眼肌麻痹合并抗Caspr2抗体阳性一例报道并文献复习

Ophthalmoplegia complicated with positive anti-Caspr2 antibody:a case report and literature review

目的探讨接触蛋白相关样蛋白2(contactin-associated protein 2,Caspr2)抗体与眼肌麻痹的相关性。方法报道1例眼肌麻痹合并抗Caspr2抗体阳性患者的临床资料,通过文献复习对眼肌麻痹的病因进行分析。结果患者为中年男性,亚急性起病,缓慢进展,以复视为主要表现;神经系统查体发现双眼睑下垂,双眼内收及上下视运动受限;血清抗Caspr2抗体阳性;抗乙酰胆碱受体(AChR)抗体、抗肌肉特异性酪氨酸肌酶抗体(Musk)抗体、副肿瘤综合征相关抗体、神经节苷脂抗体(GM1、GQ1b)均阴性;重复频率电刺激、肌电图及胸部CT检查结果均正常;PET-CT检查见双侧大脑皮层代谢活性减低,双侧基底节及丘脑代谢活性增高,经排他诊断,不除外眼肌麻痹与Caspr2抗体阳性有关;给予大剂量甲强龙冲击并序贯递减,经治疗1个月后患者症状减轻,3个月后症状完全消失,血清抗Caspr2抗体转阴。结论该例患者的眼肌麻痹可能与抗Caspr2抗体阳性有关。

Objective To investigate the correlation between contactin-associated protein 2(Caspr2)antibody and ophthalmoplegia.Methods We collected the clinical data of a case of ophthalmoplegia with positive anti-Caspr2 antibody,the etiology of ophthalmoplegia was analyzed by literature review,and corresponding treatments were given to observe the prognosis.Results The patient was a middle-aged man with subacute onset and slow progression,mainly manifested as diplopia,neurological examination revealed bilateral eyelid drooping,limited binocular adduction and restricted up and down movement;serum anti-Caspr2 antibody was positive,anti-AChR antibody,anti-Musk antibody,anti-paraneoplastic syndrome antibody and ganglioside antibody(GM1,GQ1b)were negative.Repetitive nerve stimulation(RNS),electromyography(EMG)and chest CT were normal;PET-CT showed decreased metabolic activity in bilateral cerebral cortex,increased metabolic activity in bilateral basal ganglia and thalamus.We thought the ophthalmoplegia might be related to the Caspr2 antibody,so we gave the patient a high dose of methylprednisolone and the patient gradually tapered off corticosteroids,the patient s symptoms alleviated after a month.Three months later,all the symptoms disappeared completely,and serum anti-Caspr2 antibody became negative.Conclusions Ophthalmoplegia in this patient may be associated with positive anti-Caspr2 antibody.