慢性炎癥性脫髓鞘性多發(fā)性神經(jīng)根神經(jīng)病進展

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1、Click to edit Master title style,Click to edit Master text styles,Second level,Third level,Fourth level,Fifth level,11/7/2009,#,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,2017/1/6,#,Chronic Inflammatory Demyelinatin Polyradiculoneuropathy:update on clinical features,phenotypes and treatment options,D

2、epartment of Neurology,Fujian Provincial Hospital,Xingyong Chen,第一頁，共二十一頁。,Introduction,Traditionally,chronic inflammatory demyelinating polyradiculoneuropathy(CIDP)has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes.,The European Federation of Neurological

3、 Societies/Peripheral Nerve Society(EFNS/PNS)CIDP treatment guideline has defined several clinical pictures as atypical CIDP phenotypes,additional to the classical(typical)picture of CIDP,第二頁，共二十一頁。,prevalence of around 6 cases per 100,000,predominantly affect males,and typically occur in middle to

4、old age,CIDP can also occurin children.,The incidence of CIDP increases with age,rising to 1.5 times the overall average in people over 65years of age,第三頁，共二十一頁。,Clinical presentation,Classic CIDP is characterized by the occurrence of symmetrical weakness in both proximal and distal muscles,impaired

5、 sensation and parasthesia and absent or diminished tendon reflexes,The disease evolves over more than 8 weeks,thus distinguishing the condition from GBS which has an acute onset.,The time course maybe relapsing,chronic progressive,monophasic or GBS like onset.,第四頁，共二十一頁。,Newly recognised clinical f

6、eatures in CIDP,Acute onset,of CIDP may occur in up to 18%of CIDP patients,resembling the GBS,diagnosis was,changed to CIDP,in,5%of GBS,patients,Fatigue,can be the main complaint in CIDP patients(75%),Activity-induced weakness,Severe pain,is present in only a minority of CIDP patients;,Tremor,can be

7、 a disabling symptom in CIDP(50%),a higher incidence of almost 40%of,restless legs syndrome,in patients with CIDP,Autonomic symptoms:,23%,of which,gastrointestinal and genitourinary symptoms,were most frequent,(mild),severe autonomic dysfunction should be regarded as a red flag,when considering the

8、diagnosis of CIDP.,第五頁，共二十一頁。,CIDP PHENOTYPES,The clinical presentation of CIDP is variable,determined by the number and distribution of the demyelinating peripheral nerve lesions,第六頁，共二十一頁。,SM:sensory and motor.PE:plasma exchange;RR:relapsingremitting;CS:corticosteroids;,第七頁，共二十一頁。,Atypical CIDP,第八

9、頁，共二十一頁。,Distal paresthesia and hypesthesia are the most frequent symptoms,followed by proprioceptive ataxia,第九頁，共二十一頁。,DADSP,(distal acquired demyelinating symmetric polyneuropathy):distal or predominantly sensory large fibre neuropathy,length dependent,axonal neuropathy.,DML:distal motor latency,第

10、十頁，共二十一頁。,TheLewisSumnersyndrome(LSS),ormultifocalacquireddemyelinatingsensoryandmotorneuropathy(MADSAM),hasbeenreportedinupto15%ofpatientsfulfillingtheEFNS/PNScriteriaforCIDP,第十,一,一頁,，,，共,二,二十,一,一頁,。,。,Thecombinationofsymmetricpatternofweaknesswithoutbulbarinvolvement,clinicallydistinguishesthisphe

11、notypefromlowermotorneurondisease,butdistinctioncanbedifficult,especiallyifelectrophysiologicalcriteriaofdemyelinationarenotmetcompletely.,第十,二,二頁,，,，共,二,二十,一,一頁,。,。,FocalCIDPisdefinedasinvolvementofthebrachial,orlumbosacralplexusorofoneormoreperipheral,nerves in one upperor lowerlimb(EFNS).,第十三頁,，,

12、，共二十,一,一頁。,Associated conditions,CIDPmaybeassociated withvariousdiseases,such as infectionwiththehuman immunodeficiencyvirus orhepatitis C,Sj,grenssyndrome,inflammatory boweldisease,melanoma,lymphoma,diabetes mellitus,andIgM,IgG,or IgA monoclonalgammopathyof unknown significance.,Thepathogeneticrele

13、vance of such concurrent diseases is unclear,第十四頁,，,，共二十,一,一頁。,update,Anti-neurofascin(,神經(jīng),(shnjng),束蛋白,)IgG4 antibodies were associated with asubgroup ofpatients with CIDPshowingayoungerageat onset,ataxia,tremor,CNSdemyelination,anda poor responseto IV immunoglobulin,第十五頁,，,，共二十,一,一頁。,Diffusion-wei

14、ghted images inpatient10showed signal abnormalitiesin thespleniumofthecorpus callosum(,胼胝,(pinzh),體壓部,).,Fluid-attenuatedinversion recoveryimages in patients 10 and 31showedmultiplesclerosislikelesionsin thejuxtaventricular,（腦室旁,）,）,regions.,第十六頁,，,，共二十,一,一頁。,TherapeuticoptionsforCIDP,第十七頁,，,，共二十,一,

15、一頁。,Theacquiredchronicdemyelinating neuropathiesinclude,：,chronicinflammatorydemyelinating polyneuropathy(CIDP),neuropathy associated with monoclonal IgM antibodiestomyelin-associated glycoprotein(MAG;anti-MAGneuropathy),multifocal motorneuropathy(MMN),POEMSsyndrome.,They havecharacteristic-though o

16、verlapping-clinical presentations,aremediatedbydistinctimmunemechanisms,and respondtodifferent therapies.,第十八,頁,頁，共,二,二十一,頁,頁。,Figure1|Asuggested diagnosticpathwayforchronicacquireddemyelinating polyneuropathies.Differentialdiagnosisisbasedonelectrodiagnosticstudies,biopsies,serum biomarkersand antibodies.Abbreviations:CIDP,chronicinflammatory demyelinatingpolyneuropathy;IFE,immunofixationelectrophoresis;MAG,myelin-associated glycoprotein;VEGF,vascularendothelialgrowth factor.,第十九,頁,頁，共,二,二十一,頁,