Idiopathic hypertrophic pachymeningitis presenting with a superficial soft tissue mass

Abstract

Idiopathic hypertrophic pachymeningitis (IHP) is a chronic progressive diffuse inflammatory fibrosis of the dura-mater, leading to its diffuse enlargement. The following describes a case of IHP presenting with a superficial soft tissue mass. A 40-year-old female came to hospital with a subcutaneous lump over the left face and frontal headache for 6 months. An excision biopsy revealed chronic inflammation. Magnetic resonance imaging (MRI) of the brain showed left mastoiditis and early dural inflammation of the left temporal region. A few months later, she developed diplopia, complex partial seizures, and retrobulbar neuritis of the left optic nerve. Repeat MRI brain demonstrated meningeal thickening on both sides of the tentorium cerebelli extending to the left tempero-parietal meninges. The meningeal biopsy revealed markedly thickened fibro-connective dural tissue with infiltration of chronic inflammatory cells. There was no evidence of bacterial, fungal, tuberculous or neoplastic infiltration. IHP was diagnosed and steroid therapy initiated. Within weeks, she showed marked clinical improvement. IHP is a diagnosis of exclusion. The absence of underlying infective, neoplastic, or systemic autoimmune disease favors IHP. The above patient had headache, neuro-ophthalmic signs, seizures, which are features of IHP. However, superficial soft tissue involvement is rare.

Distinguishing magnetic resonance imaging features between idiopathic hypertrophic pachymeningitis and secondary hypertrophic pachymeningitis

“…However, our patients did not exhibit hypointensity with thin hyperintense edges on T2WIs as shown in prior studies [10,11]. Interestingly, three idiopathic HP patients (13%) had thick hyperintense dura with hypointense rim on T2WIs (T2-rim pattern), which was described in the prior study [12]. None of secondary HP showed T2-rim pattern.…”

Гіпертрофічний Пахіменінгіт: Клінічний Випадок І Огляд Літератури

“…A chronic process leads to significant thickening of the dura mater. In this case, fungal infection can cause osteomyelitis of the adjacent skull bones with the formation of soft tissue masses inside or outside the skull, rarely sepsis [4,6].…”

“…The most common neurological symptoms are headache, dysfunction (paresis) of cranial nerves (usually passing through the superior orbital fissure), neuro-ophthalmological disorders with visual disturbances and optic disc swelling, limb paresis, cerebellar dysfunction, simple and complex partial motor seizures, increased body temperature, etc. The diagnosis of HP is confirmed by thickening of the dura mater or detection of a soft tissue mass (e.g., cavernous sinus) on neuroimaging (CT, MRI) of the brain as well as by biopsy findings [1][2][3][4][5][6][7][8][9]. The treatment of HP should be comprehensive: etiotropic (antibiotic, antimycotic when indicated), pathogenetic (glucocorticosteroids), and symptomatic (analgesics, sedatives, or anticonvulsants).…”

Commentary

“…[ 1 ] The case report titled “Idiopathic hypertrophic pachymeningitis presenting with a superficial soft tissue” by Keshavaraj et al . [ 2 ] describe a 40-year-old woman who presented with chronic headaches and a facial mass. Magnetic resonance imaging (MRI) of the brain revealed meningeal thickening and meningeal biopsy revealed fibroconnective dural tissue with chronic inflammatory infiltrate.…”