Progressive multifocal leukoencephalopathy in a patient with Good's syndrome

Giovanna Squintani, Sergio Ferrari, Elena Bazzoli, Roberto Eleopra, Carlo La Monaca, Enrico Cagliari, Gianluigi Zanusso, Maria Cristina Mantovan, Salvatore Monaco

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
JournalInternational Journal of Infectious Diseases
Volume14
Issue number5
DOIs
Publication statusPublished - May 2010

Keywords

  • Good's syndrome
  • Hypogammaglobulinemia
  • JC virus
  • Progressive multifocal leukoencephalopathy
  • Thymoma

ASJC Scopus subject areas

  • Microbiology (medical)
  • Infectious Diseases
  • Medicine(all)

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