慢性炎症性脱髓鞘性多发性神经根神经病进展课件

1、Department of Neurology, Fujian Provincial HospitalXingyong Chen 第一页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展IntroductionTraditionally, chronic inf lammatory demyelinating polyradiculoneuropathy (CIDP) has been considered a heterogeneous disorder including a broad spectrum of clinical phenotypes. The European Fed

2、eration 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 CIDP第二页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展prevalence of around 6 cases per 100,000 predominantly

3、affect males, and typically occur in middle to old ageCIDP can also occur in children. The incidence of CIDP increases with age, rising to 1.5 times the overall average in people over 65 years of age第三页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Clinical presentationClassic CIDP is characterized by the occurrence of

4、 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,

5、monophasic or GBS like onset.第四页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Newly recognised clinical features in CIDPAcute onset of CIDP may occur in up to 18% of CIDP patients, resembling the GBSdiagnosis was changed to CIDP in 5% of GBS patientsFatigue can be the main complaint in CIDP patients(75%)Activity-induc

6、ed 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 with CIDPAutonomic symptoms : 23%, of which gastrointestinal and genitourinary symptoms were most frequent (mild

7、)severe autonomic dysfunction should be regarded as a red flag when considering the diagnosis of CIDP.第五页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展CIDP PHENOTYPESThe clinical presentation of CIDP is variabledetermined by the number and distribution of the demyelinating peripheral nerve lesions第六页，共二十一页。慢性炎症性脱髓鞘性多发

8、性神经根神经病进展SM: sensory and motor. PE: plasma exchange; RR:relapsingremitting; CS:corticosteroids;第七页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Atypical CIDP第八页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Distal paresthesia and hypesthesia are the most frequent symptoms, followed by proprioceptive ataxia第九页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展DA

9、DSP(distal acquired demyelinating symmetric polyneuropathy ) : distal or predominantly sensory large fibre neuropathy length dependent axonal neuropathy.DML: distal motor latency第十页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展The LewisSumner syndrome (LSS), or multifocal acquired demyelinating sensory and motor neuro

10、pathy (MADSAM), has been reported in up to 15% of patients fulfilling the EFNS/PNS criteria for CIDP第十一页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展The combination of symmetric pattern of weakness without bulbar involvement clinically distinguishes this phenotype from lower motor neuron disease, but distinction can

11、be difficult, especially if electrophysiological criteria of demyelination are not met completely.第十二页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展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).第十三页，共二十一页。慢性炎症性脱髓鞘性多发性神经根

12、神经病进展 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

13、 pathogenetic relevance of such concurrent diseases is unclear第十四页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展updateAnti-neurofascin(神经(shnjng)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 immuno

14、globulin第十五页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Diffusion-weighted images in patient 10 showed signal abnormalities in the splenium of the corpus callosum(胼胝(pinzh)体压部). Fluid-attenuated inversion recovery images in patients 10 and 31 showed multiple sclerosislike lesions in the juxtaventricular（脑室旁） regions

15、.第十六页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Therapeutic options for CIDP第十七页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展The acquired chronic demyelinating neuropathies include ：chronic inflammatory demyelinating polyneuropathy (CIDP) neuropathy associated with monoclonal IgM antibodies to myelin-associated glycoprotein (MAG; an

16、ti-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. 第十八页，共二十一页。慢性炎症性脱髓鞘性多发性神经根神经病进展Figure 1 | A suggested diagnostic pathway for chroni

17、c 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.第十九