[Case of highly active anti-retroviral therapy-induced immune reconstitution inflammatory syndrome in AIDS-related progressive multifocal leukoencephalopathy]

Abstract

A 44-year-old man presented with difficulties in gripping a ball with the left hand upon playing tennis. He developed muscle weakness involving the left limbs, as well as memory decline, with subsequent gradual worsening of such symptoms. Cranial MRI showed multiple high intensity lesions in the white matter on T2-weighted imaging and FLAIR imaging. Serologic testing was positive for HIV infection and laboratory studies revealed a CD4+T cell count of 103 cells/microl and a plasma HIV-1 RNA load of 240,000 copies/ml. Accordingly, the diagnosis of AIDS was made. Although the initial result of DNA amplification by PCR for detection of the JC virus with CSF was negative, PML was suspected because of the presence of multifocal white matter lesions. Four weeks after the commencement of highly active anti-retroviral therapy (HAART), the HIV-1 RNA load was decreased from 680,000 to 480 copies/ml, although the CD4+T cell count was unchanged. JC virus DNA became positive at the second examination. Five weeks after the commencement of HAART, general fever, disturbance of consciousness, and brain edema suddenly developed, and the patient died within two days. Histological examination of the autopsied brain revealed demyelination and reactive gliosis within the white matter and JC virus antigen was detected in oligodendrocytes, consistent with a diagnosis of PML. The most striking feature was an intense perivascular infiltration by CD8+T cells with proteinaceous fluid exudation, suggesting an occurrence of HAART-induced immune reconstitution inflammatory syndrome. Immune reconstitution inflammatory syndrome should be considered as a fatal complication of HAART in patients with AIDS-related PML.