肥厚性硬脑膜炎5例报道及文献复习

    Hypertrophic cranial pachymeningitis:a report of 5 cases and review of the literature

    • 摘要: 目的:探讨肥厚性硬脑膜炎(hypertrophic cranial pachymeningitis,HCP)的临床、病理和影像学特点。方法:对5例HCP患者的临床资料结合文献进行回顾性分析。结果:5例患者均表现为头痛,其中2例伴言语障碍;1例伴肢体抽搐,反应迟钝,颅神经受损,行走不稳;1例伴记忆障碍。MRI平扫2例显示脑实质内有水肿改变,增强后5例硬脑膜广泛强化,尤其是额、顶部硬脑膜、大脑镰及小脑幕强化明显。1例行硬脑膜组织活检发现硬脑膜明显增厚,病理示硬脑膜纤维增生,大量浆细胞、淋巴细胞、巨噬细胞、嗜中性粒细胞浸润,灶性钙化,考虑为慢性非特异性炎;"硬膜下方"脑组织血管扩张,浆细胞、巨噬细胞为主的炎细胞浸润,相应部位脑实质也为非特异性炎性改变。结论:HCP的病因复杂,临床表现多有头痛、肢体抽搐、反应迟钝、言语障碍、记忆障碍、行走不稳、颅神经受损等症状;病理示硬脑膜纤维增生,炎细胞浸润,相应部位脑实质也可有非特异性炎性改变;头颅MRI增强发现典型的影像学改变有利于临床诊断。

       

      Abstract: Objective:To determine the clinical,pathological and radiological features of hypertrophic cranial pachymeningitis (HCP).Methods:The clinical data of 5 patients with HCP were studied retrospectively,and the literature was reviewed.Results:All the 5 patients presented the symptom of headache.Two of them were accompanied by language barriers,1 by limb convulsion,reaction slowness,cranial nerve damage and walking instability,and 1 by memory impairment.MRI scan showed that 2 cases displayed edema changes within the brain parenchyma,and there was a wide range of enhanced thickening in all the 5 cases after enhancement,particularly at the frontal and top of the dura mater,the falx and the tentorium.The brain biopsy of 1 patient showed that the dura mater was significantly thickened,and the dural fibrosis pathology indicated the existence of a large number of plasma cell,lymphocyte,macrophage,neutrophil infiltration and focal calcification,which was considered to be a non-specific chronic inflammation.The brain blood vessels under the dura mater were dilated,with plasma cell and macrophage infiltration.The corresponding brain parenchyma also displayed non-specific inflammatory changes.Conclusions:Causes of HCP are complicated.The common clinical manifestations include headache,body convulsion,reaction slowness,speech impairment,memory impairment,walking instability and cranial nerve damage;Pathology showed dural fibrosis and inflammatory cell infiltration;the corresponding parts of the brain parenchyma may also display non-specific inflammatory changes,and such typical changes in head MRI enhanced imaging is beneficial to clinical diagnosis.

       

    /

    返回文章
    返回