Idiopathic hypertrophic cranial pachymeningitis.
Keywords:
pachymeningitis, optic neuropathy, headache, meningitis, steroids
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.Downloads
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How to Cite
1.
Lizarazo J, Parra E, Gutiérrez P, Mora C, Vargas J. Idiopathic hypertrophic cranial pachymeningitis. Biomed. [Internet]. 2004 Jun. 1 [cited 2024 Aug. 18];24(2):125-32. Available from: https://revistabiomedica.org/index.php/biomedica/article/view/1258
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Published
2004-06-01
Issue
Section
Case presentation
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