CAJ | 학술논문

目的探讨影像呈结节状占位样的特发性肥厚性硬脑膜炎(IHCP)的临床、影像及病理特点.方法对4例影像呈结节状的IHCP患者的临床资料进行回顾性分析.结果 4例均为男性,平均发病年龄40.25(33 ～50)岁,病程长(4个月～12年),病情反复发作.主要表现为慢性头痛、多组脑神经麻痹及癫痫发作.头颅影像示局部硬脑膜增厚,呈团块或结节状,与脑膜瘤等肿瘤相似.2例组织病理学检查示硬脑膜结缔组织增生、散在中性粒细胞及浆细胞浸润.甲泼尼龙或联合免疫抑制剂治疗有效.结论结节状占位样IHCP多以慢性头痛起病,糖皮质激素或联合免疫抑制剂治疗有效,病情易复发.头颅影像可见硬脑膜肥厚,呈团块或结节状占位,易与脑肿瘤、炎性肉芽肿相混淆.
목적 탐토영상정결절상점위양적특발성비후성경뇌막염(IHCP)적림상、영상급병리특점.방법 대4례영상정결절상적IHCP환자적림상자료진행회고성분석.결과 4례균위남성,평균발병년령40.25(33 ～50)세,병정장(4개월～12년),병정반복발작.주요표현위만성두통、다조뇌신경마비급전간발작.두로영상시국부경뇌막증후,정단괴혹결절상,여뇌막류등종류상사.2례조직병이학검사시경뇌막결체조직증생、산재중성립세포급장세포침윤.갑발니룡혹연합면역억제제치료유효.결론 결절상점위양IHCP다이만성두통기병,당피질격소혹연합면역억제제치료유효,병정역복발.두로영상가견경뇌막비후,정단괴혹결절상점위,역여뇌종류、염성육아종상혼효.
Objective To explore the clinical features,neuroimaging and histopathological findings in patients with idiopathic hypertrophic cranial pachymeningitis (IHCP) with a nodular space occupying effect.Methods Four IHCP cases with a nodular space occupying effect diagnosed in our hospital were retrospectively studied.Results All the 4 patients were men with a mean onset age of 40.25 (33 ～ 50) years old.They all had long disease duration and relapses.The common symptoms of IHCP were chronic headache,multiple cranial nerve palsies and epileptic seizures.CT and MRI of the brain revealed prominent dural partial thickening,which indicated a mass or nodular space occupying effect that mimicked intracranial tumour-like meningioma.The histopathological findings of dura in 2 cases revealed connective tissue proliferation,scattered neutrophile granulocytes and plasmacytes infiltration.Combination therapy of corticosteroid or/and immunosuppressive drugs was effective for the IHCP patients.Conclusions IHCP patients with a nodular space occupying effect usually onset with chronic headache and are often recurrent.The combination therapy of corticosteroid or/and immunosuppressive drugs is effective.The image of the brain presents prominent dural partial thickening,indicating a mass or nodular space occupying effect,which often lead to confusion with intracranial tumours or granulomatosis.