伴严重轴索损害的咽-颈-臂变异型吉兰-巴雷综合征临床特点

The clinical,electrophysiological study of pharyngeal-cervical-brachia variation type GBS with severe anxoal damage

目的探讨咽-颈-臂(PCB)变异型吉兰-巴雷综合征(GBS)的临床特点。方法报道2014年本院收入1例PCB变异型GBS的临床表现和辅助检查结果、临床治疗效果。结果该患者临床表现为咽部-颈部-上臂肌肉无力,电生理提示运动轴索型周围神经损害。腰穿提示明显蛋白细胞分离。给予甲强龙冲击治疗后患者症状明显好转。结论 PCB变异型GBS主要表现为咽-颈-臂肌无力,而下肢通常不受累,本例患者另外突出的临床表现是发病初即出现的头部疼痛,1月后发现明显胸锁乳突肌、胸大肌、斜方肌萎缩。查体可见眼震。PCB变异型GBS为免疫介导炎性谱系疾病,临床症状可以与Bickerstaff脑干脑炎有部分交叉。

Objective To investigate the clinical features of pharyngeal-cervical-brachia （PCB） variation type Guillain-Barres syndrome （GBS）. Methods A case of PCB variant GBS was reported in our hospital in 2014, and the case characteristic, clinical treatment effect were reviewed. Results The clinical manifestations of the patients were the weakness of the pharynx and neck and the upper arm muscle, and the electrical physiology indicated that the peripheral nerve anxoal injury. Lumbar puncture result was marked protein cell separation. The symptoms of patients treated with methylprednisolone were significantly improved. Conclusion PCB -the variants of GBS were mainly for the pharynx, neck and arm muscle weakness, and lower limb usually not involved, the patient was the early onset of the pain in the head, after 1 month he was found obvious muscle atrophy of sternocleidomastoid, pectoralis major, trapezius. The physical signs were nystagmus. The PCB variant GBS was immune mediated inflammatory disease, the clinical symptoms could be partially crossed with Bickerstaff encephalitis.