特发性肥厚性硬脑膜炎的临床分析

Clinical analysis of idiopathic hypertrophic cranial pachymeningitis

摘要:

目的 探讨特发性肥厚性硬脑膜炎（idiopathic hypertrophic cranial pachymeningitis，IHCP）的临床特点、发病机制、影像学特征、病理改变、治疗方法及预后。方法 回顾性分析诊治的4例IHCP患者的临床资料并结合国内外相关的文献，予以总结归纳。结果头痛常为IHCP首发症状，大多隐匿起病，反复发生或迁延不愈。患者可出现多组脑神经受损的表现。4例患者中3例免疫指标异常，1例伴有免疫系统疾病；故本病可能与免疫功能异常有关。结论特发性肥厚性硬脑膜炎临床表现多样，头颅影像学检查具有特异性，确诊依赖于硬脑膜病理学检查。

Abstract:

Abstract:ObjectiveTo study the clinical characteristics, pathogenesis, radiological and pathological features, treatment and prognosis of idiopathic hypertrophic cranial pachymeningitis (IHCP).MethodsClinical data of 4 patients suffering from IHCP and hospitalized in our department was analyzed retrospectively with literature review.ResultsIn these cases, headache was the initial symptom with characteristics of occult onset, recurrence and protracted course. Chronic headache and multiple progressive cranial nerve palsy were the chief clinical manifestations in IHCP. Abnormality of immunologic indexes was detected in 3 patients and one patient was accompanied by systemic immunologic disease. Therefore, abnormality of immunologic function might participate in the pathogenesis of IHCP.ConclusionsClinical manifestations of IHCP are usually diversified, partly thickened and enhanced dura mater on MRI is the specific radiological sign of this disease. The diagnosis would depend on the pathological examination of dura mater.