Abstract
Progressive multifocal leukoencephalopathy has long been described as the sole feature of JC virus (JCV) infection of the central nervous system. Over the past decade, its spectrum has extended to new forms of encephalopathy, notably affecting neurons. We describe an HIV-infected patient with worsening cerebellar symptoms, progressive cerebellar atrophy and a hot-cross-bun sign attributed to JCV–granule-cell neuronopathy (GCN), and 20 years of follow-up.
Highlights
Progressive multifocal leukoencephalopathy (PML) remains an important complication of HIV-1/AIDS, despite widespread combined antiretroviral therapy
JC virus (JCV)– GCN diagnosis relies on brain MRI, visualizing marked cerebellar atrophy, and cerebrospinal fluid (CSF) analyses, showing normal cell count and protein level, but with JCV-positive PCR negative for all other infectious pathogens
Brain MRI can help distinguish these two entities: fluid-attenuated inversion recovery (FLAIR) cerebellar atrophy and hyperintensities are more asymmetric in JC Virus–Granule-Cell Neuronopathy (JCV–GCN) than in multiple-system atrophy (MSA), while putamen atrophy with the “putaminal rim sign” is present only in MSA [22]
Summary
Progressive multifocal leukoencephalopathy (PML) remains an important complication of HIV-1/AIDS, despite widespread combined antiretroviral therapy (cART). In December 2014, on raltegravir (800 mg/d), darunavir (800 mg/d) and ritonavir (100 mg/d), he underwent a new thorough work-up because of worsening gait ataxia: CD4-cell count: 832/μl; CD4/CD8 ratio: 1.15; PLVL:
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