title: [Idiopathic hypertrophic cranial pachymeningitis].
abstract: Idiopathic hypertrophic cranial pachymeningitis is an infrequent chronic inflammatory process of unknown etiology which causes thickening of the dura mater and progressive neurologic alterations due to the compression of adjacent structures. A case is presented of an adult woman with a clinical syndrome consisting of headache, progressive visual loss and bilateral optic neuropathy. The diagnosis was based upon visualization of the thickened dura mater in neuroimaging studies and the exclusion of known causes by histopathological examination. Diagnosis and follow-up of this condition are currently easier with the use of nuclear magnetic resonance with contrast medium. Biopsy of the dura mater continues to be the gold standard for the definitive diagnosis of this disease. Steroid therapy causes clinical improvement in most of the patients; however, relapses are frequent, making necessary the concomitant use of other immunosuppressive agents such as cyclophosphamide or azathioprine. Mortality is low but definitive neurologic sequelae are common.
authors.affiliation: Hospital Universitario Erasmo Meoz, Cúcuta, Norte de Santander, Colombia. jflizar@telecom.com.co
authors.forename: Jairo
authors.initials: J
authors.lastname: Lizarazo
authors.forename: Edgar
authors.initials: E
authors.lastname: Parra
authors.forename: Patricia
authors.initials: P
authors.lastname: Gutiérrez
authors.forename: Carlos
authors.initials: C
authors.lastname: Mora
authors.forename: Juan
authors.initials: J
authors.lastname: Vargas
mesh_terms: D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D006984:Hypertrophy; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000473:pathology*; D008875:Middle Aged