| 熊铁根，项薇，武肖娜，彭凯润，杨红军，邓兵梅.急性泛自主神经病的临床分析及文献复习[J].神经损伤功能重建,2020,15(12):718-721 |
| 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 |
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| DOI： |
| 中文关键词: 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 |
| 英文关键词: Guillain-Barré syndrome acute panautonomic neuropathy orthostatic hypotension corticosteroid immunoglobulins |
| 基金项目:广州市科技计划项
目（No. 2019040103
86） |
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| 中文摘要: |
| 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。 |
| 英文摘要: |
| 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. |
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