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JC virus granule cell neuronopathy: A novel clinical syndrome distinct from progressive multifocal leukoencephalopathy
Author(s) -
Koralnik Igor J.,
Wüthrich Christian,
Dang Xin,
Rottnek Matthew,
Gurtman Alejandra,
Simpson David,
Morgello Susan
Publication year - 2005
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.20431
Subject(s) - progressive multifocal leukoencephalopathy , jc virus , white matter , leukoencephalopathy , granule cell , pathology , cerebellum , atrophy , purkinje cell , central nervous system , medicine , biology , magnetic resonance imaging , virus , neuroscience , virology , dentate gyrus , disease , radiology
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. Evolution over 8 years was marked by symptomatic improvement corresponding to highly active antiretroviral therapy (HAART), with modest increase in CD4 + T‐cell counts. We propose to call this novel syndrome JCV granule cell neuronopathy (JCV GCN). Ann Neurol 2005;57:576–580