急性泛自主神经病的临床分析及文献复习  

Clinical Characteristics of Acute Panautonomic Neuropathy and Literature Review

作　　者：熊铁根 项薇 武肖娜 彭凯润 杨红军 邓兵梅 XIONG Tie-gen;XIANG Wei;WU Xiao-na;PENG Kai-run;YANG Hong-jun;DENG Bing-mei(Department of Neurology,General Hospital of Southern Theatre Command,Guangzhou 510010,China)

机构地区：[1]中国人民解放军南部战区总医院神经内科,广州510010

出　　处：《神经损伤与功能重建》2020年第12期718-721,共4页Neural Injury and Functional Reconstruction

基　　金：广州市科技计划项目(No.201904010386)。

摘　　要：目的:探讨急性泛自主神经病(APN)的临床特点,为临床早期诊断和治疗提供依据。方法:报道我院2例APN,并结合文献进行回顾性分析。结果:2例APN患者均为中年男性;急性或亚急性起病,均有明确的自主神经功能障碍。2例患者脑脊液和神经传导速度检查未见明显异常。早期激素、免疫球蛋白治疗等可改善病情;病例1随访8年,遗留有胃肠功能低下,长期需要胃肠外营养维持;病例2随访3年,遗留轻度体位性低血压,站立后偶有轻度头晕,可缓慢行走,生活自理。搜索既往报道的APN患者54例,体位性低血压是最常见的临床表现,占67%;早期激素及免疫球蛋白治疗,大部分患者预后较好。结论:APN以广泛的自主神经功能障碍尤其是体位性低血压为主要临床表现,辅助检查无特征性,激素与丙球治疗有效,预后较好,可能遗留有不同程度后遗症。Objective:To investigate the clinical characteristics of acute panautonomic neuropathy(APN),which may be helpful for its early diagnosis and treatment.Methods:In this paper,2 cases of APN from our hospital were reported and the relevant literature was reviewed.Results:The 2 APN patients were both male and middle-aged.Both patients showed acute or subacute onset with apparent dysfunction of the autonomic nervous system.Both cases presented normal cerebrospinal fluid and nerve conduction velocity.Early treatment with corticosteroid and immunoglobulins resulted in improved prognosis in the patients.Patient 1 was followed for 8 years and showed a lingering reduction in gastrointestinal function that required the use of parenteral nutrition for management.Patient 2 was followed for 3 years and showed residual mild orthostatic hypotension marked by occasional dizziness after standing;the patient was able to walk slowly and was capable of self-care.We collected the data of 54 cases of APN from MEDLINE.Most of these patients(67%)presented with orthostatic hypotension.Early intervention with corticosteroid and immunoglobulins resulted in a good prognosis for most patients.Conclusion:APN presents with widespread dysautonomia,especially orthostatic hypotension.Auxiliary examinations show no outstanding feature.Corticosteroid or immunoglobulins therapy are effective and result in a good prognosis.Patients may develop sequelae of varying severity.

关 键 词：吉兰-巴雷综合征 急性泛自主神经病 体位性低血压 激素 免疫球蛋白 

分 类 号：R741[医药卫生—神经病学与精神病学] R741.02[医药卫生—临床医学]