Progressive multifocal leukoencephalopathy in a patient with Good's syndrome
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- 作者：Giovanna Squintani ; Sergio Ferrari ; Elena Bazzoli ; Roberto Eleopra ; Carlo La Monaca ; Enrico Cagliari ; Gianluigi Zanusso ; Maria Cristina Mantovan ; Salvatore Monaco
- 关键词：Thymoma ; Hypogammaglobulinemia ; JC virus ; Progressive multifocal leukoencephalopathy ; Good's syndrome
- 刊名：International Journal of Infectious Diseases
- 出版年：2010
- 出版时间：May 2010
- 年：2010
- 卷：14
- 期：5
- 页码：e444-e447
- 全文大小：2330 K
文摘
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.