摘要
目的探讨抗接触蛋白相关蛋白-2(CASPR2)抗体相关自身免疫性脑炎患者的临床特点、治疗及预后情况,以提高临床对该疾病的认识。方法回顾性分析2018年10月至2020年4月郑州大学第一附属医院收治的5例抗CASPR2抗体相关脑炎患者的临床特点、治疗及预后。结果5例患者中,男2例、女3例,年龄10~62岁,病程5~30 d。主要临床表现:表现为边缘脑炎症状者4例,其中精神行为异常者3例,癫痫发作者2例,记忆力下降者2例;自主神经症状者3例,均表现为窦性心动过速;周围神经高兴奋性症状者2例,其中双手抖动者1例,肌束颤动者1例;睡眠障碍者2例;神经性疼痛1例,表现为肌肉疼痛;小脑症状1例,表现为眼震。脑脊液、血清CASPR2抗体单阳性各2例,双阳性1例。头颅MRI异常2例,均表现为双侧颞叶内侧T2 Flair高信号。5例患者中,接受免疫治疗者3例,其中2例接受糖皮质激素冲击治疗,1例接受血浆置换及口服小剂量激素治疗,2例仅对症治疗,均有不同程度改善。5例患者随访1~19个月,均未出现复发,其中3例恢复正常,1例遗留记忆力下降,不能独立行走,1例死于脑血管意外。结论本组抗CASPR2抗体自身免疫性脑炎患者以边缘脑炎症状最常见,还可出现自主神经症状、周围神经高兴奋性、睡眠障碍等症状,多数患者免疫治疗后预后较好,部分患者可能预后不良。
Objective To investigate the clinical manifestations,treatment and prognosis of patients with anti-Caspr2 antibody-associated autoimmune encephalitis,so as to improve the understanding of the disease.Methods The clinical characteristics,treatment and prognosis of 5 patients with anti-CASPR2 antibody-related encephalitis admitted to the First Affiliated Hospital of Zhengzhou University from October 2018 to April 2020 were retrospectively analyzed.Results Among the 5 patients,there were 2 males and 3 females,aged 10-62 years,and their disease duration was 5-30 d.The main clinical manifestations were:there were 4 cases with limbic encephalitis,including 3 cases with mental and behavior disorder,2 cases with epileptic seizure and 2 cases with memory loss;there were 3 cases with autonomic nervous symptoms,all of which were sinus tachycardia;2 cases with peripheral nerve hyperexcitability syndromes,including 1 case with hands shaking and 1 case with fasciculation;2 cases with sleep disorder;1 case with neuropathic pain,showing muscle pain;1 case with cerebellar symptoms,showing nystagmus.CASPR2 antibody test results:cerebrospinal fluid and blood were single positive in 2 cases each,and double positive in 1 case.There were 2 cases with abnormal cranial MRI showed T2 flair hyperintensity in bilateral medial temporal lobe.Among the 5 patients,3 received immunotherapy,among which 2 were treated with glucocorticoid pulse and 1 were treated with plasmapheresis and oral small dose glucocorticoid and 2 received symptomatic treatment only;all of them improved in different degrees.After 1-19 months of follow-up,none of the patients had recurrence,3 of which were recovered,1 case with memory loss could not walk independently;1 case died of cerebrovascular accident.Conclusions Limbic encephalitis is the most common manifestation in patients with anti-CASPR2 antibody autoimmune encephalitis.Autonomic nervous symptoms,peripheral nerve hyperexcitability,sleep disorders and other symptoms may also occur.Most patients have a good prognosis after immunotherapy,while some patients may have poor prognosis.
作者
古帅鑫
陈立杰
GU Shuaixin;CHEN Lijie(不详;Department of Neurology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou Henan 450052,China)
出处
《中国神经免疫学和神经病学杂志》
CAS
北大核心
2021年第4期297-301,共5页
Chinese Journal of Neuroimmunology and Neurology
作者简介
通讯作者:陈立杰,Email:zuoya120@sina.com。
