影像呈结节状占位样的特发性肥厚性硬脑膜炎临床分析

A clinical analysis of idiopathic hypertrophic cranial pachymeningitis with a nodular space-occupying effect

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.

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.