IgG4-Related Intracranial Hypertrophic Pachymeningitis : A Case Report and Review of the Literature.
10.3340/jkns.2014.55.5.300
- Author:
Satoru TAKEUCHI
1
;
Hideo OSADA
;
Soichiro SENO
;
Hiroshi NAWASHIRO
Author Information
1. Department of Neurosurgery, National Defense Medical College, Saitama, Japan. s.takeuchi@room.ocn.ne.jp
- Publication Type:Case Report
- Keywords:
Pachymeningitis;
IgG4-related disease;
MRI;
Immunohistochemistry
- MeSH:
Dura Mater;
Generalization (Psychology);
Humans;
Immunohistochemistry;
Magnetic Resonance Imaging;
Male;
Meningitis*;
Middle Aged;
Plasma Cells;
Seizures
- From:Journal of Korean Neurosurgical Society
2014;55(5):300-302
- CountryRepublic of Korea
- Language:English
-
Abstract:
Hypertrophic pachymeningitis is an uncommon disorder that causes a localized or diffuse thickening of the dura mater. Recently, the possibility that IgG4-related sclerosing disease may underlie some cases of intracranial hypertrophic pachymeningitis has been suggested. We herein report the tenth case of IgG4-related intracranial hypertrophic pachymeningitis and review the previous literature. A 45-year-old male presented with left-sided focal seizures with generalization. Magnetic resonance imaging (MRI) revealed a diffuse thickening and enhancement of the right convexity dura matter and falx with focal nodularity. The surgically resected specimens exhibited the proliferation of fibroblast-like spindle cells and an infiltration of mononuclear cells, including predominantly plasma cells. The ratio of IgG4-positive plasma cells to the overall IgG-positive cells was 45% in the area containing the highest infiltration of plasma cells. On the basis of the above findings, IgG4-related sclerosing disease arising from the dura mater was suspected. IgG4-related sclerosing disease should be added to the pachymeningitis spectrum.
