抗神经束蛋白186抗体介导的急性免疫性感觉性多发性神经根病的临床特点及文献复习

Anti-neurofascin 186 antibody associated acute immune sensory polyradiculopathy:a case report and review of literature

目的:阐述抗神经束蛋白186(NF186)抗体介导的急性免疫性感觉性多发性神经根病(AISP)的临床特征,以加强对AISP/慢性免疫性感觉性多发性神经根病(CISP)的认识。方法:总结2021年12月收入山西医科大学第一医院的1例NF186抗体阳性AISP患者的临床特征,并对既往文献报道的AISP/CISP患者进行系统回顾。结果:患者为62岁男性,表现为急性起病的感觉性共济失调,血清中抗NF186抗体阳性(1∶32),体感诱发电位(SEP)脊髓电位和皮质电位均未记录到反应。糖皮质激素治疗后患者临床症状、SEP均显著改善,目前停药2个月,未复发。经复习文献,目前报道的资料完整的AISP、CISP病例共23例(包括本例),发病年龄(54.7±17.7)岁,男女比例1.88∶1。3例患者为急性起病,归类于AISP。95.7%(22/23)的患者表现为不伴肢体无力的感觉性共济失调,95.0%(19/20)的患者SEP检查结果显示皮质电位潜伏期延长甚至未记录到反应,95.5%(21/22)的患者存在不同程度的脑脊液蛋白升高,神经根增粗或者异常强化并不常见;10例接受免疫治疗的患者均对糖皮质激素或静脉注射免疫球蛋白有效。筛查抗神经节苷脂抗体或郎飞结区-结旁区相关抗体的AISP/CISP文献仅6篇,未见NF186抗体阳性的报道。3例AISP患者均具有CISP/慢性炎性脱髓鞘性多发性神经根神经病的某些特点,AISP与CISP患者在除起病方式以外的临床特征上无差异。结论:抗NF186抗体可以介导AISP,表现为急性起病的感觉性共济失调,糖皮质激素治疗有效。AISP与CISP除起病方式外,二者从临床、电生理、病理、致病抗体方面均难以区分,因此二者可能是一种疾病的两种不同表现方式。

Objective To describe the clinical features of a patient of anti-neurofascin 186(NF186)antibody associated acute immune sensory polyradiculopathy(AISP),and enhance understanding of AISP/chronic immune sensory polyradiculopathy(CISP).Methods The clinical characteristics,diagnosis and treatment of a domestic AISP patient with NF186 antibody positive admitted to the First Hospital of Shanxi Medical University in December 2021 were summarized,and the previously reported cases of AISP/CISP were systematically reviewed.Results The patient was a 62-year-old male with acute onset.The clinical manifestations included severe sensory ataxia,increased protein in cerebrospinal fluid,no response to stimulation of the central segment of somatosensory evoked potentials(SEP),normal sensory and motor nerve conduction,and positive serum anti-NF186 antibody(1∶32).After glucocorticoid treatment,the clinical symptoms and SEP were significantly improved.The drug was stopped for 2 months,and there was no recurrence.There were 23 cases of AISP and CISP with complete data reported in the literature(including this patient).The age of onset was(54.7±17.7)years,and the ratio of male to female was 1.88.Three patients with acute onset were classified as AISP.A total of 95.7%(22/23)of patients showed sensory ataxia without limb weakness,95.0%(19/20)of patients showed prolonged cortical potential latency or even no response,and 95.5%(21/22)of patients showed increased cerebrospinal fluid protein in varying degrees,and nerve root thickening or abnormal enhancement was not common.All 10 patients receiving immunotherapy responded to corticosteroids or intravenous immune globulin.Only 6 AISP/CISP articles reported screening for anti-ganglioside antibodies or Ranvier′s node-paranodal region-related antibodies,and no positive NF186 antibodies were reported.All the 3 patients with AISP had some characteristics of CISP/chronic inflammatory demyelinating polyradiculoneuropathy,and there was no significant difference between AISP and CISP patients in clinical features except the mode of onset.Conclusions NF186 antibody could cause AISP,which presents as acute onset sensory ataxia.AISP is responsive to glucocorticoid therapy.Except for the mode of onset,AISP and CISP are difficult to distinguish from clinical,electrophysiological,pathological aspects and pathogenic antibodies,so they may be two different manifestations of the same disease.