慢性炎症性脱髓鞘性多发性神经根神经病进展.pptx

Chronic Inflammatory Demyelinatin Polyradiculoneuropathy:update on clinical features,phenotypes and treatment optionsDepartment of Neurology,Fujian Provincial HospitalXingyong Chen IntroductionTraditionally,chronic inflammatory demyelinating polyradiculoneuropathy(CIDP)has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes.The European Federation of Neurological 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 CIDPprevalenceofaround6casesper100,000predominantlyaffectmales,andtypicallyoccurinmiddletooldageCIDPcanalsooccurinchildren.TheincidenceofCIDPincreaseswithage,risingto1.5timestheoverallaverageinpeopleover65yearsofageClinical presentationClassic CIDP is characterized by the occurrence of symmetrical weakness in both proximal and distal muscles,impaired sensation and parasthesia and absent or diminished tendon reflexesThe 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.Newly recognised clinical features in CIDPAcuteonsetofCIDPmayoccurinupto18%ofCIDPpatients,resemblingtheGBSdiagnosiswaschangedtoCIDPin5%ofGBSpatientsFatiguecanbethemaincomplaintinCIDPpatients(75%)Activity-inducedweaknessSeverepainispresentinonlyaminorityofCIDPpatients;TremorcanbeadisablingsymptominCIDP(50%)ahigherincidenceofalmost40%ofrestlesslegssyndromeinpatientswithCIDPAutonomicsymptoms:23%,ofwhichgastrointestinalandgenitourinarysymptomsweremostfrequent(mild)severeautonomicdysfunctionshouldberegardedasaredflagwhenconsideringthediagnosisofCIDP.CIDP PHENOTYPESThe clinical presentation of CIDP is variabledetermined by the number and distribution of the demyelinating peripheral nerve lesionsSM:sensory and motor.PE:plasma exchange;RR:relapsingremitting;CS:corticosteroids;Atypical CIDPDistal paresthesia and hypesthesia are the most frequent symptoms,followed by proprioceptive ataxiaDADSP(distal acquired demyelinating symmetric polyneuropathy):distal or predominantly sensory large fibre neuropathy length dependent axonal neuropathy.DML:distal motor latencyThe LewisSumner syndrome(LSS),or multifocal acquired demyelinating sensory and motor neuropathy(MADSAM),has been reported in up to 15%of patients fulfilling the EFNS/PNS criteria for CIDPThe combination of symmetric pattern of weakness without bulbar involvement clinically distinguishes this phenotype from lower motor neuron disease,but distinction can be difficult,especially if electrophysiological criteria of demyelination are not met completely.Focal CIDP is defined as involvement of the brachial or lumbosacral plexus or of one or more peripheral nerves in one upper or lower limb(EFNS).Associated conditionsCIDP may be associated with various diseases,such as infection with the human immunodeficiency virus or hepatitis C,Sjgrens syndrome,inflammatory bowel disease,melanoma,lymphoma,diabetes mellitus,and IgM,IgG,or IgA monoclonal gammopathy of unknown significance.The pathogenetic relevance of such concurrent diseases is unclearupdateAnti-neurofascin(神经束蛋白)IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset,ataxia,tremor,CNS demyelination,and a poor response to IV immunoglobulinDiffusion-weightedimagesinpatient10showedsignalabnormalitiesinthespleniumofthecorpuscallosum(胼胝体压部).Fluid-attenuated inversion recovery images in patients 10 and 31showedmultiplesclerosislikelesionsinthejuxtaventricular（脑室旁）regions.Therapeutic options for CIDPThe acquired chronic demyelinating neuropathies include：chronic inflammatory demyelinating polyneuropathy(CIDP)neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein(MAG;anti-MAG neuropathy)multifocal motor neuropathy(MMN)POEMS syndrome.They have characteristic-though overlapping-clinical presentations,are mediated by distinct immune mechanisms,and respond to different therapies.Figure 1|A suggested diagnostic pathway for chronic acquired demyelinating polyneuropathies.Differential diagnosis is based on electrodiagnostic studies,biopsies,serum biomarkers and antibodies.Abbreviations:CIDP,chronic inflammatory demyelinating polyneuropathy;IFE,immunofixation electrophoresis;MAG,myelin-associated glycoprotein;VEGF,vascular endothelial growth factor.THANKS