X-linked hyper-IgM syndrome associated with a rapid course of multifocal leukoencephalopathy

Zsuzsanna Aschermann, Eva Gomori, Gabor G. Kovacs, Endre Pal, Gabor Simon, Samuel Komoly, Laszlo Marodi, Zsolt Illes

Research output: Contribution to journalArticle

12 Citations (Scopus)


Objective: To report an exceptional association between X-linked hyper-IgM syndrome and progressive multifocal leukoencephalopathy. Design: Clinical, immunological, and histological analysis. Patient: A 19-year-old male patient with X-linked hyper-IgM syndrome developed typical signs and symptoms of progressive multifocal leukoencephalopathy. Results: The serum level of IgA was decreased; the serum level of IgM was slightly increased; and the serum level of IgG was normal as a result of monthly infusions of immunoglobulin. The expression of CD40 ligand on T cells was markedly reduced in the patient. Magnetic resonance imaging indicated confluent lesions involving the majority of the right hemisphere with a mass effect. The patient died after 6 weeks despite combined antiviral treatment. Conclusion: Progressive multifocal leukoencephalopathy may follow a rapid course in patients with X-linked hyper-IgM syndrome because of global defects of cellular and B cell responses.

Original languageEnglish
Pages (from-to)273-276
Number of pages4
JournalArchives of Neurology
Issue number2
Publication statusPublished - Feb 1 2007

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology

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