1. 周围神经病 Peripheral Neuropathy Department of Neurology, The 2nd affiliated hospital, Harbin Medical University

2. Peripheral Neuropathy  Outline  Guillain-Barre Syndrome  Idiopathic facial palsy   rigeminal neuralgia

3. OUTLINE Anatomy Pathological Processes Clinical classifation Symptoms Investigation

4. Anatomy Peripheral nerves are made up of numerous axons bound together by three types of connective tissue—endoneurium, perineurium, epineurium. The vasa located in the epineurium provides the blood supply. (fig.1)fig.1 Peripheral nerve trunks contain myelinated and unmyelinated fibres Crinial nerve Spinal nerve

5. Pathological Processes Wallerian degeneration(fig.2)fig.2 Axonal degeneration Neuronal degeneration Segmental demyelination(fig.3)fig.3

6. Symptoms Sensory disturbance Motor deficits Tendon reflexes Autonomic disturbances Others

7. Investigation Nerve conduction velocity(NCV) Electromyography(EMG) Nerve biopsy Blood tests CSF examination

8. Trigeminal Neuralgia 三叉神经痛

9. Etiology and Pathology A facial pain syndrome of unknown cause. Demyelination(fig.4)fig.4

10. Clinical Features It develops in middle to late life. It consists of severe paroxysms electric- shock-like pain, usually in the V3 and V2 division of the trigeminal nerve (fig.5), lasting for several seconds or minutes each time.fig.5 Involvement of V1 division or bilateral disease occurs in less than 5% of cases.

11. Clinical Features Occurrence during sleep is rare. Painfree intervals may last for minutes to weeks, but long-term spontaneous remission is rare. Sensory stimulation of trigger zones about the cheek, nose, or mouth by touch, cold, wind, talking, or chewing can precipitate the pain.

12. Clinical Features Tic douloureux( 痛性抽搐 ) Course could be periodic Physical examination is normal

13. Diagnosis and Differential Diagnosis Diagnosis depending on the clinical features Differential Diagnosis Secondary trigeminal neuralgia Tooth ache Glosspharyngeal neuralgia( 舌咽神经痛 )

14. Treatment Drug is preferential AEDs: carbamazapine 0.1 bid po phenitoin, clonazepam Baclofen( 氯苯氨丁酸 ) VitaminB12 封闭疗法 : 无水酒精, 甘油 经皮半月神经节射频电凝疗法 手术治疗

15. 特发性面神经麻痹 Idiopathic Facial Palsy (Bell’s palsy)

16. Idiopathic Facial Palsy (Bell’s palsy) Anatomy(fig.6)fig.6 Etiology and pathogenesis Clinical features Diagnosis and differential diagnosis Treatment

18. Etiology and Pathology 尚未完全阐明，可有一些激发因素 受冷、病毒感染和自主神经不稳  神经 营养血管收缩而毛细血管扩张  组织水 肿  压迫面神经  面神经水肿和脱髓鞘， 严重者有轴突变性。

19. Clinical features Onset □ Occurs in any age, usually unilateral. □ Paralysis: Progresses over 3 to 72 hours □ Pain (50%): Near mastoid process □ Excess tearing (33%) □ Other: Hyperacusis; Dysgeusia

20. Clinical features Signs □ Facial weakness(fig.7)fig.7 Upper & Lower Unilateral Degree: Partial (30%); Complete (70%) □ Stapedius dysfunction (33%): Hyperacusis □ Lacrimation: mildly affected in some patients

21. □ Taste: No clinically significant changes in most patients. □ There should be no sensory loss in the face. □ Hunt syndrome. □ No abnormalities beyond the territory of the facial nerve.

22. Clinical features Prognosis better □ Incomplete paralysis □ Early improvement □ Slow progression □ Younger age

23. Clinical features Prognosis better □ Normal taste □ Electrodiagnostic tests normal 病后 10 天面神经出现失神经，恢复时间延长, 平均需要 3 个月。一般 1 ～ 2 月内可逐渐痊愈。

24. Diagnosis and diffential diagnosis 诊断：根据急性起病的周围性面瘫即可诊 断。 鉴别： 1 格林 - 巴利综合征 2 各种耳源性面神经麻痹 3 颅后窝的肿瘤或脑膜炎引起的周围性 面瘫

25. Treatment 原则：改善局部血液循环，减轻面神经水肿， 促进功能恢复。 Corticosteroids: prednisone 60mg qd po dexamethason VitaminB1,B12 Baclofen Rehabilitation Physiotherapy

26. 急性炎症性脱髓鞘性多发性神经病 (acute inflammatory demyelinating polyneuropathies, AIDP)

27. 概念 急性炎症性脱髓鞘性多发性神经病 (acute infla- mmatory demyelinating polyneuropathies, AIDP) 又称格林 - 巴利综合症 (Guillain-Barré Syndrome格林 - 巴利 GBS), 是以周围神经和神经根的脱髓鞘及小血 管周围淋巴细胞及巨噬细胞的炎性反应为病理 特点的自身免疫病。

28. Epidemiology Incidence: 0.6 to 1.9/100,000/year Male: Female = 1.25: 1 Peak ages: 16~25 years old 45~60 years old Certain forms of GBS appear to occur more frequently in certain areas of China.

29. Etiology and pathogenesis The precise cause is unclear. GBS often follows minor infective illness, inoculations or surgical procedures. Clinical and epidemiologic evidence suggest an association with preceding Campylobacter Jejuni(CJ) infection.(fig.8)fig.8 The pathogenesis resembles EAN Molecular mimicry

30. Clinical features GBS Prodrome It often follows 1-4 weeks after a respiratory infection or diarrhea. Campylobacter jejuni(CJ) has been particularly implicated as a cause of the diarrhea.

31. Clinical features Weakness: Most often symptomatic in legs Distribution: Proximal + Distal; Symmetric Severity: Quadriplegia in 30%; Bedbound another 30% Respiratory failure.

32. Clinical features Sensory: ususlly less marked than motor symptoms.  Paraesthesias: Initial symptom in 50%; Eventually occur in 70% to 90%  Pain  Loss: with classic glove-and-stocking pattern of sensory loss, but rarely occurs.

33. Clinical features Cranial nerve: Ⅶ, Ⅸ, Ⅹ facial weakness is present in 50% of cases. Autonomic dysfunction tachycardia, cardiac irregularitis, labile blood presure, disturbed sweating and so on. Monophase course

34. Clinical features Clinical classification  AIDP  AMAN  AMSAN  Fisher syndrome  Unclassifiable GBS

35. Investigations CSF: a characteristic abnormality, with increased protein concentration but a normal cell count. Eletrophysiologic studies  marked slowing of motor and sensory condu- ction velocity,  evidence of denervation and axonal loss.  F wave reflex is delayed or absent. Sural nerve biopsy: demyelination

36. Diagnostic criteria for GBS(1) Required for diagnosis Progressive weakness of more than one limb. Distal areflexia with proximal areflexia or hyporeflexia.

37. Diagnostic criteria for GBS(2) Supportive of diagnosis Progression for up to 4 weaks. Relatively symmetric deficits. Mild sensory involvement. Cranial nerve(especially Ⅶ )involvement. Recovery beginning within 4 weeks after progression stops. Autonomic dysfunction. No fever at onset. Increased CSF protein after 1 week. CSF white blood cell count  10/  l. Nerve conduction slowing or block by several weeks.

38. Differential diagnosis Hypokalemic periodic paralysis (Hopp) Poliomyelitis Myasthenia gravis(MG)

39. Treatment Assisting respiration: Patients who are seve - rely affected are best managed in ICU where facilities are available for monitoring and assis- ted respiration if necessary. Sometimes antibiotic is necessary for preventing respiratory tract’s infection. 肺活量 <20~25ml/kg, 动脉氧分压 <70mmHg Symptomatic therapy: The aim is to prevent such complications as respiratory failure or vascular collapse.

40. Treatment Preventing complications: 坠积性肺炎 褥疮 下肢深部静脉血栓、肺栓塞 肢体挛缩、畸形 吞咽麻痹 尿潴留 疼痛 焦虑及抑郁

41. Treatment Etiological therapy:  Plasma exchange(plasmapheresis)  Intravenous immunoglobulin: 0.4g /kg /d for 5 days  Corticosteroids: it has not been successful in acute GBS and can bring about adverse outcome. Rehabilitation

42. Prognosis The disorder is self-limiting,and improvement occurs over the weeks or months following onset. About 70-75% of patients recover completely, 25% are left with mild neurologic deficits, and 5% die, usually as a result of respiratory failure. The prognosis is poorer when there is evidence of preceding CJ infection.

43. 慢性炎症性脱髓鞘性多发性神经病 （ CIDP ）

44. CIDP 主要特点： 慢性进行性或慢性复发性病程。 起病隐袭，少有前驱感染。 进展期平均 3 月，可有复发 - 缓解。 对称肢体远端、近端无力，躯干肌、呼 吸肌、颅神经受累少见。 运动、感觉障碍同存。

45. CIDP 主要特点： CSF 可见蛋白 - 细胞分离。 NCV 、 F 波潜伏期均较 AIDP 重。 腓肠神经活检：炎症性节段性脱髓鞘与再 生共存， “ 洋葱皮样 ” 改变 ( fig.9 ) 。 fig.9 MRI ：神经增粗。 激素疗效肯定： prednison 100mg qd po(2-4W) 。