Central nervous system manifestation of IgG4-related disease.
OBJECTIVE
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.


METHODS
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.


CONCLUSIONS
IgG4-RD should be considered in patients with unusual neurologic manifestations suggestive of autoimmune disease.
Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.
Keren
K
Regev
Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.
Tami
T
Nussbaum
Department of Pathology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.
Emanuela
E
Cagnano
Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.
Nir
N
Giladi
Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel.
Arnon
A
Karni
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D001706:Biopsy; D002493:Central Nervous System Diseases / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D000069283:Rituximab; D016896:Treatment Outcome
D000305:Adrenal Cortex Hormones; D058846:Antibodies, Monoclonal, Murine-Derived; D007074:Immunoglobulin G; D000069283:Rituximab