Progressive multifocal leukoencephalopathy and immune reconstitution inflammatory syndrome in an HIV patient with favorable outcome using combination of antiretroviral therapy and systemic corticosteroids: A case report

Abstract

Progressive multifocal leukoencephalopathy (PML) is a devastating, progressive disease characterized by multifocal demyelination. Reactivation of a polyomavirus, JC virus (JCV), is a major cause of PML, inducing a noninflammatory, lytic reaction, demyelination, necrosis, and cell death. Immune reconstitution inflammatory syndrome associated with progressive multifocal leukoencephalopathy (PML-IRIS), a paradoxical neurological deterioration, despite dramatic improvement in CD4 and viral load, has been reported following initiation of "Highly active antiretroviral therapy" (HAART) in 18% of patients. Patients present with visual deficits (45%), decline in cognitive skills, confusion, personality change (38%); weakness, including hemi- or monoparesis; and ataxia and seizures (20%). Some patients develop PML during immune reconstitution. Survival is very poor in patients with CD4+ T-cell count < 200 cells/mmł. No definitive treatment has been established. Here we describe the diagnosis and management of a 50-year-old Afro-Caribbean man who was found to have AIDS, treated with antiretroviral regimen but soon develop worsening of neurological symptoms and poor functional performance status. He was found to have PML on brain MRI and JC virus on CSF fluid analysis. His course was further complicated with immune reconstitution inflammatory syndrome (IRIS). Patient responded dramatically to HAART with good CNS penetration along with adjuvant corticosteroids treatment. After 24-month follow-up, patient is alive with stable disease. © 2012 Hilli JA, et al.