Idiopathic Chronic Hypertrophic Craniocervical Pachymeningitis

Abstract

A 55-year-old woman with a unique form of chronic hypertrophic pachymeningitis involving the posterior fossa and upper cervical spine is reported. Unlike other cases previously described, the clinical picture was dominated by signs of increased intracranial pressure, lower cranial nerve disorders, and a progressive cervical radiculomyelopathy. The diagnosis was made by means of a contrast-enhanced magnetic resonance imaging scan and confirmed by histological examination of the excised dura. Surgical treatment with removal of the hypertrophic dura provided temporary relief, although the natural history of the disease was not modified. Exhaustive bacteriological and histopathological studies failed to identify a specific cause for this diffuse hypertrophy of the cranial and cervical dura. The literature is reviewed, and other histologically documented cases are discussed.

Hypertrophic pachymeningitis in a southern Chinese population: a retrospective study

“…Locations of the thickened dura matter differ among different causes. The posterior fossa was the most affected area in idiopathic HP and tuberculous meningitis HP according to our study, which was also reported in other studies [6,8,9]. It is notable that posterior fossa HP can cause acute noncommunicating hydrocephalus [9].…”

“…Therefore, patients with otitis media or concurrent multiple cranial nerve deficits should receive contrast-enhanced MRI to rule out HP. In addition, the involvement of the junction of the craniocervical area has been reported in several cases [8,24] and causes obstructive hydrocephalus along with cerebellar tonsillar herniation.…”

“…IHSP mainly affects the cervical and thoracic spine (86%) and, in rare situations, involves the entire spine (7%) [8]. Only a few cases of the craniospinal form of idiopathic HP have been reported [24,39].…”

Hypertrophic Cranial Pachymeningitis Associated with Middle Ear Inflammation

“…Corticosteroid therapy has been effective in alleviating the symptoms and arresting the progression of HCP, but a relapse frequently occurs when the dosage is tapered (2,3,10). The immunosuppressive drugs, such as azathioprine (3,26) and cyclophophamide (3,13), have shown variable efficacy.…”

Hypertrophic Cranial Pachymeningitis Presenting as Steroid-Responsive Hearing Loss

“…It often causes headache and multiple cranial nerve palsies due to fibrous entrapment or ischemic damage. 1,2 Although several causes, either infectious or autoimmune, have been suggested, most aspects of this rare disease remain to be elucidated. It is usually diagnosed by contrast-enhanced magnetic resonance imaging (MRI) or histologic examination of the excised dura.…”