title: Progressive multifocal leukoencephalopathy in a patient with Good's syndrome.
abstract: Good's syndrome (GS) is an immunodeficiency characterized by thymoma, hypogammaglobulinemia, and impaired T-cell function. The clinical manifestations of GS include recurrent or chronic infections from common or opportunistic pathogens. Encephalitis is a rare event, with only anecdotal reports of cytomegalovirus infection. Herein we report the case of a 79-year-old woman with GS who developed subacute motor deficits and cognitive changes. Magnetic resonance imaging (MRI) of the brain disclosed white- and gray-matter lesions, mostly in the right frontal and parietal areas. Polyoma virus JC, the agent of progressive multifocal encephalopathy (PML), was identified in cerebrospinal fluid samples and brain biopsy specimens. After diagnosis, the disease had a rapid fatal course. The present case represents the first reported association between GS and PML.
authors.affiliation: Neurology Unit, Ospedale Civile Maggiore, Piazzale Stefani, 1, 37134, Verona, Italy. neurogio@tiscali.it
authors.forename: Giovanna
authors.initials: G
authors.lastname: Squintani
authors.forename: Sergio
authors.initials: S
authors.lastname: Ferrari
authors.forename: Elena
authors.initials: E
authors.lastname: Bazzoli
authors.forename: Roberto
authors.initials: R
authors.lastname: Eleopra
authors.forename: Carlo
authors.initials: C
authors.lastname: La Monaca
authors.forename: Enrico
authors.initials: E
authors.lastname: Cagliari
authors.forename: Gianluigi
authors.initials: G
authors.lastname: Zanusso
authors.forename: Maria Cristina
authors.initials: MC
authors.lastname: Mantovan
authors.forename: Salvatore
authors.initials: S
authors.lastname: Monaco
mesh_terms: D000368:Aged; D001921:Brain / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000473:pathology; D007577:JC Virus / Q000302:isolation & purification*; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications* / Q000473:pathology / Q000821:virology