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1、 周周 围围 神神 经经 病病 Peripheral NeuropathyPeripheral neuropathy is a large group of diseases:Damage of nerves outside of the spinal cord and brain.Dysfunction of these nerves.Caused by any of risk factorsAnatomyandfunctionofperipheralnervebasictounderstandneuropathyAnatomy cranial nerve olfactory nerve o
2、lfactory nerve optic nerveoptic nerve oculomotor nerveoculomotor nerve trochlear nervetrochlear nerve trigeminal nervetrigeminal nerve abducent nerve facial abducent nerve facial nervenerve vestibulocochlear nerve vestibulocochlear nerve glossopharyngeal nerve glossopharyngeal nerve vagus nerve vagu
3、s nerve accessory nerve accessory nerve hypoglossal nerve hypoglossal nerveSpinalnervesHistologyElectrophysiologyUnmyelinated nerve fiberUnmyelinated nerve fiberMyelinated nerve fiberMyelinated nerve fiberFunctionsMotor nerveMotor nerveMotor nerveMotor nerveSensory nerveSensory nerveSensory nerveSen
4、sory nerveAutonomic nerveAutonomic nerveAutonomic nerveAutonomic nervereflexreflexreflexreflexStep 1:ClinicalfeaturesofneuropathieshistoryandexaminationofpatientisthecluestodiagnosisofneuropathyMuscle weaknessWeaknessinthearmsorlegsWeaknessinthearmsorlegs：difficultywalking,difficultywalking,running,
5、climbstairs,Stumblingortiringeasily.running,climbstairs,Stumblingortiringeasily.Difficultieswithcarryingaloadofgroceries,Difficultieswithcarryingaloadofgroceries,openingjars,turningdoorknobs,orcombinghair.openingjars,turningdoorknobs,orcombinghair.Cranialnerveparalysis:Cranialnerveparalysis:ophthalm
6、oplegia,ophthalmoplegia,facialweakness,dysarthria,facialweakness,dysarthria,RespiratoryinsufficientRespiratoryinsufficientMuscleatrophy.Muscleatrophy.Reflexlost.Reflexlost.tips:tips:Lower motor Lower motor neurogenic neurogenic damagedamageAnatomic Anatomic distributiondistributionSymmetricSymmetric
7、 proximal proximal distal distalAsymmetricAsymmetricsingle nerve single nerve multiple nervemultiple nerveplexus plexus nerve rootnerve rootSensory abnormalitiesParesthesias Paresthesias Spontaneous sensations,Spontaneous sensations,numbness,tingling,pins and needles,prickling,numbness,tingling,pins
8、 and needles,prickling,burning,cold,pinching,sharp deep stabs,and burning,cold,pinching,sharp deep stabs,and electric shocks or buzzing.electric shocks or buzzing.Dysesthesias Dysesthesias Unpleasant abnormal sensations Unpleasant abnormal sensations brought on by touching or other stimuli.brought o
9、n by touching or other stimuli.Anesthesia Anesthesia a lack of sensation,leading to a lack of sensation,leading to injuries,cuts and burns due to the lack of injuries,cuts and burns due to the lack of normal warning sensations.a lack of sensation normal warning sensations.a lack of sensation of posi
10、tion,which leads to uncoordinated and of position,which leads to uncoordinated and unsteady walking.unsteady walking.tips:tips:l lHearing patientHearing patient s s descriptiondescriptionl lAnatomic Anatomic distributiondistributionSymmetricSymmetric “Glove and StockingGlove and Stocking”distributio
11、ndistributionAsymmetricAsymmetric single nerve single nerve multiple nervemultiple nerve plexus plexus nerve root nerve rootAutonomic Nerve Damage blurred visionblurred vision decreased ability to sweat(anhidrosis)decreased ability to sweat(anhidrosis)decreased ability to regulate body temperaturede
12、creased ability to regulate body temperature disturbances of bladder functiondisturbances of bladder function orthostatic hypotensionorthostatic hypotension ConstipationConstipation diarrhea,nausea after meals,abdominal bloatingdiarrhea,nausea after meals,abdominal bloating sexual dysfunction(impote
13、nce)sexual dysfunction(impotence)thinning of the skin,with easy bruisability and thinning of the skin,with easy bruisability and poor healing.poor healing.Step 2:Laboratory findingsnerve conductionnerve conduction study can confirm study can confirm neuropathy and distinguish between neuropathy and
14、distinguish between damage to axons or the myelin sheath.damage to axons or the myelin sheath.electromyographyelectromyography differentiates myogenic differentiates myogenic(muscle tissue)from neurogenic(nerve(muscle tissue)from neurogenic(nerve tissue)causes of weakness and can tissue)causes of we
15、akness and can confirm abnormal neuromuscular confirm abnormal neuromuscular junctions.junctions.nerve biopsynerve biopsy is needed only when other is needed only when other tests are inconclusive.tests are inconclusive.Step3:causes Disease炎症性炎症性炎症性炎症性/免疫介导型神经病免疫介导型神经病免疫介导型神经病免疫介导型神经病中毒性神经病中毒性神经病中毒性
16、神经病中毒性神经病维生素缺乏维生素缺乏维生素缺乏维生素缺乏遗传性神经病遗传性神经病遗传性神经病遗传性神经病神经病伴肿瘤神经病伴肿瘤神经病伴肿瘤神经病伴肿瘤异常球蛋白血征相关的神经病异常球蛋白血征相关的神经病异常球蛋白血征相关的神经病异常球蛋白血征相关的神经病感染相关的神经病感染相关的神经病感染相关的神经病感染相关的神经病系统性疾病系统性疾病系统性疾病系统性疾病外伤切割、挤压、嵌压外伤切割、挤压、嵌压外伤切割、挤压、嵌压外伤切割、挤压、嵌压神经病伴肿瘤神经病伴肿瘤神经病伴肿瘤神经病伴肿瘤肿瘤的远隔效应肿瘤的远隔效应肿瘤直接浸润肿瘤直接浸润异常球蛋白血征相关的异常球蛋白血征相关的异常球蛋白血征相关
17、的异常球蛋白血征相关的神经病神经病神经病神经病感染相关的神经病感染相关的神经病感染相关的神经病感染相关的神经病 HIVHIV 麻风麻风 Lyme Lyme 病病 带状疱疹带状疱疹系统性疾病系统性疾病系统性疾病系统性疾病糖尿病神经病糖尿病神经病 甲状腺功能低下甲状腺功能低下肾脏、肺、肝脏衰竭肾脏、肺、肝脏衰竭危重病神经病危重病神经病器官移植相关神经病器官移植相关神经病外伤切割、挤压、嵌压外伤切割、挤压、嵌压外伤切割、挤压、嵌压外伤切割、挤压、嵌压Pathomechanism WalleriandegenerationWalleriandegeneration axonaldegeneration
18、axonaldegeneration neuronaldegenerationneuronaldegeneration segmentaldemyelinationsegmentaldemyelination onion-bulbformationonion-bulbformation InterstitialneuropathyInterstitialneuropathy VasculiticneuropathyVasculiticneuropathy周围神经病的临床病理分类周围神经病的临床病理分类对称性广泛性多神经病对称性广泛性多神经病对称性广泛性多神经病对称性广泛性多神经病l l四肢远端
19、对称性感觉四肢远端对称性感觉四肢远端对称性感觉四肢远端对称性感觉运动障碍和皮肤植物运动障碍和皮肤植物运动障碍和皮肤植物运动障碍和皮肤植物神经功能异常。神经功能异常。神经功能异常。神经功能异常。l l电生理检查：广泛神电生理检查：广泛神电生理检查：广泛神电生理检查：广泛神经传导异常和失神经经传导异常和失神经经传导异常和失神经经传导异常和失神经电位。电位。电位。电位。l l常见病因：中毒，代常见病因：中毒，代常见病因：中毒，代常见病因：中毒，代谢，遗传，免疫，结谢，遗传，免疫，结谢，遗传，免疫，结谢，遗传，免疫，结缔组织病等缔组织病等缔组织病等缔组织病等单神经病单神经病单神经病单神经病/多发单神经
20、病多发单神经病多发单神经病多发单神经病l l单个神经支配区域感觉单个神经支配区域感觉单个神经支配区域感觉单个神经支配区域感觉异常，局部肌肉无力萎异常，局部肌肉无力萎异常，局部肌肉无力萎异常，局部肌肉无力萎缩，非对称性。缩，非对称性。缩，非对称性。缩，非对称性。l l电生理检查：单神经电生理检查：单神经电生理检查：单神经电生理检查：单神经l l常见原因：外伤，血管常见原因：外伤，血管常见原因：外伤，血管常见原因：外伤，血管病，感染，肿瘤浸润，病，感染，肿瘤浸润，病，感染，肿瘤浸润，病，感染，肿瘤浸润，结缔组织病，甲低，等结缔组织病，甲低，等结缔组织病，甲低，等结缔组织病，甲低，等吉兰吉兰巴雷综合
21、征巴雷综合征Guillain-BarresyndromeGuillain-Barresyndromel l18591859年年 LandryLandry s Acending paralysis s Acending paralysis l l18921892年年 acute febrile polyneuroritis acute febrile polyneuroritis l l19181918年年 acute infective polineuritis acute infective polineuritis l l19161916年年 Guillain-Barre-Strohl G
22、uillain-Barre-Strohl syndrome syndrome Pathologic changes of AIDPPathologic changes of AIDPMechanismAntecedenteventsforGBS InfectionsInfections virusesviruses E-B virus E-B virus Cytomegalovirus Cytomegalovirus HIV HIV Infuenza virus Infuenza virus Coxsackie viruses Coxsackie viruses Herpes simplex
23、Herpes simplex Hepatitis Hepatitis A A and and C C virusesviruses Others OthersBacterial infectionBacterial infection Campylobacter jejuniCampylobacter jejuni Mycoplasma Mycoplasma pneumoniacpneumoniac Escherichia coli Escherichia coli Others Others ParasticParastic Mararia Mararia Toxoplasmosis Tox
24、oplasmosisAntecedenteventsforGBS Systemic illnessesSystemic illnesses HodgkinHodgkin s diseases disease lymphocytic leukemia lymphocytic leukemia Hyperthoidism Hyperthoidism Collagen vascular diseases Collagen vascular diseases Sarcoidosis Sarcoidosis Renal disease Renal disease Other medical condit
25、ionOther medical condition PregnancyPregnancy Surgical procedures Surgical procedures Bone marrow transplantation Bone marrow transplantation Immunizations Immunizations Envenomization Envenomization Drug ingestion Drug ingestionImmunopathology Mechanism molecular mimicry Classification of GBSAIDPAI
26、DP A Acutecute I Inflammatory nflammatory D Demyelinating emyelinating P Polyneuropathyolyneuropathy Miller FisherMiller FisherMiller FisherMiller Fisher syndrome syndrome AcuteAcute panautonomic neuropathy(APN)panautonomic neuropathy(APN)acute sensory neuropathy,ASNacute sensory neuropathy,ASN AMAN
27、/AMSANAMAN/AMSAN A Acutecute MMotor/otor/S Sensoryensory A Axonalxonal NNeuropathyeuropathyConcept of GBSAcute or subacute onsetAcute or subacute onsetPeripheral neuropathyPeripheral neuropathyalbuminocytologic dissociation in CSF albuminocytologic dissociation in CSF Immune-mediated neuropathyImmun
28、e-mediated neuropathyspontaneous recovery in most cases spontaneous recovery in most cases Monophasic courseMonophasic courseEpidemiologyIncidence 1-2 cases per 100,000 general Incidence 1-2 cases per 100,000 general populationpopulationAll ages,mean age is 40yAll ages,mean age is 40yAll races and n
29、ationalitiesAll races and nationalitiesA slight male predominanceA slight male predominance ClinicalfeaturesofAIDP(1)50%have 50%have paresthesias and painparesthesias and pain at beginning at beginning followed by followed by weaknessweakness most begin in the legs most begin in the legs 10%begin in
30、 the arm 10%begin in the arm rarely begins in the face rarely begins in the face weaknessweakness may involve:may involve:proximal of lower limbs proximal of lower limbs upper limbs,upper limbs,facial muscle,facial muscle,pharyngeal and neck muscle pharyngeal and neck muscle，respiratory respiratory
31、muscles muscles,1/3 1/3 of of patients patients require require ventilator!ventilator!ClinicalfeaturesofAIDP(2)3-5%have complete ophthalmoplegia3-5%have complete ophthalmoplegia15%have autonomic manifestation15%have autonomic manifestation labile blood pressure,labile blood pressure,cardiac arrhythm
32、ias,cardiac arrhythmias,bladder dysfunction,bladder dysfunction,constipation,constipation,abdominal distension,abdominal distension,bloating bloatingNeurologic examinationQuadriplegiaQuadriplegia:Muscle weakness and:Muscle weakness and atrophyatrophyMuscle stretch Muscle stretch reflexes are absentr
33、eflexes are absent or or depresseddepressedCranial nerve paralysis Cranial nerve paralysis Sensory loss in a stocking-glove Sensory loss in a stocking-glove distributiondistributionPain in low back,buttocks,thighPain in low back,buttocks,thigh Exacerbated by straight leg raising Exacerbated by strai
34、ght leg raisingAutonomic dysfunctionAutonomic dysfunctionCSF90%90%of of cases,cases,protein protein is is elevated elevated by by the the nadir of illness nadir of illness without leukocytosiswithout leukocytosis.5%5%of of cases,cases,pleocytosis pleocytosis 10 10(10-20)(10-20)cells/mmcells/mm3 3.OB
35、 OB(oligoclonal bands)(oligoclonal bands)IgGIgG/24h increasing/24h increasingMBPMBP increasing increasingSpecial Antibodies Special Antibodies:GM1:GM1Electro-diagnostic studies AIDP:AIDP:Demyelination Axonal Demyelination Axonal AMAN/AMSAN:AMAN/AMSAN:Axonal Demyelination Axonal DemyelinationSural ne
36、rve biopsydemyelinating and remyelinatingdemyelinating and remyelinatingaxonal degenerationaxonal degenerationInfiltration of inflammatory cells:Infiltration of inflammatory cells:lymphocytes,macrophageslymphocytes,macrophagesdeposition of Ig and complementsdeposition of Ig and complementsCourse Mos
37、t Most patients patients of of AIDP AIDP become become maximally maximally weak weak within within 11-12 11-12 daysdays of of onset.onset.AMSN AMSN and and AMAN AMAN usually usually reach reach nadir within 6 days.nadir within 6 days.Most Most patients patients progressprogress steadily,steadily,occ
38、asionally,occasionally,stuttering stuttering or or stepwise stepwise course.course.The The average average time time to to onset onset of of recovery recovery is 4 weeksis 4 weeks.DiagnosisClinical feature:acute progressive Clinical feature:acute progressive quadriplegia quadriplegia CSF:dissociatio
39、n of protein/leukocytesCSF:dissociation of protein/leukocytesF-wave,NCV,EMG,MEPF-wave,NCV,EMG,MEPSural nerve biopsySural nerve biopsySpecial antibodies in serum and CSFDifferential diagnosisPeriodic paralysisPeriodic paralysisPoliomyelitisPoliomyelitisMyelitisMyelitisToxic neuropathyToxic neuropathy
40、LymeLyme s diseases diseaseMyositisMyositisTreatment of GBS（1）Support careSupport carePrevent complicationsPrevent complicationsRehabilitationRehabilitationPsychology supportPsychology supportimmunotherapyimmunotherapyTreatment of GBS（2）ImmunotherapyImmunotherapyPlasma exchange(PE)Plasma exchange(PE
41、)Iv IgIv IgPE+IV Ig?PE+IV Ig?Corticosteroid?Corticosteroid?IV IgIV Ig CorticosteroidCorticosteroid？Prognosis80%recovery within 6 months80%recovery within 6 months15%have severe residual disability15%have severe residual disabilityMortality rate is 3%-5%.Mortality rate is 3%-5%.Cause Cause of of deat
42、h:death:ARDS ARDS with with or or without sepsis,dysautonomia.without sepsis,dysautonomia.Chronic inflammatory demyelinating polyradiculoneuritis (CIDP)Similar with AIDPCourses:chronic progressive 2 months or relapse-remissionPathology:demyelination,axonal degeneration,onion bulb formationCauses:The
43、rapy:IVIg,plasma exchange,steroid单神经病和多发单神经病单神经病和多发单神经病mononeuropathy and multiple mononeuropathies常见疾病举例：常见疾病举例：常见疾病举例：常见疾病举例：1 1 1 1、颅神经：、颅神经：、颅神经：、颅神经：动眼神经麻痹，动眼神经麻痹，动眼神经麻痹，动眼神经麻痹，面神经麻痹面神经麻痹面神经麻痹面神经麻痹，面肌痉挛，面肌痉挛，面肌痉挛，面肌痉挛，三叉神经痛三叉神经痛三叉神经痛三叉神经痛2 2、臂丛神经炎，、臂丛神经炎，肘管综合症肘管综合症（尺神经麻痹），（尺神经麻痹），腕管综合症腕管综合症（正中神
44、经麻痹），桡神经麻痹（正中神经麻痹），桡神经麻痹3 3、肋间神经、躯干神经肋间神经、躯干神经4 4、坐骨神经痛，坐骨神经痛，股外侧皮神经股外侧皮神经炎，炎，腓总神经麻痹，腓总神经麻痹，胫神经麻痹胫神经麻痹周围神经病常见病因周围神经病常见病因炎症性炎症性/免疫介导型神经病免疫介导型神经病Guillain-Barre 综合征综合征CIDP血管炎性神经病血管炎性神经病肉芽肿性神经病肉芽肿性神经病结缔组织疾病伴神经病结缔组织疾病伴神经病血管炎血管炎中毒性神经病中毒性神经病中毒性神经病中毒性神经病药物药物药物药物酒精酒精酒精酒精金属金属金属金属 化学物质化学物质化学物质化学物质维生素缺乏维生素缺乏维生素
45、缺乏维生素缺乏维生素维生素维生素维生素B1B1维生素维生素维生素维生素B12B12 维生素维生素维生素维生素 E E遗传性神经病遗传性神经病遗传性运动感觉神经遗传性运动感觉神经遗传性运动感觉神经遗传性运动感觉神经病病病病 遗传性压迫易感性神遗传性压迫易感性神遗传性压迫易感性神遗传性压迫易感性神经病经病经病经病巨轴索性神经病巨轴索性神经病巨轴索性神经病巨轴索性神经病遗传性感觉和植物神遗传性感觉和植物神遗传性感觉和植物神遗传性感觉和植物神经病经病经病经病家族遗传性淀粉样变性家族遗传性淀粉样变性家族遗传性淀粉样变性家族遗传性淀粉样变性神经病神经病神经病神经病过氧化性疾病（肾上腺过氧化性疾病（肾上腺过
46、氧化性疾病（肾上腺过氧化性疾病（肾上腺白质营养不良，白质营养不良，白质营养不良，白质营养不良，Refsum Refsum 病）病）病）病）卟啉病卟啉病卟啉病卟啉病脂蛋白病（脂蛋白病（脂蛋白病（脂蛋白病（TangierTangier病，病，病，病，Abetaliproteinemia)Abetaliproteinemia)溶酶体酶缺陷溶酶体酶缺陷溶酶体酶缺陷溶酶体酶缺陷(Fabry Fabry 病病病病)遗传性运动感觉神经病遗传性运动感觉神经病遗传性运动感觉神经病遗传性运动感觉神经病（hereditary motor hereditary motor sensory neuropathy,sen
47、sory neuropathy,HMSNHMSN）又称腓骨肌萎缩症又称腓骨肌萎缩症又称腓骨肌萎缩症又称腓骨肌萎缩症（Charcot-Merie-Tooth Charcot-Merie-Tooth 综综综综合征合征合征合征CMTCMT）遗传性压迫易感性神经病遗传性压迫易感性神经病Fabry病病家族遗传性淀粉样变性神经病家族遗传性淀粉样变性神经病神经病伴肿瘤神经病伴肿瘤神经病伴肿瘤神经病伴肿瘤肿瘤的远隔效应肿瘤的远隔效应肿瘤直接浸润肿瘤直接浸润异常球蛋白血征相关的异常球蛋白血征相关的异常球蛋白血征相关的异常球蛋白血征相关的神经病神经病神经病神经病感染相关的神经病感染相关的神经病感染相关的神经病感染相关的神经病 HIVHIV 麻风麻风 Lyme Lyme 病病 带状疱疹带状疱疹系统性疾病系统性疾病系统性疾病系统性疾病糖尿病神经病糖尿病神经病 甲状腺功能低下甲状腺功能低下肾脏、肺、肝脏衰竭肾脏、肺、肝脏衰竭危重病神经病危重病神经病器官移植相关神经病器官移植相关神经病外伤切割、挤压、嵌压外伤切割、挤压、嵌压外伤切割、挤压、嵌压外伤切割、挤压、嵌压麻风病神经病麻风病神经病麻风病神经病麻风病神经病治疗治疗病因治疗病因治疗神经营养治疗神经营养治疗对症治疗对症治疗功能康复和并发症的治疗功能康复和并发症的治疗