POSSIBLE JCV GRANULAR CELL NEURONOPATHY IN A PATIENT WITH HIV INFECTION

Abstract

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease caused by the JC virus, usually in the context of immunodeficiency, most commonly HIV infection. JCV, however, has been found to infect cerebellar granule cells, resulting in cerebellar atrophy without white matter disease. This has recently been coined JCV granule cell neuronopathy (JCV GCN).1 We report a case that highlights several issues relating to diagnosis and management. ### Case report. A 42-year-old man presented with a 4-week history of dysarthria, clumsiness, unsteady gait, and mild nonspecific headache. His background was significant for HIV infection, diagnosed in 1982, and chronic hepatitis C infection. His disease had been complicated by esophageal candidiasis, Kaposi sarcoma, and peripheral neuropathy. Various combinations of antiretroviral drugs had been given without sustained success because of poor adherence. He consumed alcohol socially only. Nine months prior to his current presentation, he had Pneumocystis jirovecii pneumonia, and recommenced highly active antiretroviral therapy (HAART): lopinavir/ritonavir and tenofovir/emtricitabine. His CD4 count was 17, 24 3 months later, and 87cells/μL 9 months later when he presented with cerebellar symptoms. Viral load was 27,900, 1,200 and 140 cpmL at those times. He had been variably adherent to HAART. On examination, he was malnourished and had florid bilateral cerebellar signs. He had nystagmus in all directions, dysmetria, …