14例电压门控钾通道复合物相关抗体自身免疫性脑炎临床分析

Clinical analysis of 14 patients with voltage-gated potassium channels complex antibody encephalitis

目的探讨14例电压门控钾通道(VGKC)复合物相关抗体脑炎患者的临床特点及诊治。方法选择2015-11—2017-09期间作者医院确诊VGKC患者14例,患者均随访至2017年10月底,收集其临床资料进行分析总结。结果 14例患者均为中老年男性,3例以劳累、1例以情绪激动为诱因。有前驱症状者4例,表现为阵发性头晕、心慌、纳差、发作性心动过缓。13例患者病程中至少出现癫痫发作、认知障碍、精神症状中的1项,其中12例患者出现认知功能障碍,8例出现癫痫发作,6例出现精神行为异常。10例有睡眠障碍。8例患者出现自主神经功能障碍。14例患者中3例头MRI异常,主要表现为颞叶、海马、基底节T2FLAIR序列高信号。6例患者出现低钠血症。14例患者中12例脑脊液富亮氨酸胶质瘤失活蛋白1(LGI1)抗体阳性(其中3例同时血清LGI1抗体阳性),1例血清LGI1抗体阳性,1例脑脊液接触蛋白相关蛋白2(Caspr2)抗体阳性。14例患者筛查均未发现肿瘤。静注丙种球蛋白、糖皮质激素或二者联用治疗本病有效。1例LGI1脑炎患者经丙种球蛋白、甲泼尼龙冲击治疗6个月后复发,再次治疗仍有效。结论 VGKC相关抗体脑炎主要表现为边缘叶脑炎、肌肉颤搐、不明原因自主神经功能损害等,免疫治疗有效,可有复发。

Objective To analyse the clinical characteristics,diagnosis and therapy of voltage-gated potassium channels complex(V>UCc)related antibodies autoimmune encephalitisC AE)of 14 patients.Methods We retrospectively analyzed the clinical data of the 14 patients,and made a follow-up for 1-24 months.Results Three patients were precipitaeed by fatigue and one was precipitaeed by emotional agitation.Four patients had prodromal symptoms such as paroxysmal dizziness,palpitation,poor appetite,episodic bradycardia.Thirteen patients had at least one of the following symptoms:seizure,cognitive decline,and psychiatric symptoms.Among them,12 patients had cognitive decline,8 patients had seizures,and 6 patients had psychiatric symptoms.Ten patients suffered from sleep disorders and 8 patients experienced autonomic dysfunction.Brain MRI scans exhibited abnormalities in 3 cases,mainly involving the temporal lobe,hippocampus,and basal ganglia.Six patients developed hyponatremia.One patient showed Caspr2-Ab in the cerebrospinal fluid(CSF),and L>Il-Ab in the CSF and/or the blood was positive in the rest of 13 patient..No tumor was detected by screening.Immunotherapy was effective for all the patients and one patient relapsed.Conclusions VGKCs related antibodies encephalitis is mainly characterized by limbic encephalitis,myokymia and autonomic dysfunction.Immunotherapy is effective and some patients may relapse.