急性炎症性脱髓鞘性-多发性神经病课件

单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,潍医附院 放疗科,*,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,*,急性炎症性脱髓鞘性多发性神经病,Acute Inflammatory Demyelinating Polyneuropathy,AIDP,浙江大学医学院附属第一医院,急性炎症性脱髓鞘性多发性神经病Acute Inflamma,Introduction,Landry,-,Landrys paralysis 1859,Landry reported,an acute,ascending,predominantly motor paralysis with respiratory failure,leading to death,Guillair-Barre 1916 2,例,Guillain,Barre and,strohl,(1916)reported a benign polyneuritis with,albuminocytologic dissociation in the CSF(raised concentration of CSF protein but a normal cell count),蛋白细胞分离,是本病的特征,IntroductionLandry-Landrys,Guillain,Barre,Landry,Strohl,GuillainBarre LandryStrohl,Introduction,In 1956,C Miller Fisher described a triad of acute ophthalmoplegia,ataxia,and areflexia,now known as Fishers syndrome,During the past 15 years,GBS has become clear that this clinical picture,now called Guillain-Barr syndrome,and have different pathological subtypes,IntroductionIn 1956,C Miller,Epidemiology,Worldwide incidence,0.6-4/100 000 per year throughout the world,China incidence,0.66 per 100 000 for all ages,可发生于任何年龄，男女发病率相似，夏秋多见,EpidemiologyWorldwide incidenc,急性炎症性脱髓鞘性-多发性神经病课件,临床表现：中国,儿童和青少年，夏初。,EMG,：轴索损害，,AMAN,。,EMG,符合,AMAN,的为,65,，符合,AIDP,的为24。,66有,CJ,抗体，,42,有,GM1,抗体，其他神经节苷脂抗体为,17,26,。与西方国家不同，,GM1,抗体与,AMAN,或,AIDP,无关。近来发现,AMAN,与,GD1a,抗体相关密切。,临床表现：中国儿童和青少年，夏初。,临床表现：中国,病理：,AMAN,：,IgG,和补体在轴索周围沉积，巨噬细胞侵入轴索周围间隙，严重者有轴索变性。,AIDP,：,IgG,和补体在髓鞘外沉积，巨噬细胞也在髓鞘外，“撕开”髓鞘。,AMSAN,：感觉轴索比运动轴索损害重。,EMG,不能预测病理。,临床表现：中国病理：,Pathogenesis and Pathophysiology,The cause of this syndrome is unknown,but it is generally viewed to be an autoimmune response to a bacterial or viral infection.,病因尚未完全阐明,Pathogenesis and Pathophysiolo,Etiology,C,ampylobacter,J,ejuni,Epstein-Barr Virus,(EBV),Cytomegalovirus,(CMV),HIV,Vaccinations,空肠肠弯曲菌,EtiologyCampylobacter Jejuni空肠,Pathogenesis and Pathophysiology,An acute immune-mediated polyneuropathy,component of pathogen was similar with myelin sheath of peripheral nerve,与感染有关的自身免疫性疾病,病原体某些成分与周围神经的髓鞘成分相似,Pathogenesis and Pathophysiolo,Pathophysiology,主要病理特点,(principal characteristic of pathology),节段性脱髓鞘,(segmental demyelization),小血管周围炎性细胞浸润,Pathophysiology主要病理特点(principa,急性炎症性脱髓鞘性-多发性神经病课件,急性炎症性脱髓鞘性-多发性神经病课件,急性炎症性脱髓鞘性-多发性神经病课件,Clinical manifestations,多数患者有前驱症状,(,起病前,13,周）,呼吸道感染症状,喉痛、鼻塞、发热,消化道症状,腹泻、呕吐,Clinical manifestations多数患者有前驱,Clinical manifestations,Progressive ascending symmetrical weakness of the limbs,Involvement of proximal and distal muscles,Numbness and tingling in the hands and feet,Back pain,Clinical manifestationsProgres,Clinical manifestations,Depressed or absent reflexes,Involvement of cranial nerves(facial nerves most commonly involved),Respiratory failure(involved respiratory muscles),Progression to peak disability in 4 wk,autonomic nerve symptom,Clinical manifestationsDepress,Assessment,Cerebrospinal fluid,Increased protein usually after 7 to 10 days.,While some protein is normally present,an increased amount without an increase in the number of white blood cells may indicate GBS,蛋白细胞分离,AssessmentCerebrospinal fluid,Assessment,Nerve conduction velocity test,Nerve conduction studies are a dependable and early diagnostic indicator of GBS.,shows demyelization and damage to the nerve sheath,F,反应、,H,反射异常,PL,延长，,NCV,减慢,传导阻滞现象，伴或不伴有波幅降低,AssessmentNerve conduction vel,Assessment,腓肠神经活检,节段性脱髓鞘,小血管周围炎性细胞浸润,Electrocardiogram(EKG),May show abnormalities in cardiac rhythm,心律失常,Assessment腓肠神经活检,Subtypes of GBS,经典型,AIDP,Fisher,综合症,(Miller Fisher syndrome),：,三联征,-,“,眼外肌麻痹,、,共济失调、腱反射消失,”，,还有中枢神经系统损害,It was thought to be a variant of GBS and comprise complete ophthalmoplegia with ataxia and are flexia,脑神经型,Subtypes of GBS 经典型 AIDP,Subtypes of GBS,轴突型,纯运动型（,AMAN,）,运动 感觉 型（,AMSAN,）,急性感觉性多发性神经炎（,ASP,）,急性全自主神经病（,APN,）,假性肌营养不良,复发型,Subtypes of GBS 轴突型,Diagnosis,Required for diagnosis,Progressive weakness of one or more limb,Distal areflexia with proximal areflexia or hyporeflexia,DiagnosisRequired for diagnosi,Diagnosis,Supportive diagnosis,Progression of symptoms over days to 4 wk,Relative symmetry of deficits,Mild sensory involvement,Cranial nerve involvement(especially VII),Recovery beginning within 4 wk,DiagnosisSupportive diagnosis,Diagnosis,Supportive diagnosis,Autonomic dysfunction,No fever,Increased CSF protein after 1 wk,CSF white blood cell count 10/L,Nerve conduction slowing or blocked by several weeks,DiagnosisSupportive diagnosis,Diagnosis,Against diagnosis,Significant asymmetric weakness,Bowel or bladder dysfunction at onset or persistent,CSF white blood cell count 50 or PMN count 0L,Well-demarcated sensory level,DiagnosisAgainst diagnosis,Diagnosis,Excluding diagnosis,Isolated sensory involvement,without weakness,Another polyneuropathy that explains clinical picture,DiagnosisExcluding diagnosis,Differential diagnosis,Acquired hypokalemia,Botulism,Myasthenia gravis,Periodic paralysis,Poliomyelitis,Polymyositis,Tick paralysis,Diphtheria,Transverse myelitis,Heavy metal(lead and arsenic poisoning