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
19、ration after 1 week, but obvious in 2-3 weeks；CSF white blood cell count 10/mm3 ） (ie, CSF albuminocytologic dissociation 蛋白蛋白- -细胞分细胞分离离); 2.Electrophysiologic神经电生理神经电生理studies may reveal marked slowing of motor conduction velocity(MCV 运动传导速度运动传导速度) and sensory conduction velocity(SCV 感觉传导速度感觉传导速度)
20、, or evidence of denervation失神经失神经 and axonal loss. There are some reduced or loss with F waves and H reflex in the early stage of course 在疾病的早期阶段就可出现在疾病的早期阶段就可出现F波和波和H反射消失或减慢反射消失或减慢.The main clinical features of GBS (AIDP) :acute or subacute onset; Generally symmetrical progressive lower motor neur
21、ons paralysis(LMNPLMNP) of limbs and cranial nerves; Weakness usually begins in the legs, more marked proximally than distally lower neurons lesion (hypotonia, hyporeflexia,wasting of affected muscles); The th, oculormotor nerves (, and ), , and cranial nerves may be involved ;The F waves and H refl
22、ex reduced or loss,and slowing of MCV and SCV, but the NCV may notreveal marked slowing in the early stage of course CSF albuminocytologic dissociation. E. Diagnosis The features useful for diagnosing GBSare summarized in below table. 1.Required for diagnosis Progressive weakness of more than one li
23、mb；一个以上肢体进行性无力一个以上肢体进行性无力 Distal areflexia with proximal areflexia or hyporeflexia 腱反射消失或降低腱反射消失或降低( (肢体远端和近端肢体远端和近端2.Supportive of diagnosis Progressive for up to 4 weeks; 病情进展可达病情进展可达4 4周周 Relatively symmetric deficits;症状和体征相对对称症状和体征相对对称 Mild sensory involvement; 轻度感觉障碍轻度感觉障碍 Cranial n.(especially
24、 ) involvement;颅神经颅神经(尤以面神经尤以面神经)麻痹麻痹 Recovery beginning within 4 wks after progressive stops 病情停止进展后在病情停止进展后在4 4周内开始恢复周内开始恢复 Autonomic dysfunction;植物神经功能紊乱植物神经功能紊乱 No fever at onset 发病时无发热发病时无发热 Increased CSF protein after 1 week,but obvious in 3 weeks CSF white blood cell count 10/mm3 脑脊液蛋白脑脊液蛋白1
25、1周后增周后增高但高但3 3周后达高峰周后达高峰, ,白细胞数白细胞数10/mm3 Nerve conduction slowing or block by several weeks. 神经传导速度减慢或在数周内消失神经传导速度减慢或在数周内消失Against diagnosisAgainst diagnosis Markedly asymmetric weakness 显著的不对称性显著的不对称性 Bowel or bladder dysfunction ( at onset or Persistent ) 直肠或膀胱功能紊乱直肠或膀胱功能紊乱( (发病时或病程中发病时或病程中) ) CS
26、F white blood cell count 50 脑脊液中白细胞大于脑脊液中白细胞大于5050 Well-demarcated sensory level 明显的感觉平面明显的感觉平面Excluding diagnosis Isolated sensory involvement 单独的感觉受损单独的感觉受损 Another polyneuropathy that explains clinical picture Differential diagnosisDifferential diagnosis 1.MG 重症肌无力重症肌无力: The most important sympto
27、m of MG is the fatigability, causing fluctuating weakness which is worse after exercise and usually at the end of the day. Edrophonium(Tensilon) test is positive.2.Hypokalemic periodic paralysis(低钾性周期性麻低钾性周期性麻痹痹See P346): Periodic episode, hypokalemia and abnormal of ECG心电图心电图.3.CIDP慢性炎症性脱髓鞘性多发性神经病慢
28、性炎症性脱髓鞘性多发性神经病 (See P129) : CIDP is clinically similar to GBS except that it follows a chronic progressive courseover 2 months or a course characterized byrelapses,and no improvement is apparent within the 6 months after onset. F. Treatment1. Intravenous immunoglobulin (IVIG,静脉静脉用免疫球蛋白用免疫球蛋白) appear
29、s to be equally effective and should be used in preference to plasmapheresis in adults with cardiovascular instability and in children;the two therapies are not additive.2. Plasmapheresis(血浆置换血浆置换) appears to reduce the time required for recovery and may decrease the likelihood of residual neurologi
30、c deficits. 3.Symptomatic therapy对症治疗 is needis need. Vitamine B1,B12,Vitamine C, To prevent respiratory failure and vascular collapse,when the patient has short breath, who are severely affected are best managed in the intensive care units(ICU), or the blood oxygen saturation declines sometimes tra
31、cheotomy气管切开气管切开is necessary for the patient with respiratory canalblotched by secretion or sputum, and the assistant respiratory machine is necessary for thepatient with respiratory failure呼吸衰竭呼吸衰竭 . Corticosteroids are not indicated because it may affect the outcome adversely or delay recovery. G.
32、 Prognosis It is self-limiting and cease to progress by about 4 weeks, improvement occurs over the wks or mons following onset. A typical caseA woman, 30yrs old. She was admitted in hospital with symmetrically weakness and paresthesia of arms and legs for one day. Its normal of sphincter. The examin
33、ation of NS showed that: wakefulness, a litter dysarthria,bilateral peripheral facial palsy. There are low-grade of muscle toneand loss of tendon reflex on four limbs. There are analgesia anddysaphia below the wrists and ankles also. The muscular strength ofarms is 2 grade and 1 grade on legs. The p
34、athologic reflexes aredeficit. There are muscle pain or tenderness of the bilateral calves. 典型病例典型病例 某女，某女，30岁，因四肢无力、手和足麻木岁，因四肢无力、手和足麻木1天入院，大小便正常。天入院，大小便正常。神经系统检查：神志清楚，语言稍含糊。双侧周围性面神经麻痹。四肢肌张力低，神经系统检查：神志清楚，语言稍含糊。双侧周围性面神经麻痹。四肢肌张力低，腱反射消失，双上肢肌力腱反射消失，双上肢肌力2级、双下肢肌力级、双下肢肌力1级。双上肢腕以下和双下肢踝以下痛觉级。双上肢腕以下和双下肢踝以下痛觉
35、触觉轻度减退。病理反射（）。双侧腓肠肌压痛。触觉轻度减退。病理反射（）。双侧腓肠肌压痛。 对此患者，对此患者，请你判断是什么性质的瘫痪（上运动神经元或下运动神经元性瘫请你判断是什么性质的瘫痪（上运动神经元或下运动神经元性瘫痪）？痪）？提出应该完成那些主要的检查？提出应该完成那些主要的检查？最可能的诊断是什么？最可能的诊断是什么？应主要与哪些应主要与哪些疾病鉴别？疾病鉴别？ end Key points1. Albuminocytologic dissociation in CSF蛋白-细胞分离2.the main clinical features of GBS (AIDP) :acute o
36、r subacute onset; Generally symmetrical progressive lower motor neurons paralysis(LMNP) of limbs andcranial nerves; Weakness usually begins in the legs, more marked proximally than distally lower neurons lesion (hypotonia, hyporeflexia,wasting of affected muscles); The th, oculomotor nerves (, and )
37、, , and cranial nerves may be involved ; The respiratory muscles involved and palsy may cause respiratory failure in the sever cases .3. What changes of electrophysiologic in the GBS (AIDP)?The F waves and H reflex reduced or loss in the early stage of course but the NCV may not reveal marked slowing .4. How do differential diagnosis with other LMNP?
