Li Qian, Chen Qing, Zhang Ting, Xu Ying, Kan Yanmin, Zhang Jing
Neurology Department, The Third Central Hospital of Tianjin, Tianjin, China.
Tianjin Key Laboratory of Extracorporeal Life Support for Critical Diseases, Tianjin, China.
Front Neurol. 2023 Mar 9;14:1124540. doi: 10.3389/fneur.2023.1124540. eCollection 2023.
Anti-contactin-1 (CNTN1) IgG4 antibody-associated nodopathies is an autoimmune antibody-mediated peripheral neuropathy with a unique clinical presentation, pathophysiology, electrophysiology, and therapeutic response. The critical histopathological features are a dense lymphoplasmacytic infiltrate, a storiform pattern of fibrosis, and obliterative phlebitis. Here, a 62-year-old male patient presented with subacute unilateral limb onset, progressive exacerbation, marked weakness of the extremities, cranial, and autonomic nerve involvement. Neurophysiology showed slowed motor nerve conduction velocity (MCV), prolonged distal motor delay (DML), slowed sensory nerve conduction velocity (SCV), decreased sensory nerve activity potential (SNAP) amplitude, decreased amplitude of bilateral neuromotor conduction, abnormal cutaneous sympathetic response (SSR) in both lower extremities, axonal damage, prolonged F-wave latency, and discrete waves. In the initial phase, there was a response to intravenous immunoglobulin (IVIG), and corticosteroids and rituximab were also effective. After 1 year follow-up, the patient improved significantly. This article reports on a patient with nodular disease with anti-contactin-1 (CNTN1) IgG4 antibodies and reviews the literature to improve clinicians' understanding of the disease.
抗接触蛋白-1（CNTN1）IgG4抗体相关的结节病是一种自身免疫性抗体介导的周围神经病，具有独特的临床表现、病理生理学、电生理学和治疗反应。关键的组织病理学特征是密集的淋巴浆细胞浸润、束状纤维化模式和闭塞性静脉炎。在此，一名62岁男性患者表现为亚急性单侧肢体起病、进行性加重、四肢明显无力、累及颅神经和自主神经。神经生理学检查显示运动神经传导速度（MCV）减慢、远端运动潜伏期（DML）延长、感觉神经传导速度（SCV）减慢、感觉神经动作电位（SNAP）波幅降低、双侧神经运动传导波幅降低、双下肢皮肤交感反应（SSR）异常、轴索性损伤、F波潜伏期延长和离散波。在初始阶段，患者对静脉注射免疫球蛋白（IVIG）有反应，皮质类固醇和利妥昔单抗也有效。经过1年的随访，患者有显著改善。本文报道了一例抗接触蛋白-1（CNTN1）IgG4抗体相关结节病患者，并复习文献以提高临床医生对该病的认识。
