多发性神经病PPT课件

急性炎症性脱髓鞘性多发性神经病Acute Inflammatory Demyelinating Polyneuropathy, AIDP,1,Introduction,Landry -Landrys paralysis 1859Landry reported an acute, ascending, predominantly motor paralysis with respiratory failure, leading to deathGuillair-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,2,Guillain,Barre,Landry,Strohl,3,Introduction,In 1956, C Miller Fisher described a triad of acute ophthalmoplegia, ataxia, and areflexia, now known as Fishers syndromeDuring the past 15 years, GBS has become clear that this clinical picture, now called Guillain-Barr syndrome, and have different pathological subtypes,4,Epidemiology,Worldwide incidence0.6 -4/100 000 per year throughout the worldChina incidence0.66 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,5,6,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1726。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。,7,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。,8,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,9,Etiology,Campylobacter JejuniEpstein-Barr Virus (EBV)Cytomegalovirus (CMV)HIVVaccinations,空肠肠弯曲菌,10,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,11,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,12,13,14,15,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,16,Clinical manifestations,Progressive ascending symmetrical weakness of the limbsInvolvement of proximal and distal musclesNumbness and tingling in the hands and feetBack pain,17,Clinical manifestations,Depressed or absent reflexesInvolvement of cranial nerves (facial nerves most commonly involved)Respiratory failure(involved respiratory muscles)Progression to peak disability in 4 wkautonomic nerve symptom,18,Assessment,Cerebrospinal fluidIncreased 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,19,Assessment,Nerve conduction velocity test Nerve conduction studies are a dependable and early diagnostic indicator of GBS. shows demyelization and damage to the nerve sheathF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,20,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,21,Subtypes of GBS,经典型 AIDPFisher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,22,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,23,Diagnosis,Required for diagnosisProgressive weakness of one or more limbDistal areflexia with proximal areflexia or hyporeflexia,24,Diagnosis,Supportive diagnosisProgression of symptoms over days to 4 wkRelative symmetry of deficits Mild sensory involvementCranial nerve involvement (especially VII)Recovery beginning within 4 wk,25,Diagnosis,Supportive diagnosisAutonomic dysfunctionNo fever Increased CSF protein after 1 wkCSF white blood cell count 10/LNerve conduction slowing or blocked by several weeks,26,Diagnosis,Against diagnosisSignificant asymmetric weaknessBowel or bladder dysfunction at onset or persistentCSF white blood cell count 50 or PMN count 0LWell-demarcated sensory level,27,Diagnosis,Excluding diagnosisIsolated sensory involvement, without weaknessAnother polyneuropathy that explains clinical picture,28,Differential diagnosis,Acquired hypokalemiaBotulismMyasthenia gravisPeriodic paralysisPoliomyelitis,PolymyositisTick paralysisDiphtheriaTransverse myelitisHeavy metal (lead and arsenic poisoning),29,Differential diagnosis,低钾性周期性瘫痪(hypokalemic periodic paralysis)无病前感染史，常有发作史无感觉和脑神经损害，脑脊液正常电解质（血钾3.5)及心电图检查异常补钾治疗有效,30,Differential diagnosis,重症肌无力(myasthenia gravis)骨骼肌 病态易疲劳性、波动性no sensory symptoms tendon reflexes are unimpaired,31,Differential diagnosis,脊髓灰质炎(poliomyelitis)早期出现括约肌功能障碍无感觉障碍Fever, meningeal symptoms, early pleocytosis, and purely motor and usually asymmetrical areflexic paralysis.,32,Differential diagnosis,急性脊髓炎（acute myelitis）The immediate problem is to differentiate GBS from acute spinal cord disease (marked by sensorimotor paralysis below a level on the trunk and sphincteric paralysis).,33,Clinical management,General treatment 一般治疗Immunotherapy 免疫治疗,34,General treatment,保持呼吸道通畅辅助呼吸密切观察，测肺活量20ml/kgICU必要时气管插管，使用呼吸器预防呼吸道感染翻身、拍背、稀化痰液、吸痰,35,36,General treatment,预防并发症(prevention of complication)坠积性肺炎褥疮血栓性静脉炎防止肢体挛缩尿路感染,37,General treatment,预防并发症(prevention of complication)合理的正压通气、吸出分泌物经常翻身，保持床单平整皮下应用肝素有临床指征时，应用广谱抗生素等,38,General treatment,对症处理必要时心电监护高血压小剂量受体阻滞剂低血压补液心动过速通常不需要治疗心动过缓阿托品疼痛卡马西平,39,Immunotherapy,机理抑制免疫反应，去除致病因子对神经损害，使髓鞘有时间再生方法血浆置换静脉注射免疫球蛋白皮质醇激素治疗,40,Plasma exchange,The usefulness of plasma exchange in the evolving phase of GBS.In patients who are treated within 2 weeks of onset, there is a reduction in the period of hospitalization in the length of time that the patient requires mechanical ventilation. However, when plasma exchange is delayed for 2 weeks or longer after the onset of the disease, the procedure has, with a few notable exceptions, been of little value.,41,Plasma exchange,血浆置换机制：去除血浆中致病因子，可明显缩短病程，使用越早，疗效越好，专用设备，价格昂贵适用于急性进行性加重的GBS用法：40ml/kg禁忌症：严重感染， 心律失常、心功能不全， 凝血功能障碍,42,Intravenous immunoglobulin,静脉注射免疫球蛋白尽早施行用法：0.4g/(kg.d)5天禁忌症：免疫球蛋白过敏，先天性IgA缺乏 PE 和IVIG不必联合应用,43,Corticosteroids,皮质类固醇 有争议理论上合理研究表明无效经验：青年人大剂量早期使用,44,Corticosteroids,The value of corticosteroids in the treatment of GBS has been disputed for decades.Although corticosteroids can no longer recommended as routine treatment for acute GBS.We have observed a few instances in which the intravenous administration with high-close corticosteroids seemingly halted the progress of the disease.,45,Prognosis,PrognosisThe majority of patients recover completely or nearly completely In about 10 percent of patients, the residual disability is pronounced,46,47,预后,80%患者恢复完全死亡率大约5%(呼吸肌麻痹),48,谢谢!,49,