title: [Hypertrophic pachymeningitis: three adult cases and a review of the literature].
abstract: Hypertrophic pachymeningitis (HP) is thought to have an autoimmune etiology but its precise cause and treatment remains to be elucidated. Here, we report the clinical details and therapeutic responses of 3 patients with HP and reviewed 66 previously reported cases in the literature. Among these patients, headache was the most frequent complaint. Cranial nerve involvement was also frequently observed, with the optic nerve being the most frequently impaired followed by the oculomotor, trochlear, and abducens nerves in frequency. Elevated C-reactive protein levels and erythrocyte sedimentation rates were found in approximately 97% of the patients. Steroids were the most commonly prescribed therapy, but no definite protocols for the standard dose and duration in HP have been proposed thus far. The average initial dose of prednisolone (PSL) was 42.7 mg/day, and the average maintenance dose was 12.4 mg/day in the chronic stage. Recurrence occurred in many patients when the dose of PSL was reduced to under 20 mg/day. Therefore, steroids should be tapered extremely slowly.
authors: Department of Neurology, Fujita Health University School of Medicine.
authors: Akihiro
authors: A
authors: Ueda
authors: Madoka
authors: M
authors: Ueda
authors: Takateru
authors: T
authors: Mihara
authors: Shinji
authors: S
authors: Ito
authors: Kunihiko
authors: K
authors: Asakura
authors: Tatsuro
authors: T
authors: Mutoh