title: Combined therapy of corticosteroid and azathioprine in hypertrophic cranial pachymeningitis.
abstract: Hypertrophic cranial pachymeningitis (HCP) is a rare inflammatory disease of unknown origin in which recurrence is frequently observed in spite of the initial response to steroid therapy. Three patients, 1 man and 2 women, aged 63, 66, and 67 years, with severe intractable headache were evaluated by brain MRI. All patients were initially given prednisolone (60 mg/day, oral) or dexamethasone (20 mg/day, i.v.), and followed by long-term (at least 1 year) azathioprine therapy. All patients were evaluated by follow-up laboratory tests and brain MRI study, and completed the 2-year follow-up period. Symptoms including headache were initially improved with corticosteroid therapy, but patients became steroid-dependent. Azathioprine administration in these steroid-dependent patients permitted the complete cessation of corticosteroid and led to the clinical and radiological recovery. In conclusion, initial high-dose corticosteroid administration followed by long-term azathioprine therapy may be the ideal treatment of HCP at present.
authors.affiliation: Department of Neurology, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: I S
authors.initials: IS
authors.lastname: Choi
authors.forename: S C
authors.initials: SC
authors.lastname: Park
authors.forename: Y K
authors.initials: YK
authors.lastname: Jung
authors.forename: S S
authors.initials: SS
authors.lastname: Lee
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage*; D000368:Aged; D001379:Azathioprine / Q000008:administration & dosage*; D018450:Disease Progression; D004359:Drug Therapy, Combination; D004388:Dura Mater / Q000187:drug effects / Q000473:pathology*; D005260:Female; D006801:Humans; D006984:Hypertrophy / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008875:Middle Aged; D055502:Secondary Prevention; D016896:Treatment Outcome
chemical_list: D000305:Adrenal Cortex Hormones; D001379:Azathioprine