七年制医学课件 神经病学 13Peripheral neuropathy.ppt
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1、Peripheral NeuropathyPeripheral Neuropathy周 围 神 经 病See P116-118,P127-129YAN YONGDep. of Neurology, the 1st Hospital, Chongqing University of Medical SciencePREFACE Peripheral Neuropathy(PN) Cranial n. Spinal n. (Ten pairs) Mononeuropathy Polyneuropathy a single several PN numerous PN ulnar n. ulnar
2、n.+ terminal radical radial n. radial n. Nerves medial n. (末梢性末梢性) (根性根性) peroneal n. tibialis n. Peripheral Neuropathy(PN)脑神经脑神经Cranial nerves. 除嗅、视神经外的十对脑神经,最常见的有除嗅、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、舌咽和舌下神经三叉神经、眼球运动神经、面神经、舌咽和舌下神经 脊神经脊神经Spinal n. 单神经病单神经病Mononeuropathy(symplex / multiplex) : 尺神经、桡神经
3、、正中神经、腓总神经、胫神经 多发性神经病多发性神经病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:感染后免疫介导性病变: 急性炎症性脱髓鞘性多发性神经病( 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 neuropathiesIdiopath
4、ic inflammatory neuropathiesAcute inflammatory demyelinating polyneuropathy(AIDP,or acute Idiopathic polyneuropathy,Guillain-Barre syndrome),Chronic inflammatory demyelinating polyneuropathy(CIDP)Metabolic and nutritional neuropathiesDiabetes;Other endocrinopathies: hypothyroidism,acromegaly肢端肥大Urem
5、ia; 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 failureVasculitic neuropathiesPolyarteritis nodosa结节性多动脉炎结节性多动脉炎; Rheumatoid arthritis;Systemic lupus erythematousus系统性红斑狼疮系统性红斑狼疮(SLE)Neoplastic a
6、nd paraproteinemic neuropathiesCompression and infiltration by tumor;Paraneoplastic syndromes副肿瘤综合征副肿瘤综合征; paraproteinemiasAmyloidosis淀粉样变性淀粉样变性Drug-induced and toxic neuropathiesAlcohol; other drugsToxins: Organic compounds: Hexacarbons; organophosphates; Heavy metals: Arsenic; lead; Thallium; Gold
7、; Platinum Tryptophan(contaminant)Hereditary neuropathies Idiopathic: Hereditary motor and sensory neuropathies; Hereditary sensory neuropathies遗传性感觉神经病遗传性感觉神经病 Friedreichs ataxia; Famillial amyloidosis淀粉样变性 Metabolic: Porphyria卟啉病 Metachromatic leukodystrophy Krabbes disease Abetalipoproteinemia Re
8、fsums disease Fabrys diseaseEntrapment压迫或嵌压性压迫或嵌压性 neuropathies carpal tunnel syndrome腕管综合征腕管综合征 PATHOLOGY of PNDPATHOLOGY of PND1.Wallerian degeneration1.Wallerian degeneration华勒变性华勒变性2.Axonal degeneration2.Axonal degeneration轴突变性轴突变性3.Segmental demyelination3.Segmental demyelination节段性脱髓鞘节段性脱髓鞘The
9、 features of PN damage 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
10、be seen in PN disorders, but sphincter disturbance are rare. Acute inflammatory demyelinating polyradiculoneuritis (AIDP) (急性炎症性脱髓鞘性多发性神经病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急性炎症性多发性神经病)Guillain-Barre syndrome(GBSGBS ) ( (吉兰吉兰- -巴雷巴雷、格林格林-巴利综合征巴利综合征)Acu
11、te idiopathic polyneuropathy (急性特发性多发性神经病急性特发性多发性神经病) GBS is an acute or subacute onset of generally symmetrical and progressive lower motor neurons paralysis(LMNPLMNP) of limbs and cranial nerves. Its a real radical neuropathies. it can follow minor infective illnesses, inoculations接种,or surgical p
12、rocedures or may occur without obvious precipitants. A. Etiology of GBS Its precise cause is unclear, but it appears to have an immunologic basis. B.Pathology of GBS Both demyelinating 脱髓鞘脱髓鞘(on anterior roots and peripheral nerves) and axonal forms 轴索轴索型型(some with axonal degeneration in CNS ). C.
13、Clinical manifestations 1.Weakness is symmetrically usually begins in the legs, is often more marked proximally than 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 th
14、e muscles of respiratory are involved.The respiratory muscles palsy may cause respiratory failure that is life threatening! 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
15、pneumonia. oculomotor动眼神经动眼神经,trochlear滑车滑车,abducent外展外展, glossopharygeal舌咽舌咽,vagus迷走迷走,accessory副神经副神经 3.Autonomic dysfunction自主神经功能障碍自主神经功能障碍: There may be marked autonomic dysfunction,with tachycardia心动过速心动过速, cardiacirregularities, labile blood pressure, disturbed sweating, but sphincter disorde
16、r(fecal or urinary incontinence) are rare. 4.Sensory involvement: Distal and symmetrical, as glove-and-stocking sensory loss,it usually less markedthan motor symptoms,but frequent also. 5.Other clinical types 5.Other clinical types Miller-Fisher syndrome Its a subtype of GBS. Ataxia共济失调共济失调, hyporef
17、lexia and oculomotor paralysis; not palsy of limbs; increased protein concentration in CSF; slowly with NCV神经传导速度神经传导速度. 三个主要特征三个主要特征;共济失调、反射降低和眼球运动障碍。肢体共济失调、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。无瘫痪。脑脊液蛋白增高。神经传导速度降低。 Acute axon motor neuropathy Its a special subtype of GBS. In northern China a related
18、 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 abnormality with increased protein concent
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