Slowly progressive fatal PML-IRIS following antiretroviral initiation at CD4+ nadir of 350 cells/mm3 despite CD4+ cell count rise to 900 cells/mm3
AIDS-related progressive multifocal leukoencephalopathy (PML)-immune reconstitution inflammatory syndrome (IRIS) is a central nervous system inflammatory syndrome where immune response to John Cunningham (JC) virus antigen following antiretroviral therapy (ART) causes breakdown of the blood–brain barrier. We report a unique case of PML-IRIS, which presented with dystonic choreoathetosis after initiation of ART at a CD4+ cell count of 350 cells/mm3. This report shows continuous progression of the disease over a period of two years, despite robust immune reconstitution. The worsening of neurological symptoms, persistent positivity of JC virus in CSF, and progressive inflammatory picture on MR scans in the setting of a CD4+ cell count of 900 cells/mm3 highlight a different variant of PML-IRIS, and challenge the role of CD4+ cell count in diagnosing opportunistic infections in HIV/AIDS patients.