Guillain-Barre综合征合并中枢神经系统脱髓鞘病变的临床研究

目的 探讨Guillain-Barre综合征（GBS）合并中枢神经系统（CNS）脱髓鞘病变及两者之间的发病关系和可能的发病机制。方法 对3例合并CNS脱髓鞘病变的GBS患者的临床及实验室资料进行分析。结果 例1男，28岁，主要表现为全身软瘫和昏迷，血气分析结果正常，脑脊液有蛋白细胞分离，寡克隆带阳性，肌电图提示神经源性损害；MRI示双侧脑白质及颈髓广泛多发性脱髓鞘改变；应用血浆和免疫球蛋白治疗后好转，例2女，5岁，因进行性四肢无力，呼吸困难入院，应用呼吸机和血浆治疗过程中出现脑干反射障碍，后放弃治疗死亡，死后行腓肠神经病检示周围神经脱髓鞘病变，例3男，12岁，因进行性四肢无力伴大小便困难入院；颅脑MRI未见异常，脊髓MRI见T5-T4节段广泛脱髓鞘病变；脑脊液有蛋白细胞分离。寡克隆带阳性；肌电图示周围神经传导速度减慢。结论 合并意识障碍的GBS大多病情危重，其发病机制不详，可能与P1碱蛋白引起的自身免疫反应造成周围和GNS的共同脱髓鞘有关。

Clinical study of Guillain-Barre syndrome combined with demyelinating lesion in central nervous system

Objective To investigate Guillain Barre syndrome (GBS) combined with demyelinating neuropathy in central nervous system(CNS), and explore the possible mechanism and the relationship between the two.Methods 3 cases GBS combined with demyelinating neuropathy in CNS were observed clinically and the datum of laboratory were analysed.Results Case 1, a 28 year old man had symptoms of general flaccid paralysis and coma.The result of blood gas analysis was normal. CSF showed an albuminocytological dissociation, delayed nerve conduction velocity and missed F waves. Brain MRI showed multifocal T 2 Wight Image high signs in white matter of bilateral brain and cervical spinal cord. The patient is getting recovery by treatment with plasma and immunoglobulin. Case 2 , a 5 year old girl with progressive weakness of her limbs and respiratory arrest, appeared confusion,dully light reflex and absent corneal reflex, at last she died because of rejecting treatment.Case 3,a 12 year old boy with progressive weakness of his limbs and the difficult of relieving the bowels.Brain MRI was normal.Spinal MRI showed multifocal T 2 weight imagine hight signs from T 5 to L 4.CSF showed an albuminocytological dissociation.EMG showed a delayed nerve conduction velocity.Conclusion GBS combined with disorders of consciousness are mostly severe, the pathological mechanism is unclear. It is suggested that auto immunoreaction caused by P 1 myelin basic protein can relate to around and CNS demyelination.