Peripheral Neuropathy

周围神经病 Peripheral Neuropathy Department of Neurology,The 2nd affiliated hospital,Harbin Medical UniversityPeripheral NeuropathyvOutlinevGuillain-Barre SyndromevIdiopathic facial palsyvTrigeminal neuralgiaOUTLINEnAnatomy nPathological ProcessesnClinical classifation nSymptomsnInvestigation AnatomyPeripheral nerves are made up of numerous axons bound together by three types of connective tissueendoneurium,perineurium,epineurium.The vasa located in the epineurium provides the blood supply.(fig.1)Peripheral nerve trunks contain myelinated and unmyelinated fibresn Crinial nerven Spinal nervePathological ProcessesnWallerian degeneration(fig.2)nAxonal degenerationnNeuronal degenerationnSegmental demyelination(fig.3)SymptomsnSensory disturbancenMotor deficits nTendon reflexes nAutonomic disturbancesnOthersInvestigationnNerve conduction velocity(NCV)nElectromyography(EMG)nNerve biopsynBlood testsnCSF examination Trigeminal Neuralgia三叉神经痛Etiology and PathologynA facial pain syndrome of unknown cause.nDemyelination(fig.4)Clinical Features nIt develops in middle to late life.nIt consists of severe paroxysms electric-shock-like pain,usually in the V3 and V2 division of the trigeminal nerve(fig.5),lasting for several seconds or minutes each time.nInvolvement of V1 division or bilateral disease occurs in less than 5%of cases.Clinical FeaturesnOccurrence during sleep is rare.nPainfree intervals may last for minutes to weeks,but long-term spontaneous remission is rare.nSensory stimulation of trigger zones about the cheek,nose,or mouth by touch,cold,wind,talking,or chewing can precipitate the pain.Clinical FeaturesnTic douloureux(痛性抽搐)nCourse could be periodicnPhysical examination is normal Diagnosis and Differential DiagnosisnDiagnosis depending on the clinical featuresnDifferential Diagnosis n Secondary trigeminal neuralgian Tooth achen Glosspharyngeal neuralgia(舌咽神经痛)TreatmentnDrug is preferential AEDs:carbamazapine 0.1 bid po phenitoin,clonazepamnBaclofen(氯苯氨丁酸)nVitaminB12n封闭疗法:无水酒精,甘油n经皮半月神经节射频电凝疗法n手术治疗特发性面神经麻痹Idiopathic Facial Palsy(Bells palsy)Idiopathic Facial Palsy(Bells palsy)nAnatomy(fig.6)nEtiology and pathogenesisnClinical featuresnDiagnosis and differential diagnosisnTreatmentEtiology and Pathologyn尚未完全阐明，可有一些激发因素n受冷、病毒感染和自主神经不稳神经营养血管收缩而毛细血管扩张组织水肿压迫面神经面神经水肿和脱髓鞘，严重者有轴突变性。Clinical featuresnOnset Occurs in any age,usually unilateral.Paralysis:Progresses over 3 to 72 hours Pain(50%):Near mastoid process Excess tearing(33%)Other:Hyperacusis;Dysgeusia Clinical featuresnSigns Facial weakness(fig.7)Upper&Lower Unilateral Degree:Partial(30%);Complete(70%)Stapedius dysfunction(33%):Hyperacusis Lacrimation:mildly affected in some patients Taste:No clinically significant changes in most patients.There should be no sensory loss in the face.Hunt syndrome.No abnormalities beyond the territory of the facial nerve.Clinical featuresnPrognosis better Incomplete paralysis Early improvement Slow progression Younger age Clinical featuresnPrognosis better Normal taste Electrodiagnostic tests normal病后10天面神经出现失神经，恢复时间延长,平均需要3个月。一般12月内可逐渐痊愈。Diagnosis and diffential diagnosisn诊断：根据急性起病的周围性面瘫即可诊断。n鉴别：1 格林-巴利综合征 2 各种耳源性面神经麻痹 3 颅后窝的肿瘤或脑膜炎引起的周围性面瘫Treatmentn原则：改善局部血液循环，减轻面神经水肿，促进功能恢复。Corticosteroids:prednisone 60mg qd po dexamethasonnVitaminB1,B12nBaclofennRehabilitationnPhysiotherapy急性炎症性脱髓鞘性多发性神经病(acute inflammatory demyelinating polyneuropathies,AIDP)概念急性炎症性脱髓鞘性多发性神经病(acute infla-mmatory demyelinating polyneuropathies,AIDP)又称格林-巴利综合症(Guillain-Barr Syndrome GBS),是以周围神经和神经根的脱髓鞘及小血管周围淋巴细胞及巨噬细胞的炎性反应为病理特点的自身免疫病。Epidemiology nIncidence:0.6 to 1.9/100,000/year nMale:Female=1.25:1 nPeak ages:1625 years old 4560 years oldCertain forms of GBS appear to occur more frequently in certain areas of China.Etiology and pathogenesisnThe precise cause is unclear.nGBS often follows minor infective illness,inoculations or surgical procedures.Clinical and epidemiologic evidence suggest an association with preceding Campylobacter Jejuni(CJ)infection.(fig.8)nThe pathogenesis resembles EAN Molecular mimicryClinical featuresnGBS Prodrome It often follows 1-4 weeks after a respiratory infection or diarrhea.Campylobacter jejuni(CJ)has been particularly implicated as a cause of the diarrhea.Clinical featuresnWeakness:Most often symptomatic in legs Distribution:Proximal+Distal;Symmetric Severity:Quadriplegia in 30%;Bedbound another 30%Respiratory failure.Clinical featuresnSensory:ususlly less marked than motor symptoms.Paraesthesias:Initial symptom in 50%;Eventually occur in 70%to 90%Pain Loss:with classic glove-and-stocking pattern of sensory loss,but rarely occurs.Clinical featuresnCranial nerve:,facial weakness is present in 50%of cases.nAutonomic dysfunction tachycardia,cardiac irregularitis,labile blood presure,disturbed sweating and so on.nMonophase courseClinical featuresnClinical classification AIDP AMAN AMSAN Fisher syndrome Unclassifiable GBSInvestigationsnCSF:a characteristic abnormality,with increased protein concentration but a normal cell count.nEletrophysiologic studies marked slowing of motor and sensory condu-ction velocity,evidence of denervation and axonal loss.F wave reflex is delayed or absent.nSural nerve biopsy:demyelination Diagnostic criteria for GBS(1)Required for diagnosis Progressive weakness of more than one limb.Distal areflexia with proximal areflexia or hyporeflexia.Diagnostic criteria for GBS(2)Supportive of diagnosis Progression for up to 4 weaks.Relatively symmetric deficits.Mild sensory involvement.Cranial nerve(especially)involvement.Recovery beginning within 4 weeks after progression stops.Autonomic dysfunction.No fever at onset.Increased CSF protein after 1 week.CSF white blood cell count10/l.Nerve conduction slowing or block by several weeks.Differential diagnosisnHypokalemic periodic paralysis(Hopp)nPoliomyelitisnMyasthenia gravis(MG)TreatmentnAssisting respiration:Patients who are seve-rely affected are best managed in ICU where facilities are available for monitoring and assis-ted respiration if necessary.Sometimes antibiotic is necessary for preventing respiratory tracts infection.肺活量2025ml/kg,动脉氧分压70mmHgnSymptomatic therapy:The aim is to prevent such complications as respiratory failure or vascular collapse.TreatmentnPreventing complications:坠积性肺炎 褥疮 下肢深部静脉血栓、肺栓塞 肢体挛缩、畸形 吞咽麻痹 尿潴留 疼痛 焦虑及抑郁 TreatmentnEtiological therapy:Plasma exchange(plasmapheresis)Intravenous immunoglobulin:0.4g/kg/d for 5 days Corticosteroids:it has not been successful in acute GBS and can bring about adverse outcome.nRehabilitationPrognosisnThe disorder is self-limiting,and improvement occurs over the weeks or months following onset.About 70-75%of patients recover completely,25%are left with mild neurologic deficits,and 5%die,usually as a result of respiratory failure.nThe prognosis is poorer when there is evidence of preceding CJ infection.慢性炎症性脱髓鞘性多发性神经病 （CIDP）CIDP主要特点：n 慢性进行性或慢性复发性病程。n 起病隐袭，少有前驱感染。n 进展期平均3月，可有复发-缓解。n 对称肢体远端、近端无力，躯干肌、呼 吸肌、颅神经受累少见。n 运动、感觉障碍同存。CIDP主要特点：n CSF可见蛋白-细胞分离。n NCV、F波潜伏期均较AIDP重。n 腓肠神经活检：炎症性节段性脱髓鞘与再 生共存，“洋葱皮样”改变(fig.9)。n MRI：神经增粗。n 激素疗效肯定：prednison 100mg qd po(2-4W)。