Internal medicine

Radiology

Pathology

Hemiparesis

Disease

Lymphoma

Dementia

Rituximab

Lesion

Central nervous system

Magnetic resonance imaging

Parenchyma

IgG4-related disease

Medicine

JAMA Neurology

<h3>Importance</h3> 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. <h3>Observation</h3> 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. <h3>Conclusions and Relevance</h3> IgG4-RD should be considered in patients with unusual neurologic manifestations suggestive of autoimmune disease.

Central Nervous System Manifestation of IgG4-Related Disease