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Case Report/Case Series |

Central Nervous System Manifestation of IgG4-Related Disease

Keren Regev, MD1; Tami Nussbaum, MD1; Emanuela Cagnano, MD2; Nir Giladi, MD1; Arnon Karni, MD, PhD1
[+] Author Affiliations
1Department of Neurology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel
2Department of Pathology, Tel Aviv Sourasky Medical Center, Tel Aviv University, Tel Aviv, Israel
JAMA Neurol. 2014;71(6):767-770. doi:10.1001/jamaneurol.2014.40.
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Importance  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.

Observation  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 and Relevance  IgG4-RD should be considered in patients with unusual neurologic manifestations suggestive of autoimmune disease.

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Figure 1.
Magnetic Resonance Imaging Findings

A and B, Axial fluid-attenuated inversion recovery images of the brain reveal bilateral periventricular white matter hyperintense lesions that were detected on the scans taken 4 years earlier and at admittance. C, A new lesion that was detected during the patient’s most recent hospitalization in the right dorsal frontal lobe was biopsied for further diagnosis study. D, Lesion after biopsy.

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Figure 2.
Histologic Findings of the Biopsied Lesion

A, Lymphoplasmacytic infiltration, most prominent in the perivascular spaces (hematoxylin-eosin; original magnification ×20). B, Immunohistochemical staining of plasma cells using the CD138 marker revealed plasma cells infiltrating the brain tissue that constitute more than 10% of the detected immune cells (original magnification ×20). C, Immunohistochemical staining for IgG4, which was detected in more than 10 plasma cells per high-power field (original magnification ×20).

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