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1、單擊此處編輯母版標(biāo)題樣式,單擊此處編輯母版文本樣式,第二級,第三級,第四級,第五級,*,單擊此處編輯母版標(biāo)題樣式,單擊此處編輯母版文本樣式,第二級,第三級,第四級,第五級,*,*,急性炎癥性脫髓鞘性多發(fā)性神經(jīng)病,Acute Inflammatory Demyelinating Polyneuropathy,AIDP,浙江大學(xué)醫(yī)學(xué)院附屬第一醫(yī)院,Introduction,Landry,-,Landrys paralysis 1859,Landry reported,an acute,ascending,predominantly motor paralysis with respiratory
2、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),蛋白細(xì)胞分離,是本病的特征,Guillain,Barre,Landry,Strohl,Introduction,In 1956,C Miller Fisher
3、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 th
4、roughout the world,China incidence,0.66 per 100 000 for all ages,可發(fā)生于任何年齡，男女發(fā)病率相似，夏秋多見,臨床表現(xiàn)：中國,兒童和青少年，夏初。,EMG,：軸索損害，,AMAN,。,EMG,符合,AMAN,的為,65,，符合,AIDP,的為24。,66有,CJ,抗體，,42,有,GM1,抗體，其他神經(jīng)節(jié)苷脂抗體為,17,26,。與西方國家不同，,GM1,抗體與,AMAN,或,AIDP,無關(guān)。近來發(fā)現(xiàn),AMAN,與,GD1a,抗體相關(guān)密切。,臨床表現(xiàn)：中國,病理：,AMAN,：,IgG,和補(bǔ)體在軸索周圍沉積，巨噬細(xì)胞侵入軸索周圍間
5、隙，嚴(yán)重者有軸索變性。,AIDP,：,IgG,和補(bǔ)體在髓鞘外沉積，巨噬細(xì)胞也在髓鞘外，“撕開”髓鞘。,AMSAN,：感覺軸索比運(yùn)動(dòng)軸索損害重。,EMG,不能預(yù)測病理。,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.,病因尚未完全闡明,Etiology,C,ampylobacter,J,ejuni,Epstein-Barr
6、Virus,(EBV),Cytomegalovirus,(CMV),HIV,Vaccinations,空腸腸彎曲菌,Pathogenesis and Pathophysiology,An acute immune-mediated polyneuropathy,component of pathogen was similar with myelin sheath of peripheral nerve,與感染有關(guān)的自身免疫性疾病,病原體某些成分與周圍神經(jīng)的髓鞘成分相似,Pathophysiology,主要病理特點(diǎn),(principal characteristic of pathology)
7、,節(jié)段性脫髓鞘,(segmental demyelization),小血管周圍炎性細(xì)胞浸潤,Clinical manifestations,多數(shù)患者有前驅(qū)癥狀,(,起病前,13,周）,呼吸道感染癥狀,喉痛、鼻塞、發(fā)熱,消化道癥狀,腹瀉、嘔吐,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,Cl
8、inical 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
9、10 days.,While some protein is normally present,an increased amount without an increase in the number of white blood cells may indicate GBS,蛋白細(xì)胞分離,Assessment,Nerve conduction velocity test,Nerve conduction studies are a dependable and early diagnostic indicator of GBS.,shows demyelization and damage
10、 to the nerve sheath,F,反應(yīng)、,H,反射異常,PL,延長，,NCV,減慢,傳導(dǎo)阻滯現(xiàn)象，伴或不伴有波幅降低,Assessment,腓腸神經(jīng)活檢,節(jié)段性脫髓鞘,小血管周圍炎性細(xì)胞浸潤,Electrocardiogram(EKG),May show abnormalities in cardiac rhythm,心律失常,Subtypes of GBS,經(jīng)典型,AIDP,Fisher,綜合癥,(Miller Fisher syndrome),：,三聯(lián)征,-,“,眼外肌麻痹,、,共濟(jì)失調(diào)、腱反射消失,”，,還有中樞神經(jīng)系統(tǒng)損害,It was thought to be a va
11、riant of GBS and comprise complete ophthalmoplegia with ataxia and are flexia,腦神經(jīng)型,Subtypes of GBS,軸突型,純運(yùn)動(dòng)型（,AMAN,）,運(yùn)動(dòng) 感覺 型（,AMSAN,）,急性感覺性多發(fā)性神經(jīng)炎（,ASP,）,急性全自主神經(jīng)?。?APN,）,假性肌營養(yǎng)不良,復(fù)發(fā)型,Diagnosis,Required for diagnosis,Progressive weakness of one or more limb,Distal areflexia with proximal areflexia or hy
12、poreflexia,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 afte
13、r 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
14、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 arseni
15、c poisoning),Differential diagnosis,低鉀性周期性癱瘓,(h,ypokalemic periodic paralysis),無病前感染史，常有發(fā)作史,無感覺和腦神經(jīng)損害，腦脊液正常,電解質(zhì)（血鉀,3.5),及心電圖檢查異常,補(bǔ)鉀治療有效,Differential diagnosis,重癥肌無力,(,myasthenia gravis),骨骼肌,病態(tài)易疲勞性、波動(dòng)性,no sensory symptoms,tendon reflexes are unimpaired,Differential diagnosis,脊髓灰質(zhì)炎,(poliomyelitis),早期出
16、現(xiàn)括約肌功能障礙,無感覺障礙,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,一般治療,Immunotherapy,免疫治療,General treatment,保持呼吸道通暢,輔助呼吸,密切觀察，測肺活量,20ml/kgICU,必要時(shí)氣管插管，使用呼吸器,預(yù)防呼吸道感染,翻身、拍背、稀化痰液、吸痰,General treatmen
急性炎癥性脫髓鞘性-多發(fā)性神經(jīng)病課件
