经病理确诊的特发性肥厚性硬脑膜炎1例报道并文献复习

Hypertrophic cranial pachymeningitis:a report of a case and review of the literature

目的探讨特发性肥厚性硬脑膜炎的临床表现、影像学特征、诊断及治疗。方法报道本院1例经病理确诊的特发性肥厚性硬脑膜炎患者的临床资料并复习相关文献。结果本例患者表现为慢性反复头痛,头颅磁共振增强扫描示左侧小脑幕异常增生并强化,病理活检提示大量慢性炎症细胞浸润,经过激素冲击治疗头痛缓解,后续小剂量激素联合免疫抑制剂甲氨蝶呤口服,临床预后良好。结论特发性肥厚性硬脑膜炎病因复杂,多以慢性头痛、多组脑神经麻痹及小脑性共济失调为主要临床表现;头颅MRI可见特征性硬脑膜肥厚及强化表现;临床需与多种颅内疾病相鉴别,病理活检可确诊;激素治疗基础上联合免疫抑制剂可防止病情复发。

Objective To explore the clinical presentations、imaging features、diagnose and therapy of hypertrophic cranial pachymengitis（HCP）.Methods The clinical data of the patient with HCP were studied retrospeotively,and the literature was reviewed.Results The patient showed chronic recurrent headaches,enhanced MRI scan showed left tentorium cerebelli had informal hyperplasia and strengthening,the pathological biopsy indicated the existence of a large number of chronic inflammatory cells.After the hormone impact treatment to relieve headache,subsequent small dose of hormone combined with immunosuppressive drugs methotrexate orally,the prognosis is good.Conclusion Anti-LGI1 encephalitis often show temporal lobe epilepsy,mental disorders as the main clinical manifestations accompanied by hyponatremia.The flair MRI scans mainly show high signals in the medial temporal lobe,immune regulation and high dose hormone treatment is remarkable.