Expósito Jesica, Carrera Laura, Natera Daniel, Nolasco Gregorio, Nascimiento Andrés, Ortez Carlos
Unidad de Patología Neuromuscular, Hospital Sant Joan de Déu, Barcelona, España.
Unidad de Patología Neuromuscular, Hospital Sant Joan de Déu, Barcelona, España. E-mail:
Medicina (B Aires). 2022 Aug 30;82 Suppl 3:82-88.
Guillain-Barré syndrome (GBS) is characterized by rapidly progressive and generally ascending symmetrical muscle weakness, accompanied by decreased or absent osteotendinous reflexes. The inflammatory process may affect the myelin or the axon. There are 4 clinical forms of GBS: 1) acute inflammatory demyelinating polyradiculoneuropathy, 2) acute motor axonal neuropathy, 3) acute sensory and motor axonal neuropathy, and 4) the Miller-Fisher variant, which is characterized by ophthalmoplegia, ataxia and areflexia, with little muscle weakness. Diagnosis is based on the albumin-cytological dissociation observed at the end of the first week after the onset of symptoms and may persist until the third week, as well as on the specific neurophysiological alterations of each clinical form. The treatment of GBS will depend on the degree of severity, if the patient presents grade IV or less according to the Paradiso scale, it will be treated with Ig IV, if it presents grade V, the use of plasmapheresis and/or immunoadbosorption is recommended. In severe axonal cases, the use of corticosteroid bolus is recommended in initial stages. There is a clinical picture that overlaps GBS and chronic demyelinating polyneuropathy related to antibodies against neurophysin and contactin, in this case the appropriate therapy is rituximab.
吉兰-巴雷综合征（GBS）的特征是快速进展且通常呈上行性的对称性肌无力，并伴有腱反射减弱或消失。炎症过程可能影响髓鞘或轴突。GBS有4种临床类型：1）急性炎症性脱髓鞘性多发性神经根神经病，2）急性运动轴索性神经病，3）急性感觉和运动轴索性神经病，4）米勒-费希尔变异型，其特征为眼肌麻痹、共济失调和无反射，肌无力较轻。诊断基于症状发作后第一周结束时观察到的蛋白-细胞分离，这种分离可能持续至第三周，以及基于每种临床类型的特定神经生理学改变。GBS的治疗将取决于严重程度，如果患者根据Paradiso量表表现为IV级或以下，将采用静脉注射免疫球蛋白治疗，如果表现为V级，建议使用血浆置换和/或免疫吸附。在严重轴索性病例中，建议在初始阶段使用大剂量皮质类固醇。有一种临床表现与抗神经垂体素和接触蛋白抗体相关的GBS和慢性脱髓鞘性多发性神经病重叠，在这种情况下，合适的治疗方法是利妥昔单抗。
