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X-linked Hyper-IgM Syndrome Associated With a Rapid Course of Multifocal Leukoencephalopathy
Zsuzsanna Aschermann, MD;
Eva Gomori, MD, PhD;
Gabor G. Kovacs, MD, PhD;
Endre Pal, MD, PhD;
Gabor Simon, MD, PhD;
Samuel Komoly, MD, DSc;
Laszlo Marodi, MD, DSc;
Zsolt Illes, MD, PhD
Arch Neurol. 2007;64(2):273-276.
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.
Author Affiliations: Departments of Neurology (Drs Aschermann, Pal, Komoly, and Illes) and Pathology (Dr Gomori), University of Pecs, Pecs, Hungary; Department of Neuropathology, National Institute of Neurology and Psychiatry, Budapest, Hungary (Dr Kovacs); Department of Pediatrics, Szent Gyorgy County Hospital, Szekesfehervar, Hungary (Dr Simon); and Department of Infectious and Pediatric Immunology, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary (Dr Marodi).
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