格林-巴利综合征

title: 症状和体征.
content: 该病最初的典型表现为感觉异常、疼痛伴肌无力,症状首现于四肢远端,即手足,之后症状常常在数小时至数周内不断向近端进展,躯干、头部也会出现症状。一般两侧对称。在疾病的急性期,15%患者可能因为呼吸肌受累而有生命危险,需要机械式呼吸辅助。本病除累及运动神经、感觉神经,导致上述典型症状外,还有一些患者可能有自主神经系统受累,导致诸如血压异常、心率异常等症状,也可能导致患者的死亡。
title: 病因和诊断.
content: 本病通常被认为是自体免疫性疾病,由人体的免疫系统错误地攻击自身周围神经的髓鞘,导致患者周围神经系统出现脱髓鞘损害。有的患者还会出现轴索损害。本病的病因尚不明确。患者的免疫系统紊乱有时由感染诱发,由手术或疫苗诱发的少见,还有很多病人无明显诱发因素。本病的诊断一般基于症状和体征,要排除其他疾病的可能,并有诸如肌电图神经传导检测、脑脊液检查等辅助检查证据支持。
title: 临床亚型.
content: 依据肌无力位置、肌电图结果、抗神经节苷脂抗体检测结果等,格林-巴利综合征可分为若干不同的亚型,每种亚型都有各自不同的特点。
title: 治疗.
content: 出现严重无力症状的患者,应当接受静注免疫球蛋白或血浆置换治疗以封闭自体免疫抗体。同时还应当接受机械通气等对症支持治疗。虽然病情迅速进展且凶险,不接受正规治疗死亡率高,但经过正规治疗的患者预后往往较好,可在数周至数年内康复,但大约三分之一的患者可能会留有永久性的无力症状。
title: 流行病学.
content: 全球范围内,本病患者的死亡率大约为7.5%,而发病率则为每年十万分之一到二之间,男女之间、各国各地区之间的发病率没有明显差异。本病由法国神经病学家Georges Guillain、Jean Alexandre Barré、André Strohl在1916年首次描述,并依据前两位的姓命名为格林-巴利综合征。

Summary: 格林-巴利综合征(英语:Guillain-Barré syndrome,缩写为英语:GBS),又称基连巴瑞症候群、脱髓鞘多发性神经炎、吉兰-巴雷综合征、古兰-巴雷综合征。是一种因免疫系统损害周围神经系统,而导致的急性肌肉瘫痪疾病。本病的典型初始症状为痛觉异常及肌肉弱化。一般肌肉症状会先从手脚开始进犯,之后进犯上臂及上半身。本病症状会持续数小时至数周,在急性期间,15%的患者会进犯呼吸肌,可能危及生命,需要使用机械式呼吸辅助,有些则会影响自律神经系统,导致心率及血压异常。 此疾病的致病原因尚未明朗,病理的机制和自体免疫性疾病有关,身体内的免疫系统攻击周边神经,因此破坏了髓磷脂的绝缘。有时这种免疫失调会因为感染所引发,偶尔也会因为手术或疫苗接种所引发。此疾病一般会依症状及体征诊断,不过需排除其他可能的病因,也会透过神经传导研究测试以及检查脑脊液来验证。疾病可依照虚弱的部位、神经传导测试的结果、以及是否有对抗糖脂质抗体可以再分为几个子类。格林-巴利综合征属于急性的多发性神经病。 对于肌肉极度无力的病患,立即静脉注射免疫球蛋白、进行血浆置换术,同时施以支持疗法可有效使其中大部分恢复,患者的恢复期需数周到几年之久,约三分之一的患者会留有终生的肌肉无力的后遗症。格林-巴利综合征是一种罕见疾病,发生率约为每年十万分之二,其全球死亡率约为7.5%。性别与地域与其发生率不存在明显关联格林-巴利综合征以法国神经病学医师Georges Guillain(Georges Guillain)与Jean Alexandre Barré(Jean Alexandre Barré)的姓氏命名,两位医师与André Strohl(André Strohl)医师在1916年共同描述该疾病。

Guillain–Barré syndrome: Guillain–Barré syndrome (GBS) is a rapid-onset muscle weakness caused by the immune system damaging the peripheral nervous system. The initial symptoms are typically changes in sensation or pain along with muscle weakness, beginning in the feet and hands, often spreading to the arms and upper body, with both sides being involved. The symptoms may develop over hours to a few weeks. During the acute phase, the disorder can be life-threatening, with about 15 percent of people developing weakness of the breathing muscles and, therefore, requiring mechanical ventilation. Some are affected by changes in the function of the autonomic nervous system, which can lead to dangerous abnormalities in heart rate and blood pressure. Although the cause is unknown, the underlying mechanism involves an autoimmune disorder in which the body's immune system mistakenly attacks the peripheral nerves and damages their myelin insulation. Sometimes this immune dysfunction is triggered by an infection or, less commonly by surgery and rarely by vaccination. The diagnosis is usually made based on the signs and symptoms, through the exclusion of alternative causes, and supported by tests such as nerve conduction studies and examination of the cerebrospinal fluid. There are a number of subtypes based on the areas of weakness, results of nerve conduction studies and the presence of certain antibodies. It is classified as an acute polyneuropathy. In those with severe weakness, prompt treatment with intravenous immunoglobulins or plasmapheresis, together with supportive care, will lead to good recovery in the majority of people. Recovery may take weeks to years, with about a third having some permanent weakness. Globally, death occurs in about 7.5% of those affected. Guillain–Barré syndrome is rare, at one or two cases per 100,000 people every year. Both sexes and all parts of the world have similar rates of disease. The syndrome is named after the French neurologists Georges Guillain and Jean Alexandre Barré, who, together with French physician André Strohl, described the condition in 1916.