J Korean Neurosurg Soc.  2014 May;55(5):300-302. 10.3340/jkns.2014.55.5.300.

IgG4-Related Intracranial Hypertrophic Pachymeningitis : A Case Report and Review of the Literature

• Takeuchi S
• Osada H
• Seno S
• Nawashiro

• KMID: 2018040
• DOI: http://doi.org/10.3340/jkns.2014.55.5.300

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.

Keyword

Pachymeningitis; IgG4-related disease; MRI; Immunohistochemistry

MeSH Terms

Dura Mater
Generalization (Psychology)
Humans
Immunohistochemistry
Magnetic Resonance Imaging
Male
Meningitis*
Middle Aged
Plasma Cells
Seizures

Figure

• 

  Fig. 1 Preoperative axial (A), coronal (B), and sagittal (C) gadolinium-enhanced magnetic resonance images show irregular thickening and a marked enhancement of the right parieto-occipital dura and falx.

• 

  Fig. 2 Pathological findings. A : A macroscopic observation shows a solid yellow to white-colored lesion in the dura mater. B : The photomicrograph shows the solid lesion consisting of the proliferation of fibroblast-like spindle cells and an infiltration of mononuclear cells, including predominantly plasma cells, with abundant collagenous tissue (H&E; original magnification, ×400). C and D : IgG (C) and IgG4 (D) staining reveal that most of the plasma cells infiltrating into the sclerosing lesion are IgG-positive (IgG4-positive plasma cells/high power field, 78), and that the ratio of IgG4-positive plasma cells to the overall IgG-positive cells is 45% in the area containing the highest infiltration of plasma cells (original magnification, ×400).

• 

  Fig. 3 The twelve-month postoperative axial (A), coronal (B), and sagittal (C) gadolinium-enhanced magnetic resonance images show the disappearance of the mass in the convexity dural region. The mass adjacent to the superior sagittal sinus and falx is left in place.

Reference

1. Chan SK, Cheuk W, Chan KT, Chan JK. IgG4-related sclerosing pachymeningitis : a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009; 33:1249–1252. PMID: 19561447.

2. Choi SH, Lee SH, Khang SK, Jeon SR. IgG4-related sclerosing pachymeningitis causing spinal cord compression. Neurology. 2010; 75:1388–1390. PMID: 20938032.

3. Della Torre E, Bozzolo EP, Passerini G, Doglioni C, Sabbadini MG. IgG4-related pachymeningitis : evidence of intrathecal IgG4 on cerebrospinal fluid analysis. Ann Intern Med. 2012; 156:401–403. PMID: 22393144.

4. Divatia M, Kim SA, Ro JY. IgG4-related sclerosing disease, an emerging entity : a review of a multi-system disease. Yonsei Med J. 2012; 53:15–34. PMID: 22187229.

5. Kim EH, Kim SH, Cho JM, Ahn JY, Chang JH. Imunoglobulin G4-related hypertrophic pachymeningitis involving cerebral parenchyma. J Neurosurg. 2011; 115:1242–1247. PMID: 21854114.

6. Kosakai A, Ito D, Yamada S, Ideta S, Ota Y, Suzuki N. A case of definite IgG4-related pachymeningitis. Neurology. 2010; 75:1390–1392. PMID: 20938033.

7. Lindstrom KM, Cousar JB, Lopes MB. IgG4-related meningeal disease: clinico-pathological features and proposal for diagnostic criteria. Acta Neuropathol. 2010; 120:765–776. PMID: 20844883.

8. Lui PC, Fan YS, Wong SS, Chan AN, Wong G, Chau TK, et al. Inflammatory pseudotumors of the central nervous system. Hum Pathol. 2009; 40:1611–1617. PMID: 19656549.

9. Norikane T, Yamamoto Y, Okada M, Maeda Y, Aga F, Kawai N, et al. Hypertrophic cranial pachymeningitis with IgG4-positive plasma cells detected by C-11 methionine PET. Clin Nucl Med. 2012; 37:108–109. PMID: 22157046.

10. Park IS, Kim H, Chung EY, Cho KW. Idiopathic hypertrophic cranial pachymeningitis misdiagnosed as acute subtentorial hematoma. J Korean Neurosurg Soc. 2010; 48:181–184. PMID: 20856672.

11. Riku S, Hashizume Y, Yoshida M, Riku Y. [Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease?]. Rinsho Shinkeigaku. 2009; 49:594–596. PMID: 19928692.