Hypertrophic chronic pachymeningitis as a localized immune process in the craniocervical region

Hypertrophic chronic pachymeningitis (HCP) is a rare disorder that causes i ntracranial or spinal thickening of the dura mater. This report describes a patient with progressive HCP in the craniocervical region associated with signs of rheumatic disease. A ventricular-atrial shunt had to be inserted b ecause of increased intracranial pressure. The patient improved after suboc cipital craniotomy, C1 to C6 laminectomy, and removal of the thickened dura . Additional therapy with methotrexate stopped progression, which was docum ented by MRI and PET.