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1、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,olfactor
2、y nerve optic nerve oculomotor nerve trochlear nerve trigeminal nerve abducent nerve facial nerve vestibulocochlear nerve glossopharyngeal nerve vagus nerve accessory nerve hypoglossal nerve,Spinal nerves,Histology,Electrophysiology,Unmyelinated nerve fiber Myelinated nerve fiber,Functions,Motor ner
3、ve 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
4、 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 Symmetric proximal distal Asymmetric single
5、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 st
6、imuli. 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” distrib
7、ution Asymmetric single nerve multiple nerve plexus 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 bl
8、oating 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 n
9、eurogenic (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
10、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
11、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 Guill
12、ain- Barre-Strohl syndrome,Pathologic changes of AIDP,MechanismAntecedent events for GBS,Infections viruses E-B virus Cytomegalovirus HIV Infuenza virus Coxsackie viruses Herpes simplex Hepatitis A and C viruses Others,Bacterial infection Campylobacter jejuni Mycoplasma pneumoniac Escherichia coli O
13、thers Parastic Mararia Toxoplasmosis,Antecedent events for GBS,Systemic illnesses Hodgkins disease lymphocytic leukemia Hyperthoidism Collagen vascular diseases Sarcoidosis Renal disease,Other medical condition Pregnancy Surgical procedures Bone marrow transplantation Immunizations Envenomization Dr
14、ug ingestion,Immunopathology Mechanism molecular mimicry,Classification of GBS,AIDP Acute Inflammatory Demyelinating Polyneuropathy,Miller Fisher syndrome Acute panautonomic neuropathy(APN) acute sensory neuropathy, ASN,AMAN/AMSAN Acute Motor / Sensory Axonal Neuropathy,Concept of GBS,Acute or subac
15、ute 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,Clini
16、cal features of AIDP(1),50% have paresthesias and pain at beginning followed by weakness most begin in the legs 10% 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 r
17、equire ventilator !,Clinical features of AIDP(2),3-5% have complete ophthalmoplegia 15% have autonomic manifestation labile blood pressure, cardiac arrhythmias, bladder dysfunction, constipation, abdominal distension, bloating,Neurologic examination,Quadriplegia: Muscle weakness and atrophy Muscle s
18、tretch reflexes are absent or depressed Cranial nerve paralysis Sensory loss in a stocking-glove distribution Pain in low back, buttocks, thigh Exacerbated by straight leg raising Autonomic dysfunction,CSF,90% of cases, protein is elevated by the nadir of illness without leukocytosis. 5% of cases, p
19、leocytosis 10 (10-20) cells/mm3. OB (oligoclonal bands) IgG/24h increasing MBP increasing Special Antibodies : GM1,Electro-diagnostic studies,AIDP: Demyelination Axonal,AMAN/AMSAN: Axonal Demyelination,Sural nerve biopsy,demyelinating and remyelinating axonal degeneration Infiltration of inflammator
20、y cells : lymphocytes,macrophages deposition of Ig and complements,Course,Most patients of AIDP become maximally weak within 11-12 days of onset. AMSN and AMAN usually reach nadir within 6 days. Most patients progress steadily, occasionally, stuttering or stepwise course. The average time to onset o
21、f recovery is 4 weeks.,Diagnosis,Clinical feature: acute progressive quadriplegia CSF: dissociation of protein/leukocytes F-wave, NCV, EMG, MEP Sural nerve biopsy Special antibodies in serum and CSF,Differential diagnosis,Periodic paralysis Poliomyelitis Myelitis Toxic neuropathy Lymes disease Myosi
22、tis,Treatment of GBS （1）,Support care Prevent complications Rehabilitation Psychology support immunotherapy,Treatment of GBS （2）,Immunotherapy Plasma exchange (PE) Iv Ig PE + IV Ig? Corticosteroid? IV Ig Corticosteroid？,Prognosis,80% recovery within 6 months 15% have severe residual disability Morta
23、lity rate is 3%-5%. Cause of death: ARDS with or without sepsis, dysautonomia.,Chronic inflammatory demyelinating polyradiculoneuritis (CIDP),Similar with AIDP Courses: chronic progressive 2 months or relapse-remission Pathology: demyelination, axonal degeneration, onion bulb formation Causes: Thera
24、py: IVIg, plasma exchange, steroid,单神经病和多发单神经病 mononeuropathy and multiple mononeuropathies,常见疾病举例： 1、颅神经：动眼神经麻痹，面神经麻痹，面肌痉挛，三叉神经痛,2、臂丛神经炎，肘管综合症（尺神经麻痹），腕管综合症（正中神经麻痹），桡神经麻痹,3、肋间神经、躯干神经 4、坐骨神经痛，股外侧皮神经炎，腓总神经麻痹，胫神经麻痹,周围神经病常见病因,炎症性/免疫介导型神经病 Guillain-Barre 综合征 CIDP 血管炎性神经病 肉芽肿性神经病 结缔组织疾病伴神经病,血管炎,中毒性神经病 药物
25、酒精 金属 化学物质 维生素缺乏 维生素B1 维生素B12 维生素 E,遗传性神经病,遗传性运动感觉神经病 遗传性压迫易感性神经病 巨轴索性神经病 遗传性感觉和植物神经病,家族遗传性淀粉样变性神经病 过氧化性疾病（肾上腺白质营养不良，Refsum 病） 卟啉病 脂蛋白病（Tangier病， Abetaliproteinemia) 溶酶体酶缺陷 (Fabry 病),遗传性运动感觉神经病（hereditary motor sensory neuropathy, HMSN） 又称腓骨肌萎缩症 （Charcot-Merie-Tooth 综合征CMT）,遗传性压迫易感性神经病,Fabry 病,家族遗传性淀粉样变性神经病,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,麻风病神经病,治疗,病因治疗 神经营养治疗 对症治疗 功能康复和并发症的治疗,