Kuhn J, Harzheim A, Riku S, Müller W, Bewermeyer H
Neurologische Klinik, Krankenhaus Merheim, Kliniken der Stadt Köln gGmbH.
Nervenarzt. 2006 Apr;77(4):423-9. doi: 10.1007/s00115-005-2012-8.
Hypertrophic cranial pachymeningitis is an uncommon, fibrosing, inflammatory process that involves the dura mater. The condition is being reported more frequently owing to the use of cranial MRI. The main clinical feature is headache, whereas cranial nerve lesions, cerebellar symptoms, and epileptic seizures occur more rarely. A variety of autoimmune and infectious diseases can result in this condition, which is labeled as idiopathic in the absence of any definite inciting factor. The diagnosis of hypertrophic cranial pachymeningitis is based on neuroimaging of thickened and enhancing dura mater. It can be defined pathologically on biopsy. A specific treatment is indicated in some cases of secondary hypertrophic cranial pachymeningitis. Mostly, treatment relies on corticosteroids and immunosuppressive agents. This review summarizes the current knowledge on causes, clinical presentation, diagnosis, and treatment of this disorder.
肥厚性硬脑膜炎是一种罕见的、纤维化的炎症性疾病，累及硬脑膜。由于头颅磁共振成像（MRI）的应用，该病的报道越来越多。主要临床特征为头痛，而脑神经病变、小脑症状和癫痫发作则较为少见。多种自身免疫性和感染性疾病可导致该病，在没有任何明确诱发因素的情况下被标记为特发性。肥厚性硬脑膜炎的诊断基于硬脑膜增厚和强化的神经影像学表现。活检可进行病理诊断。继发性肥厚性硬脑膜炎的某些病例需要特定治疗。大多数情况下，治疗依赖于皮质类固醇和免疫抑制剂。本综述总结了关于该疾病病因、临床表现、诊断和治疗的现有知识。
