摘要
目的总结抗amphiphysin抗体阳性副肿瘤神经综合征(PNS)的临床特点和预后,以提高临床对此病的认识。方法回顾性分析2015年04月至2018年11月安徽医科大学第一附属医院收治12例抗amphiphysin抗体阳性的PNS患者临床资料。结果12例患者中,主要表现为肌无力或共济失调6例,肢体麻木为主2例,癫痫为主2例,认知障碍为主2例;10例患者肿瘤指标异常;2例患者影像学筛查提示肿瘤明确阳性,5例患者影像学筛查提示疑似肿瘤;病理检查明确肿瘤2例;5例患者经糖皮质激素治疗,2例经丙种球蛋白治疗,2例患者选择外科治疗。随访显示病情好转7例,死亡3例,失随访2例。结论抗amphiphysin抗体阳性相关的PNS临床症状多样,免疫治疗能使部分患者获益,及时诊断治疗利于恢复,预后一般。
Objective Clinical features and prognosis of anti-amphiphysin antibody positive paraneoplastic neurological syndrome(PNS)were summarized to provide more insights.Methods The clinical examination and laboratory data of 12 anti-amphiphysin antibody positive PNS patients from the First Affiliated Hospital of Anhui Medical University from April 2015 to November 2018 were collected and analyzed retrospectively.Results Among the 12 patients,6 patients presented with myasthenia or ataxia,2 patients presented with numbness,2 patients present with epilepsy and 2 patients presented with cognitive impairment.Abnormal tumor markers were found in 10 patients.Definite tumors were found in 2 patients and suspicious tumors were found in 5 patients with imaging examinations.Two patients were diagnosed with tumor by histopathological examination.Five patients were treated with corticosteroids.Two patients were treated with intravenous immunoglobulin.Two patients were treated by surgery.All the patients were followed up.Seven patients were improved,3 patients died and 2 patients withdrew.Conclusions Clinical symptoms of anti-amphiphysin antibody positive PNS vary.Some patients can be improved with immunotherapy.Patients can benefit from early diagnosis and therapy.
作者
陈越
郭媛媛
魏强
周农
汪凯
田仰华
CHEN Yue;GUO Yuanyuan;WEI Qiang;ZHOU Nong;WANG Kai;TIAN Yanghua(Department of Neurology,the First Affiliated Hospital of Anhui Medical University,Hefei Anhui 230032,China)
出处
《中国神经免疫学和神经病学杂志》
CAS
北大核心
2020年第3期190-194,共5页
Chinese Journal of Neuroimmunology and Neurology
基金
国家自然科学基金资助项目(81671354)
安徽省自然科学基金杰出青年基金资助项目(1808085J23)。