A 38-year-old man with hypertension is evaluated in the emergency department for a five-day history of progressive(逐步的) weakness. He first noticed low back pain about a week ago and then weakness in both legs. Yesterday, he noted weakness in his arms. On examination, he has difficulty speaking more than a few words without becoming short of breath. Mental status and cranial nerves are intact. He has 2/5 strength in both lower extremities(末端) and 3/5 strength in both upper extremities. He notes paresthesia(感觉异常) in his hands and feet, but sensory examination is unremarkable. Reflexes are absent. Pulmonary function tests reveal a forced vital capacity of 800 mL and negative inspiratory force of -20 cm H2O. He is intubated. Results from a lumbar function are pending.
Which of the following treatments is most appropriate at this time?
A. Plasma exchange only
B. Plasma exchange in combination with IV immunoglobulin
C. IV methylprednisolone(甲强龙)
D. IV rituximab
该患者患有吉兰-巴雷综合征(GBS)。腰椎穿刺通常显示脑脊液蛋白升高,细胞计数低于10/mm3(白蛋白细胞分离)。最近的美国神经病学学会指南建议使用血浆置换或IV免疫球蛋白(IV Ig)治疗以加速恢复。这两种治疗方法对晚期GBS症状患者同样有效。血浆置换需要放置透析导管,存在与操作相关的固有风险。IV Ig可能与过敏反应、无菌性脑膜炎、体液过多和急性肾衰竭相关。不建议联合两种治疗,类固醇治疗无益。利妥昔单抗在治疗GBS中无作用。
A
补充内容(来源于uptodate):
吉兰-巴雷综合征(GBS)是一种急性、单相病程的疾患,可引起快速进展性多发性神经病,表现为肌无力或瘫痪。
诊断流程如下:
原创文章(本站视频密码:66668888),作者:xujunzju,如若转载,请注明出处:https://zyicu.cn/?p=9033