Clinical Profile and Outcome of Progressive Multifocal Leukoencephalopathy in HIV Infected Indian Patients.

BACKGROUND AND OBJECTIVE: Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease of the Central nervous system (CNS) caused by the human polyoma virus JC (JCV). Human Immunodeficiency Virus (HIV) infection predisposes to PML. Very sparse data is available regarding the effect of Highly Active Anti Retroviral Therapy (HAART) on clinical outcome of PML in Indian settings. This study was carried out to look into clinical profile, survival and neurological outcome of HIV infected PML patients in HAART era.

METHODS: We looked in our cohort of HIV-1-infected individuals retrospectively. Diagnosis of PML was done on basis of clinical and radiological abnormalities highly suggestive of the condition, with or without confirmation of JCV DNA in the cerebrospinal fluid (CSF) by polymerase chain reaction (PCR).

RESULTS: Out of 892 HIV infected patients, 31 met the criteria for the diagnosis of PML. The median CD4+ cell count was 73 cells/µL (Interquartile range (IQR), 43-160 cells/µL) at the presentation of PML. Median duration of PML symptoms were 30 days (IQR, 15-60 days) before diagnosis of PML could be made. The median survival was 538 days. In those patients who survived more than one year, the median survival time was 1095 days (95% confidence interval (CI), 1090.35 -1099.64 days). Those who survived more than one year (n=13), Neurologic function were categorized as cure or improvement in 8, same status in 3 or progression in 2 patients.

CONCLUSIONS: In the pre-HAART era, PML patients had very poor prognosis with median survival of 4-6 months after diagnosis. Till date HAART is the only way for reversal of immune system in HIV infected patients and its prompt institution is the most effective therapeutic approach in increasing survival in this group. In this study, 46.4% patients survived after 1 year on HAART. Amongst them, 69% patients completed 3 years. There is strong need of research for the development of pharmacotherapy against JC virus to increase the survival.