Guo Y P, Li X Y, Liu H F, Zhang M, Shi L, Zhao X J, Li J Z, Liu X Y, Cui J
Department of Neurology, Fifth Affiliated Hospital, Zhengzhou University, Zhengzhou 450052, China.
Department of Neurosurgery, First Affiliated Hospital, Zhengzhou University, Zhengzhou 450052, China.
Zhonghua Yi Xue Za Zhi. 2020 Feb 25;100(7):513-515. doi: 10.3760/cma.j.issn.0376-2491.2020.07.007.
To investigate the clinical features, laboratory tests, imaging features, electroencephalogram (EEG) manifestations, treatment and prognosis of anti-CASPR2 antibody-associated autoimmune encephalitis with the purpose to improve the understanding of the disease. Seven patients diagnosed with CASPR2 antibody-associated encephalitis in the Fifth and first Affiliated Hospital of Zhengzhou University from January 2017 to January 2019 were selected. The clinical manifestations, course of disease, imaging, treatment plan and prognosis of the patients were retrospectively analyzed. Of the 7 patients, 4 were male and 3 were female. The median age of onset was 34 years old (range, 11-66 years). Seizures (6/7), memory decline(5/7), and neuropsychiatric disorders (4/7) were common clinical manifestations. CASPR2 antibody was detected in the CSF of 3 patients (3/7) and the serum of all patients (7/7). Notable imaging changes were observed in 5 patients (5/7). All patients received immunotherapy and were followed up for 1 month to 2 years. One patient was diagnosed with rectal cancer with a poor prognosis; all the others got a good prognosis. CASPR2 antibody-related encephalitis is a rare autoimmune disease. It is considered to present with seizures, memory loss, psychiatric symptoms, dizziness and sleep disorders as the main clinical manifestations. Early identification and treatment of the disease can help the patients achieve a good prognosis.
为探讨抗接触蛋白相关蛋白2(CASPR2)抗体相关自身免疫性脑炎的临床特征、实验室检查、影像学特征、脑电图(EEG)表现、治疗及预后情况,以提高对该疾病的认识。选取2017年1月至2019年1月在郑州大学第五附属医院及第一附属医院确诊为CASPR2抗体相关脑炎的7例患者。对患者的临床表现、病程、影像学、治疗方案及预后进行回顾性分析。7例患者中,男性4例,女性3例。发病年龄中位数为34岁(范围11 - 66岁)。癫痫发作(6/7)、记忆力减退(5/7)及神经精神障碍(4/7)为常见临床表现。3例患者(3/7)脑脊液及所有患者血清(7/7)中均检测到CASPR2抗体。5例患者(5/7)出现明显影像学改变。所有患者均接受免疫治疗,随访1个月至2年。1例患者诊断为直肠癌,预后不良;其他患者预后良好。CASPR2抗体相关脑炎是一种罕见的自身免疫性疾病。其临床主要表现为癫痫发作、记忆力丧失、精神症状、头晕及睡眠障碍。早期识别和治疗该病有助于患者获得良好预后。