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

Zsuzsanna Aschermann, E. Gömöri, Gabor G. Kovacs, Endre Pal, Gabor Simon, S. Komoly, L. Máródi, Zsolt Illes

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

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
Volume64
Issue number2
DOIs
Publication statusPublished - Feb 2007

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Type 1 Hyper-IgM Immunodeficiency Syndrome
Leukoencephalopathies
Progressive Multifocal Leukoencephalopathy
Serum
CD40 Ligand
Immunoglobulin A
Signs and Symptoms
Antiviral Agents
Immunoglobulin M
Immunoglobulins
B-Lymphocytes
Immunoglobulin G
Magnetic Resonance Imaging
Syndrome
T-Lymphocytes

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

X-linked hyper-IgM syndrome associated with a rapid course of multifocal leukoencephalopathy. / Aschermann, Zsuzsanna; Gömöri, E.; Kovacs, Gabor G.; Pal, Endre; Simon, Gabor; Komoly, S.; Máródi, L.; Illes, Zsolt.

In: Archives of Neurology, Vol. 64, No. 2, 02.2007, p. 273-276.

Research output: Contribution to journalArticle

Aschermann, Zsuzsanna ; Gömöri, E. ; Kovacs, Gabor G. ; Pal, Endre ; Simon, Gabor ; Komoly, S. ; Máródi, L. ; Illes, Zsolt. / X-linked hyper-IgM syndrome associated with a rapid course of multifocal leukoencephalopathy. In: Archives of Neurology. 2007 ; Vol. 64, No. 2. pp. 273-276.
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