Masson C, Boukriche Y, Colombani J M
Service de Neurologie, Hôpital Beaujon, 100, bd du Général Lederc, F 92110 Clichy.
Presse Med. 2001 Mar 10;30(9):411-6.
Inflammatory cranial hypertrophic pachymeningitis (ICHP) is a fibrosing inflammatory process that thickens the dura mater. This condition is increasingly reported owing to the use of CT and MRI.
Chronic headache and cranial neuropathies are the main presentations. Generally the erythrocyte sedimentation rate is elevated and cerebrospinal fluid is inflammatory.
Non-invasive imagery visualizes the thickening of the dura mater that may be focal or diffuse. On MRI diffuse intense enhancement due to intra 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 etiological diagnosis. "SECONDARY" ICHP: ICHP has many causes, infectious and noninfectious. It may be the presenting manifestation of systemic diseases as sarcoïdosis or Wegener's granulomatosis. "IDIOPATHIC" ICHP: A diagnosis of exclusion, ICHP might be an isolated intracranial localization of multifocal fibrosis, an ill-defined autoimmune disease.
A specific treatment is indicated in some cases of secondary ICHP. In most cases treatment relies on corticosteroids an/or immunosuppressive therapy.
炎性颅骨肥厚性硬脑膜炎（ICHP）是一种使硬脑膜增厚的纤维性炎症过程。由于CT和MRI的应用，这种病症的报告越来越多。
慢性头痛和颅神经病变是主要表现。一般红细胞沉降率升高，脑脊液呈炎性。
非侵入性影像学检查可显示硬脑膜增厚，增厚可能是局灶性或弥漫性的。在MRI上，由于颅内低压导致的弥漫性强化不能与ICHP相混淆。硬脑膜的局灶性增厚可能是肿瘤性的。对增厚的硬脑膜进行活检有助于确认该过程的炎性性质并指导病因诊断。“继发性”ICHP：ICHP有多种病因，包括感染性和非感染性。它可能是结节病或韦格纳肉芽肿等全身性疾病的首发表现。“特发性”ICHP：作为一种排除性诊断，ICHP可能是多灶性纤维化的孤立性颅内定位，是一种定义不明确的自身免疫性疾病。
在某些继发性ICHP病例中需要进行特异性治疗。在大多数情况下，治疗依赖于皮质类固醇和/或免疫抑制疗法。
