急性炎性脱髓鞘性多发性神经根神经病的各种类型一法道自然战友.DOCVIP

急性炎性脱髓鞘性多发性神经根神经病的各种类型(一)病例1，请大家讨论临床类型，发病机制傅××，男性，72岁，2005年10月13日早晨感双手麻木，上午工作期间感张口困难，言语不清，吞咽困难。急入某院急诊，病情进行性迅速发展，就诊时出现四肢活动无力，不能言语、张口，吞咽困难，行头CT未见明显异常，查体：意识清楚，双侧面瘫，构音障碍，软腭不能上抬，不能转头，仅眼球能够活动。四肢软瘫，病理征阴性。考虑为“脑干梗死，闭锁综合症”，当晚即因呼吸受累及行机械通气。住院期间：10月13-25日发热（体温38～ 41），血压80 ～120/ 40 ～70mmHg，心率100 ～130次/分，多汗。病程第2月转另一家医院。病程第5月，患者逐渐能够闭眼，张口，伸舌，发声；四肢近端肌力恢复至4级，远端肌力仍为0级，肌萎缩，腱反射消失，病理征阴性。手袜套样针刺觉减退；皮肤干燥，皮肤营养差；窦房结功能不全：窦性停搏，逸搏心律，房室传导阻滞（1-2度）；腹胀，排便困难，听诊为气过水声，X线表现为肠管扩张积气，结肠镜检：结肠脾曲部以上干结阻塞大便。呼吸支持模式为，自主呼吸次数为8次/分钟。2006年3月：头MRI脑干未见缺血病变。腰穿检查：WBC 2/ul，蛋白0.13g/L，OB阳性，CSF IgG鞘内合成率57（＜ 7），MBP升高。 L格林巴利综合症分型1经典格林巴利综合症?? 即aidp2急性运动轴索性神经病??即aman??为纯运动性gbs3急性运动感觉轴索性神经病?? 即amsan??病情严重??预后差4miller-fisher综合症?? 表现为眼外肌麻痹??共济失调??和腱反射消失5不能分类的gbs ??包括纯感觉性gbs???? 全自主神经功能不全性gbs???? 多数脑神经性gbs病例一??老年男性起病较急?病变累计运动神经?感觉神经?颅神经自主神经系统考虑还是经典gbs 即aidp吧A7662888战友： In case 1, this patient showed sensory symptoms of both hands first. Rapidly progressive clinical course, starting from bulabr and facial paralysis, followed by neck and four limbs weakness, was noted. Finally, the patient developed respiratory failure. The significant NEGATIVE finding was oculomotor system sparing. There was prominent autonomic system involvement, including sweating, hypotension, spiking fever, cardiac conduction block and GI dysfunction. This patient had poor recovery within a half year. The CSF study showed cyto-albuminal dissociation, which was compatible with inflammatory process in nature. I agreed the illness was PNS in nature due to negative MRI findings. Cervical myelopathy could be excluded due to bifacial and bulbar weakness. As I lnow, this patient did NOT show a clinical course of typical AIDP, because he had fulminant DESCENDING paralysis with very poor prognosis. MFS could be excluded due to oculomotor sparing. I would like to know more about the past history of the patient, including toxin, travel, or medication history.  Besides GBS, other possibilities should be in the differential diagnosi