HIV-Associated JC Virus-Granule-Cell Neuronopathy (JCV-GCN) with the Hot-Cross-Bun Sign

The “Hot Cross Bun Sign” in Spinocerebellar Ataxia Types 2 and 7–Case Reports and Review of Literature

“…Longitudinal analysis of HIV infected patients with JCV demonstrated the HCB sign had a delayed appearance even in burnt out JCV infection, indicating HIV progression or retrograde neuronal loss with gliosis. 27,58,60 The appearance of the HCBS in an HIV infected cohort will be worth investigating.…”

“…Laboratory testing for infective, autoimmune and metabolic disorders and CSF examination including Varicella Zoster Virus, Herpes Simplex Virus, Cytomegalovirus and John Cunningham Virus (JCV) PCR were normal. Her CD4 count was [6][7][8]18,44,52,[84][85][86][87] Probable Dementia with Lewy Body 1 15 SCA 3 9 6,12,44,3,5,84,86,88,89 Corticobasal Degeneration (olivopontocerebellar type) 1 14 SCA 6, 7, 8 17 4,6,44,47,84,86,90,91 Parkinsonism Related Vasculitis 1 16 SCA 10 1 84 Infection SCA 17 1 10 Rabies Encepaltiis 1 2 SCA 23 1 92 HIV related Progressive Multifocal Leukoencephalopathy 4 22,23,25,58 SCA 31 1 47 Natalizumab associated Progressive Multifocal Leukoencephalopathy 1 29 SCA 34 4 13,86,93,94 HIV related JCV granule cell neuronopathy 1 60 SCA 42 1 95 HIV 2 27,…”

Common and uncommon neuroimaging manifestations of ataxia: an illustrated guide for the trainee radiologist. Part 1 – acquired diseases

“…Progressive multifocal leukoencephalopathy (PML) is a CNS demyelinating disease caused by the JC virus ( 12 , 13 ) . It occurs mainly in patients with severe immunodeficiency, such as those with HIV/AIDS, or individuals treated with monoclonal antibody therapies such as natalizumab ( 12 ) .…”

“…It occurs mainly in patients with severe immunodeficiency, such as those with HIV/AIDS, or individuals treated with monoclonal antibody therapies such as natalizumab ( 12 ) . Typical PML is characterized by multifocal, bilateral, asymmetrical lesions involving the white matter ( 12 ) ; however, infection of the granular cells of the cerebellar cortex by the JC virus might result in cerebellar atrophy, together with damage and gliosis of the pontocerebellar fibers in the pons, producing the “hot cross bun sign”, characterized by cruciform hyperintense lesion on T2/fluid-attenuated inversion recovery in the pons and rarely depicted in PML ( 13 ) . The difference among them is believed to be related to a mutant JC virus harboring a small VP1-capsid-protein deletion, which changes the viral tropism ( 13 ) .…”

“…Typical PML is characterized by multifocal, bilateral, asymmetrical lesions involving the white matter ( 12 ) ; however, infection of the granular cells of the cerebellar cortex by the JC virus might result in cerebellar atrophy, together with damage and gliosis of the pontocerebellar fibers in the pons, producing the “hot cross bun sign”, characterized by cruciform hyperintense lesion on T2/fluid-attenuated inversion recovery in the pons and rarely depicted in PML ( 13 ) . The difference among them is believed to be related to a mutant JC virus harboring a small VP1-capsid-protein deletion, which changes the viral tropism ( 13 ) .…”