X-linked hyper-IgM syndrome associated with a rapid course of multifocal leukoencephalopathy.
OBJECTIVE
To report an exceptional association between X-linked hyper-IgM syndrome and progressive multifocal leukoencephalopathy.


METHODS
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.


CONCLUSIONS
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.
Aschermann
Zsuzsanna
Z
Department of Neurology, University of Pecs, 2 Rèt Street, Pecs, 7623 Hungary.
Gomori
Eva
E
Kovacs
Gabor G
GG
Pal
Endre
E
Simon
Gabor
G
Komoly
Samuel
S
Marodi
Laszlo
L
Illes
Zsolt
Z
D000328:Adult; D019013:CD40 Antigens / Q000378:metabolism; D006801:Humans; D053307:Hyper-IgM Immunodeficiency Syndrome, Type 1 / Q000150:complications* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D007136:Immunoglobulins / Q000008:administration & dosage; D007968:Leukoencephalopathy, Progressive Multifocal / Q000150:complications* / Q000276:immunology / Q000473:pathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male
D019013:CD40 Antigens; D007136:Immunoglobulins