We're unable to sign you in at this time. Please try again in a few minutes.
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
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.
Text Size: A A A
Published online

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.

Figures in this Article

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?


Place holder to copy figure label and caption
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.

Graphic Jump Location
Place holder to copy figure label and caption
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).

Graphic Jump Location




Also Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Please click the checkbox indicating that you have read the full article in order to submit your answers.
Your answers have been saved for later.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment


Some tools below are only available to our subscribers or users with an online account.

1 Citations

Sign in

Purchase Options

• Buy this article
• Subscribe to the journal
• Rent this article ?

Related Content

Customize your page view by dragging & repositioning the boxes below.

Articles Related By Topic
Related Collections