急性炎症性脱髓鞘性-多发性神经病.ppt

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,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,Guillain,Barre,Landry,Strohl,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,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,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,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。,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,Etiology,C,ampylobacter,J,ejuni,Epstein-Barr Virus,(EBV),Cytomegalovirus,(CMV),HIV,Vaccinations,空肠肠弯曲菌,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,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,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,Progressive ascending symmetrical weakness of the limbs,Involvement of proximal and distal muscles,Numbness and tingling in the hands and feet,Back pain,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,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,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,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,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,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,Diagnosis,Required for diagnosis,Progressive weakness of one or more limb,Distal areflexia with proximal areflexia or hyporeflexia,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,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,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,Diagnosis,Excluding diagnosis,Isolated sensory involvement,without weakness,Another polyneuropathy that explains clinical picture,Differential diagnosis,Acquired hypokalemia,Botulism,Myasthenia gravis,Periodic paralysis,Poliomyelitis,Polymyositis,Tick paralysis,Diphtheria,Transverse myelitis,Heavy metal(lead and arsenic poisoning),Differential diagnosis,低钾性周期性瘫痪h,ypokalemic 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,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,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.,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).,Clinical management,General treatment,一般治疗,Immunotherapy,免疫治疗,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,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,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,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,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,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.,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,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,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,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.,Prognosis,Prognosis,The majority of patients recover completely or nearly completely,In about 10 percent of patients,the residual disability is pronounced,预后,80%,患者恢复完全,死亡率大约,5%(,呼吸肌麻痹,),谢谢,!,