【医学课件】周围神经病-中国协和医科大学

周周 围围 神神 经经 病病 Peripheral NeuropathyPeripheral Neuropathy 中国协和医科大学 Peripheral neuropathy is a large Peripheral neuropathy is a large group of diseases:group of diseases: Damage of nerves outside of the Damage of nerves outside of the spinal cord and brain.spinal cord and brain. Dysfunction of these nerves. Dysfunction of these nerves. Caused by any of risk factors Caused by any of risk factors Anatomy and function of Anatomy and function of peripheral nerveperipheral nerve basic to understand basic to understand neuropathyneuropathy Anatomy Anatomy cranial nervecranial nerve olfactory nerve olfactory nerve optic nerveoptic nerve oculomotoroculomotor nerve nerve trochleartrochlear nerve nerve trigeminal nervetrigeminal nerve abducentabducent nerve facial nerve facial nervenerve vestibulocochlearvestibulocochlear nerve nerve glossopharyngealglossopharyngeal nerve nerve vagusvagus nerve nerve accessory nerve accessory nerve hypoglossal nerve hypoglossal nerve Spinal nerves HistologyHistology ElectrophysiologyElectrophysiology UnmyelinatedUnmyelinated nerve fiber nerve fiber MyelinatedMyelinated nerve fiber nerve fiber FunctionsFunctions Motor nerveMotor nerve Sensory nerveSensory nerve Autonomic nerveAutonomic nerve reflexreflex Step 1: Step 1: Clinical features of Clinical features of neuropathiesneuropathies history and examination of history and examination of patient is the clues to diagnosis of patient is the clues to diagnosis of neuropathyneuropathy Muscle weaknessMuscle weakness Weakness in the arms or legsWeakness in the arms or legs：difficulty walking, difficulty walking, running, climb stairs , Stumbling or tiring easily. running, climb stairs , Stumbling or tiring easily. Difficulties with carrying a load of groceries, Difficulties with carrying a load of groceries, opening jars, turning door knobs, or combing opening jars, turning door knobs, or combing hair. hair. Cranial nerve paralysis:Cranial nerve paralysis: ophthalmoplegiaophthalmoplegia, , facial weakness, facial weakness, dysarthriadysarthria, , Respiratory insufficientRespiratory insufficient Muscle atrophy. Muscle atrophy. Reflex lost. Reflex lost. tips:tips: Lower motor Lower motor neurogenicneurogenic damagedamage Anatomic Anatomic distributiondistribution SymmetricSymmetric proximal proximal distal distal AsymmetricAsymmetric single nerve single nerve multiple nervemultiple nerve plexus plexus nerve rootnerve root Sensory abnormalitiesSensory abnormalities ParesthesiasParesthesias Spontaneous sensations, Spontaneous sensations, numbness, tingling, pins and needles, prickling, numbness, tingling, pins 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. DysesthesiasDysesthesias Unpleasant abnormal sensations Unpleasant abnormal sensations brought on by touching or other stimuli. brought on 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 position, which leads to uncoordinated and of position, which leads to uncoordinated and unsteady walking.unsteady walking. tips:tips: l l Hearing patientHearing patient s s descriptiondescription l l Anatomic Anatomic distributiondistribution SymmetricSymmetric “ “Glove and StockingGlove and Stocking” ” distributiondistribution AsymmetricAsymmetric single nerve single nerve multiple nervemultiple nerve plexus plexus nerve root nerve root Autonomic Nerve DamageAutonomic Nerve Damage blurred visionblurred vision decreased ability to sweat (decreased ability to sweat (anhidrosisanhidrosis) ) decreased ability to regulate body temperaturedecreased 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 (impotence)sexual dysfunction (impotence) thinning of the skin, with easy thinning of the skin, with easy bruisabilitybruisability and and poor healing. poor healing. Step 2: Step 2: Laboratory findingsLaboratory findings nerve conductionnerve conduction study can confirm study can confirm neuropathy and distinguish between neuropathy and distinguish between damage to axons or the myelin sheath. damage to axons or the myelin sheath. electromyographyelectromyography differentiates differentiates myogenicmyogenic (muscle tissue) from (muscle tissue) from neurogenicneurogenic (nerve (nerve tissue) causes of weakness and can tissue) causes of weakness 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 Step3: causes DiseaseDisease 炎症性炎症性/ /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 维生素缺乏维生素缺乏 遗传性神经病遗传性神经病 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 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 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 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 神经病神经病 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 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 PathomechanismPathomechanism WallerianWallerian degeneration degeneration axonal degeneration axonal degeneration neuronal degenerationneuronal degeneration segmental segmental demyelinationdemyelination onion-bulb formationonion-bulb formation Interstitial neuropathyInterstitial neuropathy VasculiticVasculitic neuropathy neuropathy 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 l l 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 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 经传导异常和失神经经传导异常和失神经 电位。电位。 l l 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 缔组织病等缔组织病等 单神经病单神经病/ /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 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。 l l 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 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 吉兰吉兰巴雷综合征巴雷综合征 Guillain-BarreGuillain-Barre syndrome syndrome Guillain-BarreGuillain-Barre syndrome syndrome l l 18591859年年 LandryLandry s s AcendingAcending paralysis paralysis l l 18921892年年 acute febrile acute febrile polyneuroritispolyneuroritis l l 19181918年年 acute infective acute infective polineuritispolineuritis l l 19161916年年 GuillainGuillain- - Barre-StrohlBarre-Strohl syndrome syndrome Pathologic changes of AIDPPathologic changes of AIDP MechanismMechanismAntecedent events for Antecedent events for GBSGBS InfectionsInfections virusesviruses E-B virus E-B virus Cytomegalovirus Cytomegalovirus HIV HIV InfuenzaInfuenza virus virus Coxsackie viruses Coxsackie viruses Herpes simplex Herpes simplex Hepatitis Hepatitis A A and and C C virusesviruses Others Others Bacterial infectionBacterial infection Campylobacter Campylobacter jejunijejuni MycoplasmaMycoplasma pneumoniacpneumoniac Escherichia coli Escherichia coli Others Others ParasticParastic MarariaMararia Toxoplasmosis Toxoplasmosis Antecedent events for GBSAntecedent events for GBS Systemic illnessesSystemic illnesses HodgkinHodgkin s diseases disease lymphocytic leukemia lymphocytic leukemia HyperthoidismHyperthoidism Collagen vascular diseases Collagen vascular diseases SarcoidosisSarcoidosis Renal disease Renal disease Other medical conditionOther medical condition PregnancyPregnancy Surgical procedures Surgical procedures Bone marrow transplantation Bone marrow transplantation Immunizations Immunizations EnvenomizationEnvenomization Drug ingestion Drug ingestion ImmunopathologyImmunopathology Mechanism Mechanism molecular mimicrymolecular mimicry Classification of GBSClassification of GBS AIDPAIDP A Acutecute I Inflammatory nflammatory D Demyelinatingemyelinating P Polyneuropathyolyneuropathy Miller FisherMiller Fisher syndrome syndrome AcuteAcute panautonomicpanautonomic neuropathy(APNneuropathy(APN) ) acute sensory neuropathy, ASNacute sensory neuropathy, ASN AMAN/AMSANAMAN/AMSAN A Acutecute MMotor / otor / S Sensoryensory A Axonalxonal NNeuropathyeuropathy Concept of GBSConcept of GBS Acute or Acute or subacutesubacute onset onset Peripheral neuropathyPeripheral neuropathy albuminocytologicalbuminocytologic dissociation in CSF dissociation in CSF Immune-mediated neuropathyImmune-mediated neuropathy spontaneous recovery in most cases spontaneous recovery in most cases MonophasicMonophasic course course EpidemiologyEpidemiology Incidence 1-2 cases per 100,000 general Incidence 1-2 cases per 100,000 general populationpopulation All ages, mean age is 40yAll ages, mean age is 40y All races and nationalitiesAll races and nationalities A slight male predominanceA slight male predominance Clinical features of AIDP(1)Clinical features of AIDP(1) 50% have 50% have paresthesiasparesthesias and pain and pain at beginning at beginning followed by followed by weaknessweakness most begin in the legs most begin in the legs 10% begin in 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 muscles muscles , , 1/3 1/3 of of patients patients require require ventilator !ventilator ! Clinical features of AIDP(2)Clinical features of AIDP(2) 3-5% have complete 3-5% have complete ophthalmoplegiaophthalmoplegia 15% have autonomic manifestation15% have autonomic manifestation labile blood pressure, labile blood pressure, cardiac arrhythmias, cardiac arrhythmias, bladder dysfunction, bladder dysfunction, constipation, constipation, abdominal distension, abdominal distension, bloating bloating Neurologic examinationNeurologic examination QuadriplegiaQuadriplegia: Muscle weakness and : Muscle weakness and atrophyatrophy Muscle stretch Muscle stretch reflexes are absentreflexes are absent or or depresseddepressed Cranial nerve paralysis Cranial nerve paralysis Sensory loss in a stocking-glove Sensory loss in a stocking-glove distributiondistribution Pain in low back, buttocks, thighPain in low back, buttocks, thigh Exacerbated by straight leg raising Exacerbated by straight leg raising Autonomic dysfunctionAutonomic dysfunction CSFCSF 90% 90% of of cases, cases, protein protein is is elevated elevated by by the the nadir of illness nadir of illness without without leukocytosisleukocytosis. . 5% 5% of of cases, cases, pleocytosispleocytosis 10 10 (10-20) (10-20) cells/mmcells/mm 3 3 . . OB OB ( (oligoclonaloligoclonal bands) bands) IgGIgG/24h increasing/24h increasing MBPMBP increasing increasing Special Antibodies Special Antibodies : GM1: GM1 Electro-diagnostic studiesElectro-diagnostic studies AIDP: AIDP: DemyelinationDemyelination Axonal Axonal AMAN/AMSAN: AMAN/AMSAN: Axonal Axonal DemyelinationDemyelination SuralSural nerve biopsy nerve biopsy demyelinatingdemyelinating and and remyelinatingremyelinating axonal degenerationaxonal degeneration Infiltration of inflammatory cells : Infiltration of inflammatory cells : lymphocytes,macrophageslymphocytes,macrophages deposition of deposition of IgIg and complements and complements CourseCourse Most 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, occasionally, 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. . DiagnosisDiagnosis Clinical feature: acute progressive Clinical feature: acute progressive quadriplegia quadriplegia CSF: dissociation of protein/leukocytesCSF: dissociation of protein/leukocytes F-wave, NCV, EMG, MEPF-wave, NCV, EMG, MEP SuralSural nerve biopsy nerve biopsy Special antibodies in serum and CSFSpecial antibodies in serum and CSF Differential diagnosisDifferential diagnosis Periodic paralysisPeriodic paralysis PoliomyelitisPoliomyelitis MyelitisMyelitis Toxic neuropathyToxic neuropathy LymeLyme s diseases disease MyositisMyositis Treatment of GBS Treatment of GBS （1 1） Support careSupport care Prevent complicationsPrevent complications RehabilitationRehabilitation Psychology supportPsychology support immunotherapyimmunotherapy Treatment of GBS Treatment of GBS （2 2） ImmunotherapyImmunotherapy Plasma exchange (PE)Plasma exchange (PE) Iv Iv IgIg PE + IV PE + IV IgIg? ? Corticosteroid? Corticosteroid? IV IV IgIg CorticosteroidCorticosteroid？ PrognosisPrognosis 80% recovery within 6 months80% recovery within 6 months 15% have severe residual disability15% have severe residual disability Mortality rate is 3%-5%. Mortality rate is 3%-5%. Cause Cause of of death: death: ARDS ARDS with with or or without sepsis, without sepsis, dysautonomiadysautonomia. . Chronic inflammatory Chronic inflammatory demyelinatingdemyelinating polyradiculoneuritispolyradiculoneuritis (CIDP) (CIDP) Similar with AIDPSimilar with AIDP Courses: chronic progressive 2 months Courses: chronic progressive 2 months or relapse-remissionor relapse-remission Pathology: Pathology: demyelinationdemyelination, axonal , axonal degeneration, onion bulb formationdegener