周 圍 神 經 病課件

1、 周 圍 神 經 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 factorsAnatomy and function of peripheral nerve basic to understand neuropathyAnatomy cranial nerve olfactory

2、nerve optic nerve oculomotor nerve trochlear nerve trigeminal nerve abducent nerve facial nerve vestibulocochlear nerve glossopharyngeal nerve vagus nerve accessory nerve hypoglossal nerveSpinal nervesElectrophysiologyUnmyelinated nerve fiberMyelinated nerve fiberFunctionsMotor nerveSensory nerveAut

3、onomic nervereflexMuscle weaknessWeakness in the arms or legs：difficulty walking, running, climb stairs , Stumbling or tiring easily. Difficulties with carrying a load of groceries, opening jars, turning door knobs, or combing hair. Cranial nerve paralysis: ophthalmoplegia, facial weakness, dysarthr

4、ia, Respiratory insufficientMuscle atrophy. Reflex lost.tips: Lower motor neurogenic damageAnatomic distributionSymmetric proximal distalAsymmetricsingle nerve multiple nerveplexus nerve rootSensory abnormalitiesParesthesias Spontaneous sensations, numbness, tingling, pins and needles, prickling, bu

5、rning, cold, pinching, sharp deep stabs, and electric shocks or buzzing. Dysesthesias Unpleasant abnormal sensations brought on by touching or other stimuli. Anesthesia a lack of sensation, leading to injuries, cuts and burns due to the lack of normal warning sensations. a lack of sensation of posit

6、ion, which leads to uncoordinated and unsteady walking.Autonomic Nerve Damageblurred visiondecreased ability to sweat (anhidrosis)decreased ability to regulate body temperaturedisturbances of bladder functionorthostatic hypotensionConstipationdiarrhea, nausea after meals, abdominal bloatingsexual dy

7、sfunction (impotence)thinning of the skin, with easy bruisability and poor healing. Step 2: Laboratory findingsnerve conduction study can confirm neuropathy and distinguish between damage to axons or the myelin sheath. electromyography differentiates myogenic (muscle tissue) from neurogenic (nerve t

8、issue) causes of weakness and can confirm abnormal neuromuscular junctions. nerve biopsy is needed only when other tests are inconclusive. Step3: causes Disease炎癥性/免疫介導型神經病中毒性神經病維生素缺乏遺傳性神經病神經病伴腫瘤異常球蛋白血征相關的神經病感染相關的神經病系統(tǒng)性疾病外傷切割、擠壓、嵌壓神經病伴腫瘤腫瘤的遠隔效應腫瘤直接浸潤異常球蛋白血征相關的神經病感染相關的神經病HIV麻風Lyme 病帶狀皰疹系統(tǒng)性疾病糖尿病神經病甲狀腺

9、功能低下腎臟、肺、肝臟衰竭危重病神經病器官移植相關神經病外傷切割、擠壓、嵌壓PathomechanismWallerian degenerationaxonal degeneration neuronal degenerationsegmental demyelinationonion-bulb formationInterstitial neuropathy Vasculitic neuropathy周圍神經病的臨床病理分類對稱性廣泛性多神經病四肢遠端對稱性感覺運動障礙和皮膚植物神經功能異常。電生理檢查：廣泛神經傳導異常和失神經電位。常見病因：中毒，代謝，遺傳，免疫，結締組織病等單神經病/多

10、發(fā)單神經病單個神經支配區(qū)域感覺異常，局部肌肉無力萎縮，非對稱性。電生理檢查：單神經常見原因：外傷，血管病，感染，腫瘤浸潤，結締組織病，甲低，等吉蘭巴雷綜合征 Guillain-Barre syndromeGuillain-Barre syndrome1859年 Landrys Acending paralysis 1892年 acute febrile polyneuroritis 1918年 acute infective polineuritis 1916年 Guillain- Barre-Strohl syndrome MechanismAntecedent events for GBS

11、Infections viruses E-B virus Cytomegalovirus HIV Infuenza virus Coxsackie viruses Herpes simplex Hepatitis A and C viruses OthersBacterial infection Campylobacter jejuni Mycoplasma pneumoniac Escherichia coli OthersParastic Mararia ToxoplasmosisAntecedent events for GBSSystemic illnesses Hodgkins di

12、sease lymphocytic leukemia Hyperthoidism Collagen vascular diseases Sarcoidosis Renal diseaseOther medical condition Pregnancy Surgical procedures Bone marrow transplantation Immunizations Envenomization Drug ingestionClassification of GBSAIDP Acute Inflammatory Demyelinating PolyneuropathyMiller Fi

13、sher syndromeAcute panautonomic neuropathy(APN)acute sensory neuropathy, ASN AMAN/AMSAN Acute Motor / Sensory Axonal NeuropathyConcept of GBSAcute or subacute onsetPeripheral neuropathyalbuminocytologic dissociation in CSF Immune-mediated neuropathyspontaneous recovery in most cases Monophasic cours

14、eEpidemiologyIncidence 1-2 cases per 100,000 general populationAll ages, mean age is 40yAll races and nationalitiesA slight male predominance Clinical features of AIDP(1)50% have paresthesias and pain at beginningfollowed by weakness most begin in the legs 10% begin in the arm rarely begins in the f

15、aceweakness may involve: proximal of lower limbs upper limbs, facial muscle, pharyngeal and neck muscle， respiratory muscles , 1/3 of patients require ventilator !Clinical features of AIDP(2)3-5% have complete ophthalmoplegia15% have autonomic manifestation labile blood pressure, cardiac arrhythmias

16、, bladder dysfunction, constipation, abdominal distension, bloatingNeurologic examinationQuadriplegia: Muscle weakness and atrophyMuscle stretch reflexes are absent or depressedCranial nerve paralysis Sensory loss in a stocking-glove distributionPain in low back, buttocks, thigh Exacerbated by strai

17、ght leg raisingAutonomic dysfunctionCSF90% of cases, protein is elevated by the nadir of illness without leukocytosis.5% of cases, pleocytosis 10 (10-20) cells/mm3.OB (oligoclonal bands) IgG/24h increasingMBP increasingSpecial Antibodies : GM1Electro-diagnostic studiesAIDP: Demyelination Axonal AMAN

18、/AMSAN: Axonal DemyelinationSural nerve biopsydemyelinating and remyelinatingaxonal degenerationInfiltration of inflammatory cells : lymphocytes,macrophagesdeposition of Ig and complementsCourse Most patients of AIDP become maximally weak within 11-12 days of onset. AMSN and AMAN usually reach nadir

19、 within 6 days.Most patients progress steadily, occasionally, stuttering or stepwise course.The average time to onset of recovery is 4 weeks.DiagnosisClinical feature: acute progressive quadriplegia CSF: dissociation of protein/leukocytesF-wave, NCV, EMG, MEPSural nerve biopsySpecial antibodies in s

20、erum and CSFDifferential diagnosisPeriodic paralysisPoliomyelitisMyelitisToxic neuropathyLymes diseaseMyositisTreatment of GBS （1）Support carePrevent complicationsRehabilitationPsychology supportimmunotherapyTreatment of GBS （2）ImmunotherapyPlasma exchange (PE)Iv IgPE + IV Ig?Corticosteroid? IV Ig C

21、orticosteroid？Prognosis80% recovery within 6 months15% have severe residual disabilityMortality rate is 3%-5%. Cause of death: ARDS with or without sepsis, dysautonomia.Chronic inflammatory demyelinating polyradiculoneuritis (CIDP)Similar with AIDPCourses: chronic progressive 2 months or relapse-remissionPathology: demyelination, axonal degeneration, onion bulb formationCauses:Therapy