"T1: normal"
"T2/FLAIR: confluent hyperintense regions"
"T1 C+ (Gd): no enhancement"
"JC virus granule cell neuronopathy results from the reactivation of the John Cunningham virus (JC virus), infecting granule cell neurones in the cerebellum, in patients with compromised immune systems."
"JC virus granule cell neuronopathy results from the reactivation of the John Cunningham virus (JC virus), infecting granule cell neurones in the cerebellum, in patients with compromised immune systems."
"JC virus granule cell neuronopathy is considered a rare manifestation of JC virus reactivation, thought to be much less common than progressive multifocal leukoencephalopathy 1-4."
"JC virus granule cell neuronopathy is considered a rare manifestation of JC virus reactivation, thought to be much less common than progressive multifocal leukoencephalopathy 1-4."
"Diagnosis is made through a characteristic clinicoradiological phenotype and a positive JC virus DNA PCR in the cerebrospinal fluid (CSF)."
"Diagnosis is made through a characteristic clinicoradiological phenotype and a positive JC virus DNA PCR in the cerebrospinal fluid (CSF)."
"JC virus granule cell neuronopathy is secondary to progressive infection of granule cell neurones in the cerebellum due to the reactivation of the JC virus 1-4. It has been postulated that for the JC virus to preferentially infect granule cell neurones instead of glial cells, there must be a mutation, typically involving the C terminus of the VP1 gene, that triggers this change 6."
"JC virus granule cell neuronopathy is secondary to progressive infection of granule cell neurones in the cerebellum due to the reactivation of the JC virus 1-4. It has been postulated that for the JC virus to preferentially infect granule cell neurones instead of glial cells, there must be a mutation, typically involving the C terminus of the VP1 gene, that triggers this change 6."
"JC virus granule cell neuronopathy is secondary to progressive infection of granule cell neurones in the cerebellum due to the reactivation of the JC virus 1-4. It has been postulated that for the JC virus to preferentially infect granule cell neurones instead of glial cells, there must be a mutation, typically involving the C terminus of the VP1 gene, that triggers this change 6."
"There is derangement of the normal laminar cellular organisation of cerebellum, with the hallmark histological feature of JC virus granule cell neuronopathy being infection and loss of granule cell neurones in the internal granule cell layer, with sparing of the molecular and Purkinje layers 1,3. Additionally, there may also be involvement of nearby glial cells (e.g. oligodendrocytes and astrocytes) 1."
"There is debate as to whether these white matter changes are truly due to JC virus granule cell neuronopathy, or due to concurrent infratentorial progressive multifocal leukoencephalopathy, and presence of atypical white matter cerebellar changes (e.g. shrimp sign) should prompt consideration towards the latter 1-3. Supratentorial white matter involvement is not a feature of JC virus granule cell neuronopathy, and its involvement may be due to other concurrent manifestations of the JC virus such as progressive multifocal leukoencephalopathy or immune reconstitution inflammatory syndrome 1-3."
"There is debate as to whether these white matter changes are truly due to JC virus granule cell neuronopathy, or due to concurrent infratentorial progressive multifocal leukoencephalopathy, and presence of atypical white matter cerebellar changes (e.g. shrimp sign) should prompt consideration towards the latter 1-3. Supratentorial white matter involvement is not a feature of JC virus granule cell neuronopathy, and its involvement may be due to other concurrent manifestations of the JC virus such as progressive multifocal leukoencephalopathy or immune reconstitution inflammatory syndrome 1-3."
"There is debate as to whether these white matter changes are truly due to JC virus granule cell neuronopathy, or due to concurrent infratentorial progressive multifocal leukoencephalopathy, and presence of atypical white matter cerebellar changes (e.g. shrimp sign) should prompt consideration towards the latter 1-3. Supratentorial white matter involvement is not a feature of JC virus granule cell neuronopathy, and its involvement may be due to other concurrent manifestations of the JC virus such as progressive multifocal leukoencephalopathy or immune reconstitution inflammatory syndrome 1-3."
"Generally, management is supportive, with immune reconstitution (e.g. commencing antiretroviral therapy in patients with HIV, withdrawal of immunosuppressive therapy, etc.), with no disease-specific therapy available. However, allogeneic BK virus-specific T-cell therapy has been used at a case report level with anecdotal benefit 8."
Expected headings
"FDG-PET"
"immunosuppressive monoclonal antibody therapy (e.g. rituximab, natalizumab)"
"There is derangement of the normal laminar cellular organisation of cerebellum, with the hallmark histological feature of JC virus granule cell neuronopathy being infection and loss of granule cell neurones in the internal granule cell layer, with sparing of the molecular and Purkinje layers 1,3. Additionally, there may also be involvement of nearby glial cells (e.g. oligodendrocytes and astrocytes) 1."
"There is debate as to whether these white matter changes are truly due to JC virus granule cell neuronopathy, or due to concurrent infratentorial progressive multifocal leukoencephalopathy, and presence of atypical white matter cerebellar changes (e.g. shrimp sign) should prompt consideration towards the latter 1-3. Supratentorial white matter involvement is not a feature of JC virus granule cell neuronopathy, and its involvement may be due to other concurrent manifestations of the JC virus such as progressive multifocal leukoencephalopathy or immune reconstitution inflammatory syndrome 1-3."
"Longitudinal prognostic data is lacking in the literature; however, small case series suggest that patients will often have a progressive course that will eventually plateau over months with persisting neurological disability 2."