IgG4-related intracranial hypertrophic pachymeningitis with skull hyperostosis: a case report

Abstract

BackgroundImmunoglobulin G4 (IgG4)-related disease is a systemic syndrome, characterized by sclerosing lesions and usually associated with a raised serum IgG4 level; the pancreas, salivary glands, and lacrimal glands are typically affected. Recently, it has been suggested that IgG4-related sclerosing disease represents a subset of cases previously diagnosed as idiopathic hypertrophic pachymeningitis. This rare inflammatory disorder causes localized or diffused thickening of intracranial dura mater. Headache, cranial nerve palsy, and ataxia are the most common clinical manifestations.Herein, we report the clinical and histopathological features of a rare case of IgG4-related intracranial hypertrophic pachymeningitis involving cranial hyperostosis.Case presentationA 52-year-old man presented with refractory generalized tonic-clonic seizure. Magnetic resonance imaging revealed thickening of the meninges with enhancement near the superior sagittal sinus; skull bone defect was also noted. Extensive excision of affected skull bone and dura was performed, providing the diagnosis of IgG4-related pachymeningitis. After the surgery, the patient’s seizure stopped and he was smoothly tapered off antiepileptic medication.ConclusionTo our knowledge, this is the first reported case of IgG4-related pachymeningitis with concomitant skull hyperostosis.

Neurological Manifestations of IgG4-Related Disease

“…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 kind of enhancement pattern is considered to be associated with the lack of inflammatory reaction in pathological dura matter tissue [20,24,25]. Moreover, in some previously reported cases of HP, hyperostosis [26,27] or destruction [24,28] of adjacent bone tissue was observed.…”

MPO-ANCA-Positive Hypertrophic Pachymeningitis With Elevated Serum IgG4: A Case Report and Literature Review.

“…The serum IgG4 cell level of the patient with IgG4-RD was signi cantly increased, and the affected tissues and organs were enlarged or nodular/proliferative lesions due to the abundant in ltration of lymphocytes and IgG4-positive plasma cells and the tissue brosis. The central nervous system mainly involves the pituitary gland, dura mater and skull [13] . The disease can cause multiple organs to be affected simultaneously or successively, or it can involve only one organ.…”