Inflammatory cranial hypertrophic pachymeningitis

Definition: Inflammatory cranial hypertrophic pachymeningitis (ICHP) is a f ibrosing inflammatory process that thickens the dura mater. This condition is increasingly reported owing to the use of CT and MRI. Clinical aspects: Chronic headache and cranial neuropathies are the main pr esentations. Generally the erythrocyte sedimentation rate is elevated and c erebrospinal fluid is inflammatory. Diagnosis: Non-invasive imagery visualizes the thickening of the dura mater that may be focal or diffuse. On MRI diffuse intense enhancement due to in tra cranial hypotension must not be confused with ICHP. Focal thickening of the dura may be tumoral. Biopsy of the thickened dura mater is useful for confirming the inflammatory nature of the process and for orienting the eti ological diagnosis. "Secondary" ICHP: ICHP has many causes, infectious and noninfectious. It ma y be the presenting manifestation of systemic diseases as sarcoidosis or We gener's granulomatosis. "Idiopathic" ICHP: A diagnosis of exclusion, ICHP might be an isolated intr acranial localization of multifocal fibrosis, an iii-defined autoimmune dis ease. Treatment: A specific treatment is indicated in some cases of secondary ICH P. In most cases treatment relies on corticosteroids an/or immunosuppressiv e therapy.