抗接触蛋白相关蛋白1抗体阳性慢性炎性脱髓鞘性多发性神经根神经病一例

Anti-contactin-associated protein-1 antibody associated chronic inflammatory demyelinating polyradiculoneuropathy: a case report

抗接触蛋白相关蛋白1(Caspr1)抗体阳性慢性炎性脱髓鞘性多发性神经根神经病(CIDP)为一种罕见的自身免疫性抗体介导的周围神经病。文中报道1例62岁男性患者,临床表现为亚急性起病,肢体远端运动感觉异常,脑脊液蛋白水平升高,对血浆置换治疗有较好反应。下肢肌电图提示以运动受累为主,运动传导速度减慢、复合肌肉动作电位(CMAP)及感觉神经动作电位波幅减低,F波未引出;无症状的上肢肌电图提示正中神经CMAP波幅下降,传导速度正常。全球抗Caspr1阳性CIDP报道并不多见,文中结合相关文献复习,以提高临床医师对该疾病的认识和诊治能力。

Chronic inflammatory demyelinating polyneuropathy(CIDP)with positive anti-contactin-associated protein-1(Caspr1)antibody is a rare autoimmune antibody mediated peripheral neuropathy.A 62-year-old male patient was reported in this article,whose clinical manifestations were subacute onset,abnormal distal limb motor sensation,and increased cerebrospinal fluid protein level.The patient had a good response to plasma exchange.Electromyography of lower limbs showed that motor involvement was dominant,motor conduction velocity slowed down,compound motor active potential(CMAP)and sensory nerve active potential amplitude decreased,and F wave was not elicited;electromyography of upper limbs without symptoms showed that CMAP amplitude of median nerve decreased,and conduction velocity was normal.There are few reports of anti-Caspr1 positive CIDP in the world.The article summarized the characteristics of the patient and reviewed the relevant literature,in order to improve clinicians′understanding and diagnosis and treatment ability of the disease.