Idiopathic hypertrophic cranial pachymeningitis. Report of three cases.

Abstract

Hypertrophic cranial pachymeningitis is a rare, idiopathic form of granulomatous pachymeningitis. This report describes three cases of hypertrophic cranial pachymeningitis and discusses the clinical, radiographic, and pathological findings in these and other reported cases. These lesions typically cause progressive cranial nerve palsies, headaches, and cerebellar dysfunction. They occur in patients of all age groups; the peak incidence is in the sixth decade. Hypertrophic cranial pachymeningitis is best identified by magnetic resonance imaging. The diagnosis is established by excluding all other granulomatous and infectious diseases. A dural biopsy is essential to confirm the diagnosis. Hypertrophic cranial pachymeningitis is initially responsive to steroid therapy, but in most cases it recurs or progresses despite treatment. Surgical excision of granulomas is occasionally necessary to alleviate a mass effect. The long-term outcome remains uncertain for most patients, but progressive disease is usually fatal owing to cranial neuropathies.