Neurol Case Rep | Volume 5, Issue 1 | Case Report | Open Access

Intracranial Inflammatory Pseudotumor with Left Internal Carotid Artery Stenosis due to Immunoglobulin G4-Related Disease: A Case Report and Literature Review

Chenyang Zhao^1#, Wenyu Liu^1#, Xueqin Chen², Dong Zhou^1* and Xintong Wu^1*

¹Department of Neurology, West China Hospital, Sichuan University, PR China ²Department of Pathology, West China Hospital, Sichuan University, PR China ^#These authors contributed equally to this work and share the first authorship

^*Correspondance to: Xintong Wu 

Fulltext PDF

Abstract

Background: Immunoglobulin G4-Related Disease (IgG4-RD) is an autoimmune-mediated inflammatory fibrotic disease that can cause organ enlargement and tissue destruction. It can involve any organ but is most common in the pancreas, bile ducts, etc. An intracranial IgG4-associated inflammatory pseudotumor, as described in the case presented here, is unusual. Case Report: A 27-year-old male was admitted to hospital due to visual loss in the left eye for 11 years and left-sided facial numbness for 1 year. A mass located in the left orbit, cavernous sinus, infratemporal fossa, and parapharyngeal space was revealed by Magnetic Resonance Imaging (MRI). The left interior carotid artery was encased by the lesion. Surgery was performed for biopsy. The pathological findings showed diffuse lymphoplasmacytic infiltration with lymphoid follicles and fibrous tissue hyperplasia. Immunostaining identified many IgG4+ cells, with up to 30/High Power Field (HPF). The IgG4 serum level was also high. The patient was diagnosed with IgG4-RD and given steroid therapy. Four months after surgery and the therapy, MRI results showed that the lesion had been resolved. Conclusion: Although this patient met the diagnostic criteria and had many similarities with previously reported cases of IgG4-RD, there were also many new manifestations, such as different imaging features, stenosis of the internal carotid artery, and so on. These findings suggest that our understanding of the disease is insufficient, and that future studies with a larger sample size and long-term observation follow-up are needed.

Keywords:

IgG4-related disease; IgG4-related inflammatory pseudotumor; Pathology; Surgery

Citation:

Zhao C, Liu W, Chen X, Zhou D, Wu X. Intracranial Inflammatory Pseudotumor with Left Internal Carotid Artery Stenosis due to Immunoglobulin G4- Related Disease: A Case Report and Literature Review. Neurol Case Rep. 2022;5(1):1032..