title: JC virus granule cell neuronopathy: A cause of infectious cerebellar degeneration.
abstract: JC virus (JCV) infection of glial cells can lead to progressive multifocal leukoencephalopathy (PML) in immunocompromised patients. A newly described phenotype of the infection is infection of neurons. This distinct clinical and radiological syndrome is named JCV granule cell neuronopathy, characterized by exclusive or predominant cerebellar atrophy. We report the clinical and radiological longitudinal findings of 5 HIV-infected patients referred to us between September 2004 and November 2011 who exhibited JCV granule cell neuronopathy (4 probable cases and 1 possible). The association of immunocompromised status, progressive cerebellar syndrome, MRI abnormalities with cortical cerebellar atrophy and cerebrospinal fluid positive for JCV on PCR allowed for a highly probable diagnosis. The reversal of the immunocompromised status is the only way to stop the disease evolution. Motor functioning can remain impaired, but the illness itself, unlike progressive multifocal leukoencephalopathy, does not seem to threaten life.
authors.affiliation: Department of Neurology, Saint-Denis Hospital (93), France. Electronic address: carole.henry@ch-stdenis.fr.
authors.forename: Carole
authors.initials: C
authors.lastname: Henry
authors.affiliation: Department of Neurology, Saint-Denis Hospital (93), France.
authors.forename: Fanny
authors.initials: F
authors.lastname: Jouan
authors.affiliation: Department of Neurology, Saint-Denis Hospital (93), France.
authors.forename: Thomas
authors.initials: T
authors.lastname: De Broucker
mesh_terms: D000328:Adult; D002526:Cerebellar Diseases / Q000175:diagnosis* / Q000209:etiology / Q000821:virology; D005260:Female; D015658:HIV Infections / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D007577:JC Virus; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications / Q000175:diagnosis*; D008297:Male; D012189:Retrospective Studies
keywords: Cerebellar atrophy; Granule cell neuronopathy; HIV; Immunosuppression; JC virus; Progressive multifocal leukoencephalopathy