目的：总结以抗利尿激素分泌不当综合征（SIADH）为首发表现的吉兰-巴雷综合征（GBS）的临床特征和诊治情况。方法检索国内外1980~2014年发表的以SIADH为首发表现的GBS病例报道，对纳入病例进行分析，探讨其临床特点。结果共纳入4例病例，均为女性，发病年龄38~82岁，中位年龄62.5岁。初期表现为精神状态改变（100%）、头痛（50%）、疲乏（50%）、背痛（50%）、呼之不应（25%）、呕吐（25%）等，查体常无明显神经系统损伤定位体征（75%）。发病至GBS典型症状出现时间3~15 d，中位时间5 d。结论该病发病机制尚未明确，病情较重、诊断困难。明确诊断后，治疗反应可，预后好。需行肿瘤筛查，排除副肿瘤综合征可能。%Objective To summarize the clinical characteristics and management of Guillain-Barre syndrome(GBS) with syndrome of inappropriate antidiuretic hormone(SIADH) as the initial presentation. Methods A systematic retrieval of GBS with SIADH as the initial presentation in 1980-2014 was performed. The patients in the study were analyzed to explore the clinical characteristics. Results All the 4 patients were females,and the age of onset was from 38 to 82 years old with a median age of 62.5 years. The main symptoms and signs in the early stages of disease included altered mental status (100%),headache (50%),malaise(50%),back pain(50%),no response to voice(25%),vomiting(25%) and so on. Physical examination showed that nervous system impairment had no obvious locating features (75%). The interval time between the onset and the day on which typical symptoms of GBS appeared,varied from 3 to 15 d,with a median time of 5 d. Conclusion Pathogenesis of this disease is still not clear,it often appears with severe and complicated clinical situations,which is difficult to diagnose. Malignant tumor screening is needed to exclude the possibility that paraneoplastic syndrome.
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