Progressive multifocal leukoencephalopathy in a patient with Good's syndrome.
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.
Squintani
Giovanna
G
Neurology Unit, Ospedale Civile Maggiore, Piazzale Stefani, 1, 37134, Verona, Italy. neurogio@tiscali.it
Ferrari
Sergio
S
Bazzoli
Elena
E
Eleopra
Roberto
R
La Monaca
Carlo
C
Cagliari
Enrico
E
Zanusso
Gianluigi
G
Mantovan
Maria Cristina
MC
Monaco
Salvatore
S
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