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

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.

IgG4-Related Sclerosing Disease Causing Spinal Cord Compression: The First Reported Case in Literature

“…IgG4-RD has been reported to affect the CNS in literature. The most common CNS manifestation of IgG4-RD is hypertrophic pachymeningitis where it lead to dural hypertrophy (and thickening) which will eventually lead to symptoms such as headaches, cranial nerve dysfunctions, sensory and/or motor symptoms depending to the location, either intracranially or spinally [3]. Meningeal involvement of IgG4-RD might be confused with many differential diagnoses including; Idiopathic hypertrophic pachymeningitis, inflammatory myofibroblastic tumor, lymphoma, granulomatosis with polyangiitis, giant-cell arteritis, Langerhans-cell histiocytosis, and sarcoidosis [10].…”

Neurological Manifestations of IgG4-Related Disease

“…headache, seizures and cognitive decline) [55, 57]. Indeed, chronic headache and multiple cranial neuropathies are the most commonly reported symptoms [15, 53, 55, 57, 58]. Symptoms resulting from mechanical compression are dependent on anatomical location.…”

“…paraparesis), and this has been more frequently reported in spinal pachymeningitis. In fact, some of these patients have been treated with surgery alone, without concomitant glucocorticoid therapy [58, 168]. …”

A case report of atypical long segmental thoracic hypertrophic pachymeningitis with ossification of ligamentum flavum and literature review

“…This pattern represents more active inflammation in peripheral zone than in central zone of fibrosis [7,19,20]. Homogeneous enhancement pattern has been reported frequently in IHP [21][22][23]. A case of IHP with non-enhancement in MRI was also reported [24].…”

“…According to etiology, HP is classified as idiopathic and secondary type. Autoimmune disorders was identified as secondary causes of HP in previous reports, such as Rheumatoid arthritis [1], Multifocal fibro sclerosis [2], Wegener's granulomatosis [3], Neuro-Behçet's disease [4], sarcoidosis [5], ANCA related diseases [6][7][8], IgG4 related diseases [9][10][11][12]23] and antiphospholipid syndrome [13]. Some infectious diseases caused by bacteria [14,37], fungi [15] and Spirochete [16,17] has been reported associated with HP as well.…”