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JC virus granular neuronopathy and rhombencephalic progressive multifocal leukoencephalopathy: case report and review of the literature.
Neuropathology
June 2012
Department of Anatomical Pathology, Sunnybrook Health Sciences Centre, Sunnybrook Health Sciences Centre, University of Toronto, Toronto, Ontario, Canada.
JC virus (JCV) granular neuronopathy remains an under-appreciated phenomenon whereby JCV inhabits neurons in the granular layer of the cerebellum causing neuronal loss, gliosis and a clinical cerebellar syndrome. The following case describes a man with sarcoidosis and idiopathic leukopenia who developed a clinical cerebellar syndrome due to JCV granular neuronopathy, followed by neurological decline due to rhombencephalic progressive multifocal leukoencephalopathy. This case reminds us of the ability of JCV to produce dual neuropathology which includes JCV granular neuronopathy, and the pathogenesis and clinical implications for this phenomenon are discussed.
View Article and Find Full Text PDFFrequency and large T (LT) sequence of JC polyomavirus DNA in oligodendrocytes, astrocytes and granular cells in non-PML brain.
Brain Pathol
May 2012
Department of Medicine, Monash University, Level Seven, Alfred Centre, Alfred Hospital, Commercial Road, Melbourne, Vic. 3004, Australia.
Progressive multifocal leukoencephalopathy (PML) and JCV granular cell neuronopathy occur secondary to JCV polyomavirus (JCV) infection of oligodendrocytes and cerebellar granular cell neurons (CGNs) during immunosuppression. Pure populations of astrocytes, oligodendrocytes, CGNs and microglia from frontal cortex and cerebellum of 17 non-PML patients (9 immunocompetent; 8 immunosuppressed) were isolated by laser capture microdissection (LCM). JCV large T (LT) antigen DNA was detected by triple nested polymerase chain reaction (PCR).
View Article and Find Full Text PDFPossible JCV granular cell neuronopathy in a patient with HIV infection.
Neurology
November 2009
Department of Neurology, St Vincent's Hospital, Sydney, Australia.
JC virus granule cell neuronopathy: A novel clinical syndrome distinct from progressive multifocal leukoencephalopathy.
Ann Neurol
April 2005
Department of Neurology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA.
Progressive multifocal leukoencephalopathy (PML) typically affects the CNS white matter of the central nervous system. We present an human immunodeficiency virus-infected patient with polyomavirus JC infection restricted to granule cell neurons of the cerebellum and with corresponding neurological symptomatology. Magnetic resonance imaging demonstrated cerebellar atrophy without white matter lesions and stereotactic biopsy showed selective infection of the cerebellar granular cell layer, with preservation of Purkinje cells and absence of classic progressive multifocal leukoencephalopathy histopathology in underlying white matter.
View Article and Find Full Text PDFCellular changes in the cerebellar granular layer in AIDS-associated PML.
Neuropathol Appl Neurobiol
October 1993
Institut für Neuropathologie, Universität Münster, Germany.
Six cases of AIDS-associated progressive multifocal leukoencephalopathy (PML) exhibited peculiar cellular changes in the cerebellar granular layer. These cells without discernible cytoplasm showed hypochromatic nuclei about twice as large as those of normal granule cells. They were restricted exclusively to the granular layer and always surrounded PML foci.
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