以急性全自主神经功能障碍发病的自身免疫性自主神经病临床特点分析

doi:10.3969/j.issn.1672-6731.2020.10.005

摘要/Abstract

摘要：

研究背景 以急性全自主神经功能障碍发病的自身免疫性自主神经病临床主要表现为晕厥、胃肠动力障碍，常误诊为心血管系统、消化系统或泌尿系统疾病而延误治疗。总结以急性全自主神经功能障碍发病的自身免疫性自主神经病的临床表现、实验室、电生理学、病理学、治疗与随访特点，可为诊断与鉴别诊断、制定治疗方案提供借鉴。方法与结果 2016年2月至2019年2月共确诊3例以急性全自主神经功能障碍发病的自身免疫性自主神经病患者，均为青年女性，典型表现呈急性发病的直立性低血压和胃肠动力障碍，伴不同程度大小便障碍和全身排汗障碍（2例），或瞳孔改变（2例）。神经传导检测存在感觉神经和运动神经传导速度减慢（2例）、F波和H反射异常（2例），但大直径有髓纤维传导速度可无异常（1例）；急性期病例自主神经功能试验Valsalva率和深呼吸心率变异下降（2例），直立倾斜试验卧位与直立位血压差异> 30/15 mm Hg（2例）；腰椎穿刺脑脊液存在蛋白-细胞分离现象（1/2例）；所有患者均存在广泛性小直径有髓纤维和无髓纤维丢失。急性期患者对糖皮质激素联合静脉注射免疫球蛋白反应良好（2例）。结论以急性全自主神经功能障碍发病的自身免疫性自主神经病具有独特的临床表现，早期诊断、积极治疗可改善临床预后。

关键词: 自主神经系统疾病, 自身免疫疾病, 肌电描记术, 神经传导, 腓肠神经, 活组织检查

Abstract:

Background Autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction is often misdiagnosed because its main symptoms are syncope and gastrointestinal motility disorder. This study summarized the clinical, electrophysiological and pathological changes of peripheral nerve biopsy of patients of autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction, which could provide reference for diagnosis, differential diagnosis and treatment. Methods and Results The clinical data of 3 patients diagnosied with autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction who were hospitalized in Department of Neurology, Peking University First Hospital from February 2016 to February 2019 were collected. All of the 3 patients were young women, presented with onset symptoms as acute orthostatic hypotension and gastrointestinal motility disorder. Two patients had dysuria and dyshidrosis, and 2 patients had pupil changes. Electrophysiology revealed decreased sensory and motor nerve conduction velocity in 2 patients. Abnormal F wave and H reflex were found in 2 patients. The conduction velocity of large myelinated fibers was normal in one patient. In the autonomic function test, 2 patients had decreased Valsalva ratio, decreased heart rate variability of deep breathing. The blood pressure changes during tilt test between lying and upright position of 2 patients in acute stage was more than 30/15 mm Hg. Two patients underwent lumbar puncture, and one patient showed albuminocytologic dissociation. Sural nerve biopsies showed moderate to marked loss of small myelinated fibers and unmyelinated fibers. Two patients in acute stage were treated with immunomodulatory therapy (glucocorticoid combined with intravenous immunoglobulin), and their symptoms improved in varying degrees. Conclusions Autoimmune autonomic neuropathy with acute autonomic nerve dysfunction has unique clinical manifestations. Early consideration of autoimmune autonomic neuropathy followed by diagnostic work-up and prompt immune treatment will help to improve the prognosis.

Key words: Autonomic nervous system diseases, Autoimmune diseases, Electromyography, Neural conduction, Sural nerve, Biopsy

李凡, 孟令超, 白静, 孙云闯, 李茹洁, 石昕, 郝洪军, 高枫, 贾志荣, 黄一宁, 袁云, 王朝霞. 以急性全自主神经功能障碍发病的自身免疫性自主神经病临床特点分析[J]. 中国现代神经疾病杂志, 2020, 20(10): 868-876.

LI Fan, MENG Ling-chao, BAI Jing, SUN Yun-chuang, LI Ru-jie, SHI Xin, HAO Hong-jun, GAO Feng, JIA Zhi-rong, HUANG Yi-ning, YUAN Yun, WANG Zhao-xia. Clinical features of autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2020, 20(10): 868-876.

http://journal11.magtechjournal.com/cjcnn/CN/Y2020/V20/I10/868

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2 以急性全自主神经功能障碍发病的自身免疫性自主神经病临床特点分析

Clinical features of autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction

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2 以急性全自主神经功能障碍发病的自身免疫性自主神经病临床特点分析

Clinical features of autoimmune autonomic neuropathy with onset of acute autonomic nerve dysfunction

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