Idiopathic hypertrophic cranial pachymeningitis: Three biopsy-proven cases including one case with abdominal pseudotumor and review of the literature

Abstract

Hypertrophic pachymeningitis (HP) is a rare disorder of diverse etiology. It presents with headaches, cranial neuropathies and ataxia occurring alone or in combination. Dural biopsy is essential to exclude secondary causes of pachymeningitis. There is paucity of data on biopsied cases of HP. We report three biopsy-proven cases of idiopathic hypertrophic cranial pachymeningitis. All our patients had headaches and multiple cranial neuropathies; ataxia was seen in one patient. One patient had recurrent anterior and posterior cranial neuropathies, while one each had recurrent anterior and posterior cranial neuropathies. Two patients had profound irreversible mono-ocular visual loss. All of them showed prominent pachymeningeal thickening on imaging. Infarcts were seen in one patient, which have rarely been documented. All patients showed biopsy evidence of meningeal thickening and nonspecific chronic inflammation of the dura. The disease may have a remitting and relapsing course, and usually responds to steroids. Clinical improvement was excellent in two patients and modest in one on steroid therapy. All our patients required azathioprine during the course of therapy. Early institution and long-term maintenance of steroid therapy prevents neurologic sequelae. Occurrence of abdominal inflammatory pseudotumor in a patient of HP possibly as part of multifocal fibrosclerosis has not been described earlier.

Diffuse mass-like dural-based neurosarcoidosis with concurrent leptomeningeal involvement: an unusual case presentation and review

“…IHP is marked by chronic granulomatous inflammation leading to fibrosis. IgG4-related IHP is becoming increasingly recognized as the underlying pathology in cases previously defined as idiopathic ( 9 ). Additionally, pachymeningitis can be secondary to various etiologies, including rheumatological diseases such as rheumatoid arthritis, infections such as syphilis and tuberculosis, immunological reactions such as granulomatosis with polyangiitis, and cancer ( 10 ).…”

Idiopathic Hypertrophic Cranial Pachymeningitis With Chiari Type I Malformation: Case Report and Review of the Literature

“…Various disease associations have been recognized, including metabolic disorders, sarcoidosis, infections (tuberculosis, human T-lymphotropic virus, syphilis, meningococcal meningitis), immunosuppression [ 12 - 13 ] as well as certain autoimmune and rheumatological disorders (Wegener granulomatosis, rheumatoid arthritis, multifocal fibrosclerosis, and mixed connective tissue disorder) [ 14 ]. Other rarer associations with IHCP include synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome [ 15 ], Tolosa-Hunt syndrome [ 16 ], and pseudotumor cerebri [ 17 ]. The differentials of IHCP include meningiomas, tuberculous meningitis, neurosarcoidosis, and lymphomas [ 17 ].…”

“…Other rarer associations with IHCP include synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome [ 15 ], Tolosa-Hunt syndrome [ 16 ], and pseudotumor cerebri [ 17 ]. The differentials of IHCP include meningiomas, tuberculous meningitis, neurosarcoidosis, and lymphomas [ 17 ]. This index case is the first report of an association with a CMI.…”

Idiopathic Hypertrophic Spinal Pachymeningitis

“…Hypertrophic pachymeningitis (HP) is an uncommon inflammatory disorder causing chronic diffuse fibrosis and thickening of duramater [1,2]. HP can be classified into primary or idiopathic cases and secondary cases when an underlying etiology is identified.…”