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Fulminant JC virus encephalopathy with productive infection of cortical pyramidal neurons
Annals of Neurology , 07/02/09
Wuthrich C et al. - The polyomavirus JC (JCV) is the causative agent of progressive multifocal leukoencephalopathy and of JCV granule cell neuronopathy. A human immunodeficiency virus-negative patient who experienced development of multiple cortical lesions, aphasia, and progressive cognitive decline after chemotherapy for non-small-cell lung cancer is presented.
Igor J. Koralnik, 07/07/09
| The polyomavirus JC(JCV) is the etiologic agent of progressive multifocal leukoencephalopathy(PML) and of JCV granule cell neuronopathy(JCV GCN). We present an HIV-negative patient who developed multiple cortical lesions, aphasia and progressive cognitive decline after chemotherapy for non-small-cell lung cancer. Brain biopsy and CSF PCR demonstrated JCV and she had a rapidly fatal outcome. Post-mortem analysis showed diffuse cortical lesions and areas of necrosis at the gray-white junction. Immunostaining revealed a productive JCV-infection of cortical pyramidal neurons, confirmed by electron microscopy, with limited demyelination. This novel gray matter syndrome expands the scope of JCV clinical presentation and pathogenesis. |
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