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目的探讨肥厚性硬脑膜炎的病因、发病机制、临床及影像学表现、病理改变及其治疗方法。方法对近年来国内外有关肥厚性脑膜炎的文献资料进行查阅,采纳17篇,予以归类简述。结果肥厚性硬脑膜炎(hypertrophic pachymeningitis,HP)可能是一种自身免疫性疾病。临床表现头痛几乎是首发、必发的症状,大多为隐匿起病,反复发生或迁延不愈。患者可出现多颅神经麻痹及癫痫发作等神经系统受损的各种表现。本病的头颅MRI可见小脑幕和(或)大脑镰等处的硬脑膜局部或弥漫性增厚,增强扫描可见强化。病理特征是硬膜的致密纤维化,慢性炎性细胞如淋巴细胞和浆细胞的浸润,可有肉芽肿样改变。抗生素、皮质类固醇激素、免疫抑制剂治疗有效。出现脑实质或神经受压症状,多需手术治疗。结论特发性肥厚性硬脑膜炎临床表现多样,应注意与其它疾病鉴别。MRI表现较具特异性,确诊依赖于硬脑膜病理学检查。
Objective To investigate the etiology, pathogenesis, clinical and imaging manifestations, pathological changes and treatment of hypertrophic dura meningitis. Methods The literatures about hypertrophic meningitis at home and abroad in recent years were consulted, and 17 articles were adopted, which were classified and summarized. Results hypertrophic pachymeningitis (HP) may be an autoimmune disease. Clinical manifestations Headache is almost the first episode, will be made of the symptoms, mostly occult onset, recurrent or delayed healing. Patients with multiple cranial nerve palsy and epileptic seizures appear various manifestations of nervous system damage. The head MRI of the disease can be seen in the cerebellar tentorium (or) and other falx cerebrum at the local or diffuse thickening, enhanced scan visible enhancement. Pathological features of the dense fibrosis of the dura mater, chronic inflammatory cells such as infiltration of lymphocytes and plasma cells may have granuloma-like changes. Antibiotics, corticosteroids, immunosuppressive agents are effective. Brain parenchymal or nerve compression symptoms, need surgery. Conclusion The clinical manifestations of idiopathic hypertrophic dura meningitis are diverse, and should pay attention to the identification of other diseases. MRI performance is more specific, diagnosis depends on dura pathology.