特发性肥厚性硬脑膜炎的临床和影像学特征

目的 通过对6例特发性肥厚性硬脑膜炎临床资料的分析及文献回顾,总结该病的诊断及治疗方法,以期降低其误诊误治率. 方法 结合文献对2005至2006年我科收治的6例该病患者的临床特点、影像学表现及随访结果进行分析. 结果 特发性肥厚性硬脑膜炎均存在不同程度的免疫功能异常.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.皮质类固醇治疗有效. 结论 特发性肥厚性硬脑膜炎可能是一种自身免疫性疾病.本病临床表现多样,应注意与其它疾病鉴别.应重视头颅MRI的诊断意义.

Clinical and imaging characteristics of idiopathic hypertrophic pachymeningitis

Objective To analyze clinical data of 6 cases of idiopathic hypertrophic pachymeningitis and review related literatures before, so as to offer a guidance for its clinical diagnosis and therapy. Methods We analyzed the clinical and imaging features and data of follow_up in the patients who were treated in our hospital 2005-2006. Results Idiopathic hypertrophic pachymeningitis was always accompanied by immune fimction abnormality to different degrees. The main clinical manifestation was headache which was found in every case. In these cases, headache was the onset and recur symptom. In addition to headache, various symptoms of nervous system dysfunction especially the cranial nerves could be found in some cases. In these cases, a localized or diffuse thickening of tentorium of cerebellum and/or falciform process of cerebrum was found in MRI, and intensification was presented in enhancement scanning. Corticosteroid was effective to all of the cases. Conclusions Idiopathic hypertrophic pachymeningitis may be an autoimmune disorder, of which the symptoms are various, and need to be discriminated from other diseases. MR/of cranium is important criteria for diagnosis.