慢性炎症性脱髓鞘性多发性神经根神经病进展课件.ppt

1、IntroductionTraditionally,chronic inflammatory demyelinating polyradiculoneuropathy(CIDP)has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes.The European F e de r a ti on of N e ur ol og i ca l Societies/Peripheral Nerve Society(EFNS/PNS)CIDP treatment guid

2、eline has defined several clinical pictures as atypical CIDP phenotypes,additional to the classical(typical)picture of CIDP2prevalence of around 6 cases per 100,000 predominantly affect males,and typically occur in middle to old ageCIDP can also occur in children.The incidence of CIDP increases with

3、 age,rising to 1.5 times the overall average in people over 65 years of age3Clinical 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

4、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.4Newly recognised clinical features in CIDPAcute onset of CIDP may occur in up to 18%of CIDP patients,resembling the GBSdiagnosi

5、s was changed to CIDP in 5%of GBS patientsFatigue can be the main complaint in CIDP patients(75%)Activity-induced weaknessSevere pain is present in only a minority of CIDP patients;Tremor can be a disabling symptom in CIDP(50%)a higher incidence of almost 40%of restless legs syndrome in patients wit

6、h CIDPAutonomic 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 diagnosis of CIDP.5CIDP PHENOTYPESThe clinical presentation of CIDP is variabledetermined by the number and dis

7、tribution of the demyelinating peripheral nerve lesions6SM:sensory and motor.PE:plasma exchange;RR:relapsingremitting;CS:corticosteroids;7Atypical CIDP8Distal paresthesia and hypesthesia are the most frequent symptoms,followed by proprioceptive ataxia9DADSP(distal acquired demyelinating symmetric po

8、lyneuropathy):distal or predominantly sensory large fibre neuropathy length dependent axonal neuropathy.DML:distal motor latency10The 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

9、criteria for CIDP11The 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.12Focal CIDP is

10、defined as involvement of the brachial or lumbosacral plexus or of one or more peripheral nerves in one upper or lower limb(EFNS).13 Associated conditionsCIDP may be associated with various diseases,such as infection with the human immunodeficiency virus or hepatitis C,Sjgrens syndrome,inflammatory

11、bowel disease,melanoma,lymphoma,diabetes mellitus,and IgM,IgG,or IgA monoclonal gammopathy of unknown significance.The pathogenetic relevance of such concurrent diseases is unclear14updateAnti-neurofascin(神经束蛋白)IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger a

12、ge at onset,ataxia,tremor,CNS demyelination,and a poor response to IV immunoglobulin15Diffusion-weighted images in patient 10 showed signal abnormalities in the splenium of the corpus callosum(胼胝体压部).Fluid-attenuated inversion recovery images in patients 10 and 31 showed multiple sclerosislike lesio

13、ns in the juxtaventricular（脑室旁）regions.16Therapeutic options for CIDP17The acquired chronic demyelinating neuropathies include：chronic inflammatory demyelinating polyneuropathy(CIDP)neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein(MAG;anti-MAG neuropathy)multifo

14、cal motor neuropathy(MMN)POEMS syndrome.They have characteristic-though overlapping-clinical presentations,are mediated by distinct immune mechanisms,and respond to different therapies.18Figure 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.19THANKS20