Idiopathic hypertrophic cranial pachymeningitis: a rare but treatable cause of headache and facial pain

Khalil, Michael; Ebner, Franz; Fazekas, Franz; Enzinger, Christian

doi:10.1136/jnnp-2012-303295

Cited by 5 publications

(1 citation statement)

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“…Since the introduction of MRI, hypertrophic pachymeningitis has been increasingly reported in recent literature (1-8). The clinical presentation can be heterogeneous (7,9): headache, cranial nerve palsy, cerebellar ataxia, internal carotid ar-tery occlusion, venous sinus occlusion and obstructive hydrocephalus. Other clinical presentations include diabetic insipidus with hypophysitis, and sinus thrombosis (10).…”

Section: N Discussionmentioning

confidence: 99%

Multiple cranial nerve palsy revealing hypertrophic pachymeningitis with positive myeloperoxidase-antineutrophil cytoplasmic antibody

et al. 2013

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Pachymeningitis is a progressive disease resulting in a diffuse thickening of dura mater due to inflammation, tumor or autoimmune diseases, but most cases are idiopathic. Here, we report the case of a 60-year old man who had a progressive sensorineural hearing loss, visual disturbance and others cranial nerve involvement with an accompanying headache over several months. Brain magnetic resonance imaging showed diffusely thickened dura mater, highly enhanced after gadolinium administration, which was consistent with pachymeningitis. It was assumed to be related to autoimmune pathogenesis on the basis of elevated serum myeloperoxidase-antineutrophil cytoplasmic antibody titers. After empirical steroid and cyclophosphamide therapy, the neurological problems were partially improved. Therefore, in the case of atypical sensorineural hearing loss accompanied by cranial nerve palsy or headache, pachymeningitis should be considered in the differential diagnosis.

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Section: N Discussionmentioning

confidence: 99%