周 围 神 经 病 Peripheral Neuropathy,Peripheral 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 factors,Anatomy and function of peripheral nerve basic to understand neuropathy,Anatomy cranial nerve,olfactory nerveoptic nerveoculomotor nervetrochlear nervetrigeminal nerveabducent nerve facial nervevestibulocochlear nerveglossopharyngeal nervevagus nerve accessory nervehypoglossal nerve,Spinal nerves,Histology,Electrophysiology,Unmyelinated nerve fiberMyelinated nerve fiber,Functions,Motor nerve Sensory nerve Autonomic nerve reflex,Step 1: Clinical features of neuropathies history and examination of patient is the clues to diagnosis of neuropathy,Muscle weakness,Weakness 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, dysarthria, Respiratory insufficient Muscle atrophy. Reflex lost.,tips: Lower motor neurogenic damage Anatomic distribution Symmetricproximal distal Asymmetric single nerve multiple nerve plexus nerve root,Sensory abnormalities,Paresthesias Spontaneous sensations, numbness, tingling, pins and needles, prickling, burning, 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 position, which leads to uncoordinated and unsteady walking.,tips: Hearing patients description Anatomic distribution Symmetric“Glove and Stocking” distribution Asymmetricsingle nerve multiple nerveplexus nerve root,Autonomic Nerve Damage,blurred vision decreased ability to sweat (anhidrosis) decreased ability to regulate body temperature disturbances of bladder function orthostatic hypotension Constipation diarrhea, nausea after meals, abdominal bloating sexual dysfunction (impotence) thinning of the skin, with easy bruisability and poor healing.,Step 2: Laboratory findings,nerve conduction study can confirm neuropathy and distinguish between damage to axons or the myelin sheath. electromyography differentiates myogenic (muscle tissue) from neurogenic (nerve tissue) causes of weakness and can confirm abnormal neuromuscular junctions. nerve biopsy is needed only when other tests are inconclusive.,Step3: causes Disease,炎症性/免疫介导型神经病 中毒性神经病 维生素缺乏 遗传性神经病 神经病伴肿瘤 异常球蛋白血征相关的神经病 感染相关的神经病 系统性疾病 外伤切割、挤压、嵌压,神经病伴肿瘤 肿瘤的远隔效应 肿瘤直接浸润异常球蛋白血征相关的神经病感染相关的神经病 HIV 麻风 Lyme 病 带状疱疹,系统性疾病 糖尿病神经病 甲状腺功能低下 肾脏、肺、肝脏衰竭 危重病神经病 器官移植相关神经病外伤切割、挤压、嵌压,Pathomechanism,Wallerian degeneration axonal degeneration neuronal degeneration segmental demyelination onion-bulb formation Interstitial neuropathy Vasculitic neuropathy,周围神经病的临床病理分类,对称性广泛性多神经病 四肢远端对称性感觉运动障碍和皮肤植物神经功能异常。 电生理检查：广泛神经传导异常和失神经电位。 常见病因：中毒，代谢，遗传，免疫，结缔组织病等,单神经病/多发单神经病 单个神经支配区域感觉异常，局部肌肉无力萎缩，非对称性。 电生理检查：单神经常见原因：外伤，血管病，感染，肿瘤浸润，结缔组织病，甲低，等,吉兰巴雷综合征 Guillain-Barre syndrome,Guillain-Barre syndrome,1859年 Landrys Acending paralysis 1892年 acute febrile polyneuroritis 1918年 acute infective polineuritis 1916年 Guillain- Barre-Strohl syndrome,Pathologic changes of AIDP,MechanismAntecedent events for GBS,InfectionsvirusesE-B virusCytomegalovirusHIVInfuenza virusCoxsackie virusesHerpes simplexHepatitis A and C virusesOthers,Bacterial infectionCampylobacter jejuniMycoplasma pneumoniacEscherichia coliOthersParasticMarariaToxoplasmosis,Antecedent events for GBS,Systemic illnessesHodgkins diseaselymphocytic leukemiaHyperthoidismCollagen vascular diseasesSarcoidosisRenal disease,Other medical conditionPregnancySurgical proceduresBone marrow transplantationImmunizationsEnvenomizationDrug ingestion,Immunopathology Mechanism molecular mimicry,Classification of GBS,AIDPAcuteInflammatory Demyelinating Polyneuropathy,Miller Fisher syndrome Acute panautonomic neuropathy(APN) acute sensory neuropathy, ASN,AMAN/AMSANAcuteMotor / SensoryAxonalNeuropathy,Concept of GBS,Acute or subacute onset Peripheral neuropathy albuminocytologic dissociation in CSF Immune-mediated neuropathy spontaneous recovery in most cases Monophasic course,Epidemiology,Incidence 1-2 cases per 100,000 general population All ages, mean age is 40y All races and nationalities A slight male predominance,Clinical features of AIDP(1),50% have paresthesias and pain at beginning followed by weakness most begin in the legs10% begin in the arm rarely begins in the face weakness 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 ophthalmoplegia 15% have autonomic manifestationlabile blood pressure, cardiac arrhythmias, bladder dysfunction, constipation, abdominal distension, bloating,