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1、Peripheral Neuropathy周 围 神 经 病See P116-118,P127-129,YAN YONGDep.of Neurology,the 1st Hospital,Chongqing University of Medical Science,PREFACE,Peripheral Neuropathy(PN)Cranial n.Spinal n.(Ten pairs)Mononeuropathy Polyneuropathy a single several PN numerous PN ulnar n.ulnar n.+terminal radical radial
2、n.radial n.Nerves medial n.（末梢性)(根性)peroneal n.tibialis n.,Peripheral Neuropathy(PN)脑神经Cranial nerves.除嗅、视神经外的十对脑神经，最常见的有 三叉神经、眼球运动神经、面神经、舌咽和舌下神经 脊神经Spinal n.单神经病Mononeuropathy(symplex/multiplex)：尺神经、桡神经、正中神经、腓总神经、胫神经 多发性神经病Polyneuropathy：Terminal nerves 末梢神经炎、周围神经炎,感染、中毒各种原因 感染后免疫介导性病变：急性炎症性脱髓鞘性多发性
3、神经病（Guillain-Barre syndrome,GBS/AIDP）;慢性炎症性脱髓鞘性多发性神经病（CIDP）;Distal axonopathies远端轴索病；Myelinopathies髓鞘病,Causes of peripheral neuropathyIdiopathic inflammatory neuropathiesAcute inflammatory demyelinating polyneuropathy(AIDP,or acute Idiopathic polyneuropathy,Guillain-Barre syndrome),Chronic inflammat
4、ory demyelinating polyneuropathy(CIDP)Metabolic and nutritional neuropathiesDiabetes;Other endocrinopathies:hypothyroidism,acromegaly肢端肥大Uremia;Liver disease;VitaminB12 deficiencyInfective and granulomatous肉芽肿 neuropathiesAIDS;Leprosy麻风;Diphtheria白喉Sarcoidosis结节病;Sepses脓毒症 and multiorgan failure,Vas
5、culitic neuropathiesPolyarteritis nodosa结节性多动脉炎;Rheumatoid arthritis;Systemic lupus erythematousus系统性红斑狼疮(SLE)Neoplastic and paraproteinemic neuropathiesCompression and infiltration by tumor;Paraneoplastic syndromes副肿瘤综合征;paraproteinemiasAmyloidosis淀粉样变性Drug-induced and toxic neuropathiesAlcohol;oth
6、er drugsToxins:Organic compounds:Hexacarbons;organophosphates;Heavy metals:Arsenic;lead;Thallium;Gold;Platinum Tryptophan(contaminant),Hereditary neuropathies Idiopathic:Hereditary motor and sensory neuropathies;Hereditary sensory neuropathies遗传性感觉神经病 Friedreichs ataxia;Famillial amyloidosis淀粉样变性 Me
7、tabolic:Porphyria卟啉病 Metachromatic leukodystrophy Krabbes disease Abetalipoproteinemia Refsums disease Fabrys diseaseEntrapment压迫或嵌压性 neuropathies carpal tunnel syndrome腕管综合征,PATHOLOGY of PND1.Wallerian degeneration华勒变性2.Axonal degeneration轴突变性3.Segmental demyelination节段性脱髓鞘,The features of PN damag
8、e are the dysfunctionof motor,sensory and autonomic nerves.motor(weakness or paralysis)sensory(pain,paresthesia or anesthesia with stocking-and-glove pattern in distal of limbs)autonomic(tachycardia,cardiac Irregu-larities,labile blood pressure,disturbed sweating)can be seen in PN disorders,but sphi
9、ncter disturbance are rare.,Acute inflammatory demyelinating polyradiculoneuritis(AIDP)(急性炎症性脱髓鞘性多发性神经病，P127)Another names:Acute inflammatory polyneuropathy(急性炎症性多发性神经病)Guillain-Barre syndrome(GBS)(吉兰-巴雷、格林-巴利综合征)Acute idiopathic polyneuropathy(急性特发性多发性神经病),GBS is an acute or subacute onset of gener
10、ally symmetrical and progressive lower motor neurons paralysis(LMNP)of limbs and cranial nerves.Its a real radical neuropathies.it can follow minor infective illnesses,inoculations接种,or surgical procedures or may occur without obvious precipitants.,A.Etiology of GBS Its precise cause is unclear,but
11、it appears to have an immunologic basis.B.Pathology of GBS Both demyelinating 脱髓鞘(on anterior roots and peripheral nerves)and axonal forms 轴索型(some with axonal degeneration in CNS).,C.Clinical manifestations 1.Weakness is symmetrically usually begins in the legs,is often more marked proximally than
12、distally lower motor neurons lesion(hypotonia,hyporeflexia,wasting of affected muscles),between 10 and 14 days.The deep tendon reflexes are typically absent.The sever cases is the muscles of respiratory are involved.The respiratory muscles palsy may cause respiratory failure that is life threatening
13、!,2.Cranial nerves involvement:The th,oculomotor nerves(,and),and may be involved and produced facial palsy,ophthalmologic,and bulbar palsy which predispose to aspiration pneumonia.oculomotor动眼神经,trochlear滑车,abducent外展,glossopharygeal舌咽,vagus迷走,accessory副神经,3.Autonomic dysfunction自主神经功能障碍:There may
14、be marked autonomic dysfunction,with tachycardia心动过速,cardiacirregularities,labile blood pressure,disturbed sweating,but sphincter disorder(fecal or urinary incontinence)are rare.,4.Sensory involvement:Distal and symmetrical,as glove-and-stocking sensory loss,it usually less markedthan motor symptoms
15、,but frequent also.,5.Other clinical typesMiller-Fisher syndrome Its a subtype of GBS.Ataxia共济失调,hyporeflexia and oculomotor paralysis;not palsy of limbs;increased protein concentration in CSF;slowly with NCV神经传导速度.三个主要特征;共济失调、反射降低和眼球运动障碍。肢体无瘫痪。脑脊液蛋白增高。神经传导速度降低。,Acute axon motor neuropathy Its a spe
16、cial subtype of GBS.In northern China a related axonal form occurs frequently and has been designated acute motor axonal neuropathy.There are weakness of limbs(LMN)andwasting of affected muscles in the earlystage of course.The NCV are normal usually.,D.Investigative studies 1.CSF:A characteristic ab
17、normality with increased protein concentration after 1 week,but obvious in 2-3 weeks；CSF white blood cell count 10/mm3）(ie,CSF albuminocytologic dissociation 蛋白-细胞分离);,2.Electrophysiologic神经电生理studies may reveal marked slowing of motor conduction velocity(MCV 运动传导速度)and sensory conduction velocity(S
18、CV 感觉传导速度),or evidence of denervation失神经 and axonal loss.There are some reduced or loss with F waves and H reflex in the early stage of course 在疾病的早期阶段就可出现F波和H反射消失或减慢.,The main clinical features of GBS(AIDP):acute or subacute onset;Generally symmetrical progressive lower motor neurons paralysis(LMNP
19、)of limbs and cranial nerves;Weakness usually begins in the legs,more marked proximally than distally lower neurons lesion(hypotonia,hyporeflexia,wasting of affected muscles);The th,oculormotor nerves(,and),and cranial nerves may be involved;The F waves and H reflex reduced or loss,and slowing of MC
20、V and SCV,but the NCV may notreveal marked slowing in the early stage of course CSF albuminocytologic dissociation.,E.Diagnosis The features useful for diagnosing GBSare summarized in below table.1.Required for diagnosis Progressive weakness of more than one limb；一个以上肢体进行性无力 Distal areflexia with pr
21、oximal areflexia or hyporeflexia 腱反射消失或降低(肢体远端和近端,2.Supportive of diagnosisProgressive for up to 4 weeks;病情进展可达4周Relatively symmetric deficits;症状和体征相对对称Mild sensory involvement;轻度感觉障碍Cranial n.(especially)involvement;颅神经(尤以面神经)麻痹Recovery beginning within 4 wks after progressive stops 病情停止进展后在4周内开始恢复
22、Autonomic dysfunction;植物神经功能紊乱No fever at onset 发病时无发热Increased CSF protein after 1 week,but obvious in 3 weeks CSF white blood cell count 10/mm3 脑脊液蛋白1周后增高但3周后达高峰,白细胞数10/mm3Nerve conduction slowing or block by several weeks.神经传导速度减慢或在数周内消失,Against diagnosisMarkedly asymmetric weakness 显著的不对称性Bowel
23、or bladder dysfunction(at onset or Persistent)直肠或膀胱功能紊乱(发病时或病程中)CSF white blood cell count 50 脑脊液中白细胞大于50Well-demarcated sensory level 明显的感觉平面Excluding diagnosisIsolated sensory involvement 单独的感觉受损Another polyneuropathy that explains clinical picture,Differential diagnosis 1.MG 重症肌无力:The most import
24、ant symptom of MG is the fatigability,causing fluctuating weakness which is worse after exercise and usually at the end of the day.Edrophonium(Tensilon)test is positive.,2.Hypokalemic periodic paralysis(低钾性周期性麻痹See P346):Periodic episode,hypokalemia and abnormal of ECG心电图.3.CIDP慢性炎症性脱髓鞘性多发性神经病(See P
25、129):CIDP is clinically similar to GBS except that it follows a chronic progressive courseover 2 months or a course characterized byrelapses,and no improvement is apparent within the 6 months after onset.,F.TreatmentIntravenous immunoglobulin(IVIG,静脉用免疫球蛋白)appears to be equally effective and should
26、be used in preference to plasmapheresis in adults with cardiovascular instability and in children;the two therapies are not additive.2.Plasmapheresis(血浆置换)appears to reduce the time required for recovery and may decrease the likelihood of residual neurologic deficits.,3.Symptomatic therapy对症治疗 is ne
27、ed.Vitamine B1,B12,Vitamine C,To prevent respiratory failure and vascular collapse,when the patient has short breath,who are severely affected are best managed in the intensive care units(ICU),or the blood oxygen saturation declines sometimes tracheotomy气管切开is necessary for the patient with respirat
28、ory canalblotched by secretion or sputum,and the assistant respiratory machine is necessary for thepatient with respiratory failure呼吸衰竭.,Corticosteroids are not indicated because it may affect the outcome adversely or delay recovery.G.Prognosis It is self-limiting and cease to progress by about 4 we
29、eks,improvement occurs over the wks or mons following onset.,A typical caseA woman,30yrs old.She was admitted in hospital with symmetrically weakness and paresthesia of arms and legs for one day.Its normal of sphincter.The examination of NS showed that:wakefulness,a litter dysarthria,bilateral perip
30、heral facial palsy.There are low-grade of muscle toneand loss of tendon reflex on four limbs.There are analgesia anddysaphia below the wrists and ankles also.The muscular strength ofarms is 2 grade and 1 grade on legs.The pathologic reflexes aredeficit.There are muscle pain or tenderness of the bila
31、teral calves.典型病例 某女，30岁，因四肢无力、手和足麻木1天入院，大小便正常。神经系统检查：神志清楚，语言稍含糊。双侧周围性面神经麻痹。四肢肌张力低，腱反射消失，双上肢肌力2级、双下肢肌力1级。双上肢腕以下和双下肢踝以下痛觉触觉轻度减退。病理反射（）。双侧腓肠肌压痛。对此患者，请你判断是什么性质的瘫痪（上运动神经元或下运动神经元性瘫痪）？提出应该完成那些主要的检查？最可能的诊断是什么？应主要与哪些疾病鉴别？end,Key points1.Albuminocytologic dissociation in CSF蛋白-细胞分离2.the main clinical feature
32、s of GBS(AIDP):acute or subacute onset;Generally symmetrical progressive lower motor neurons paralysis(LMNP)of limbs andcranial nerves;Weakness usually begins in the legs,more marked proximally than distally lower neurons lesion(hypotonia,hyporeflexia,wasting of affected muscles);The th,oculomotor n
33、erves(,and),and cranial nerves may be involved;The respiratory muscles involved and palsy may cause respiratory failure in the sever cases.3.What changes of electrophysiologic in the GBS(AIDP)?The F waves and H reflex reduced or loss in the early stage of course but the NCV may not reveal marked slowing.4.How do differential diagnosis with other LMNP?,
