Central Nervous System Manifestation of IgG4-Related Disease

IgG4-related disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells. Head and brain involvement is rare in IgG4-RD, and brain parenchyma involvement has never been reported. A man in his mid-50s with multiorgan IgG4-RD developed progressive spastic hemipare...

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Published inArchives of neurology (Chicago) Vol. 71; no. 6; p. 767
Main Authors Regev, Keren, Nussbaum, Tami, Cagnano, Emanuela, Giladi, Nir, Karni, Arnon
Format Journal Article
LanguageEnglish
Published Chicago American Medical Association 01.06.2014
Subjects
Brain
Cognition & reasoning
Dementia
Immunoglobulins
Nervous system
Plasma
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Summary:IgG4-related disease (IgG4-RD) is characterized by an inflammatory reaction rich in IgG4-positive plasma cells. Head and brain involvement is rare in IgG4-RD, and brain parenchyma involvement has never been reported. A man in his mid-50s with multiorgan IgG4-RD developed progressive spastic hemiparesis and dementia. Magnetic resonance imaging of the brain revealed several cortical and subcortical lesions. Pathologic findings in the brain were consistent with IgG4-related central nervous system involvement. The patient was treated with high-dose corticosteroids followed by rituximab, and his cognitive and motor functions improved significantly. IgG4-RD should be considered in patients with unusual neurologic manifestations suggestive of autoimmune disease.
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ISSN:2168-6149
0003-9942
2168-6157