imaging of the brain revealed characteristic white matter lesions in all the patients. The estimated median survival was 40 months (95% confidence interval, 23.88-53.19 months). Interpretation and Conclusions: Our results show that PML is associated with high morbidity despite the institution of highly active ART (HAART), but mortality has significantly declined if ART is started early. Key to good response is early diagnosis and HAART.