急性炎症性脱髓鞘性多发性神经病 Acute Inflammatory Demyelinating Polyneuropathy, AIDP

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1 急性炎症性脱髓鞘性多发性神经病 Acute Inflammatory Demyelinating Polyneuropathy, AIDP
浙江大学医学院附属第一医院

2 Introduction Landry -Landry's paralysis 1859 Guillair-Barre 1916 2例
Landry reported an acute, ascending, predominantly motor paralysis with respiratory failure, leading to death Guillair-Barre 例 Guillain, Barre and strohl (1916) reported a benign polyneuritis with albuminocytologic dissociation in the CSF (raised concentration of CSF protein but a normal cell count) 蛋白细胞分离是本病的特征 1859年Landry报道一例急性、上升性，主要累及运动功能并累及呼吸及而导致死亡的病例； 1916年Guillair、Barre及strohl等报告一例良性多发性神经炎患者，脑脊液表现为蛋白细胞分离

3 Landry Strohl Guillain Barre

4 Introduction In 1956, C Miller Fisher described a triad of acute ophthalmoplegia, ataxia, and areflexia, now known as Fisher’s syndrome During the past 15 years, GBS has become clear that this clinical picture, now called Guillain-Barré syndrome, and have different pathological subtypes 1956年Miller Fisher 报告一例表现为“眼肌麻痹、共济失调和腱反射消失”三联征—即所谓的Miller Fisher综合征 过去的15年的研究显示，GBS的临床情况已经明确，而且其存在不同的病理类型。

5 Epidemiology Worldwide incidence China incidence
0.6 -4/ per year throughout the world China incidence 0.66 per for all ages 可发生于任何年龄，男女发病率相似，夏秋多见 世界范围的GBS的发生率约0.6-4/10万人 中国的发生率约0.66/10万

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7 临床表现：中国 儿童和青少年，夏初。 EMG：轴索损害， AMAN。
EMG符合AMAN的为65％，符合AIDP的为24％。 66％有CJ抗体，42％有GM1抗体，其他神经节苷脂抗体为17－26％。与西方国家不同，GM1抗体与AMAN或AIDP无关。近来发现AMAN与GD1a抗体相关密切。

8 临床表现：中国 病理： EMG不能预测病理。 AMAN：IgG和补体在轴索周围沉积，巨噬细胞侵入轴索周围间隙，严重者有轴索变性。
AIDP：IgG和补体在髓鞘外沉积，巨噬细胞也在髓鞘外，“撕开”髓鞘。 AMSAN：感觉轴索比运动轴索损害重。 EMG不能预测病理。

9 Pathogenesis and Pathophysiology
The cause of this syndrome is unknown, but it is generally viewed to be an autoimmune response to a bacterial or viral infection. 病因尚未完全阐明 GBS的发病机制仍不明确，目前认为是机体对细菌、病毒的免疫反应所致

10 Etiology Vaccinations ··········· Campylobacter Jejuni
Epstein-Barr Virus (EBV)  Cytomegalovirus (CMV) HIV Vaccinations ··········· 病因： 空肠弯曲菌 EB病毒 巨细胞病毒 艾滋病病毒 疫苗接种 空肠肠弯曲菌

11 Pathogenesis and Pathophysiology
An acute immune-mediated polyneuropathy , component of pathogen was similar with myelin sheath of peripheral nerve 与感染有关的自身免疫性疾病, 病原体某些成分与周围神经的髓鞘成分相似

12 Pathophysiology 主要病理特点(principal characteristic of pathology )
节段性脱髓鞘(segmental demyelization) 小血管周围炎性细胞浸润

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16 Clinical manifestations
多数患者有前驱症状(起病前1~3周） 呼吸道感染症状 喉痛、鼻塞、发热 消化道症状 腹泻、呕吐

17 Clinical manifestations
Progressive ascending symmetrical weakness of the limbs Involvement of proximal and distal muscles Numbness and tingling in the hands and feet Back pain 双下肢无力（80%）-驰缓性瘫痪对称性 ,少数——纯感觉异常 瘫痪(paralysis )：双下肢→上肢、颅神经 对称性驰缓性瘫痪 腱反射减弱或消失(areflexia) 1~2周内高峰 Landry上升性麻痹：（10~14天内） 无力从下肢躯干 上肢、颅神经 感觉障碍 深、浅感觉障碍 感觉异常：麻木、蚁走感、针刺感、烧灼感等伴有肌肉酸痛 “手套—短袜”型的感觉减退或缺失（主观感觉障碍很常见，客观感觉障碍却很少见）

18 Clinical manifestations
Depressed or absent reflexes Involvement of cranial nerves (facial nerves most commonly involved) Respiratory failure(involved respiratory muscles) Progression to peak disability in 4 wk autonomic nerve symptom 呼吸肌麻痹（主要死因） 颅神经功能障碍 双侧周围性面瘫最常见 其他：自主神经症状(autonomic nerve symptom) 血管舒缩功能障碍 汗腺活动功能障碍 少汗或多汗 可影响心脏：心动过速 高血压、低血压 括约肌功能障碍罕见 单相病程：4周左右开始恢复

19 Assessment Cerebrospinal fluid
Increased protein usually after 7 to 10 days. While some protein is normally present, an increased amount without an increase in the number of white blood cells may indicate GBS 蛋白细胞分离 蛋白升高起病后的7-10天左右，有时蛋白可以表现正常，但是细胞数增高同样提示GBS

20 Assessment Nerve conduction velocity test
Nerve conduction studies are a dependable and early diagnostic indicator of GBS. shows demyelization and damage to the nerve sheath F反应、H反射异常 PL延长，NCV减慢 传导阻滞现象，伴或不伴有波幅降低

21 Assessment Electrocardiogram (EKG) 腓肠神经活检
节段性脱髓鞘 小血管周围炎性细胞浸润 Electrocardiogram (EKG) May show abnormalities in cardiac rhythm 心律失常

22 Subtypes of GBS 经典型 AIDP Fisher综合症(Miller Fisher syndrome )： 脑神经型
三联征-“眼外肌麻痹、 共济失调、腱反射消失”，还有中枢神经系统损害 It was thought to be a variant of GBS and comprise complete ophthalmoplegia with ataxia and are flexia 脑神经型

23 Subtypes of GBS 轴突型 急性感觉性多发性神经炎（ASP） 急性全自主神经病（APN） 假性肌营养不良 复发型
纯运动型（AMAN） 运动 感觉 型 （AMSAN ） 急性感觉性多发性神经炎（ASP） 急性全自主神经病（APN） 假性肌营养不良 复发型

24 Diagnosis Required for diagnosis
Progressive weakness of one or more limb Distal areflexia with proximal areflexia or hyporeflexia 诊断的必须条件： 进行性加重的一个或多个肢体无力 远端腱反射消失，近端腱反射减弱或消失

25 Diagnosis Supportive diagnosis
Progression of symptoms over days to 4 wk Relative symmetry of deficits Mild sensory involvement Cranial nerve involvement (especially VII) Recovery beginning within 4 wk 支持诊断： 症状进展数天或4周 相对对称性病变 轻度感觉受累 颅神经受累特别是面神经 症状进展停止后病情开始恢复，多在4周内

26 Diagnosis Supportive diagnosis Autonomic dysfunction No fever
Increased CSF protein after 1 wk CSF white blood cell count ≤ 10/μL Nerve conduction slowing or blocked by several weeks 支持诊断 自主神经功能障碍 起始无发热 起病1周后脑脊液蛋白升高 脑脊液细胞数小于≤ 10/μL 神经传导速度减慢或阻滞

27 Diagnosis Against diagnosis Significant asymmetric weakness
Bowel or bladder dysfunction at onset or persistent CSF white blood cell count > 50 or PMN count > 0μL Well-demarcated sensory level 不支持诊断 显著的不对称性无力 疾病开始吃出现肠道和膀胱功能障碍，或持续存在 脑脊液细胞数> 50 或多核单细胞计数大于0 显著的感觉缺失平面

28 Diagnosis Excluding diagnosis
Isolated sensory involvement, without weakness Another polyneuropathy that explains clinical picture 排除诊断： 孤立性感觉受累无肌无力 其他多发性神经病变可解释临床症状

29 Differential diagnosis
Acquired hypokalemia Botulism Myasthenia gravis Periodic paralysis Poliomyelitis Polymyositis Tick paralysis Diphtheria Transverse myelitis Heavy metal (lead and arsenic poisoning) 鉴别诊断： 获得性低钾性血症 肉毒素中毒 周期性瘫痪 脊髓灰质炎 多发性肌炎 蜱性麻痹 白喉 横断性脊髓炎 重金属中毒如铅、砒霜等

30 Differential diagnosis
低钾性周期性瘫痪(hypokalemic periodic paralysis) 无病前感染史，常有发作史 无感觉和脑神经损害，脑脊液正常 电解质（血钾<3.5)及心电图检查异常 补钾治疗有效

31 Differential diagnosis
重症肌无力(myasthenia gravis) 骨骼肌 病态易疲劳性、波动性 no sensory symptoms tendon reflexes are unimpaired

32 Differential diagnosis
脊髓灰质炎(poliomyelitis) 早期出现括约肌功能障碍 无感觉障碍  Fever, meningeal symptoms, early pleocytosis, and purely motor and usually asymmetrical areflexic paralysis.

33 Differential diagnosis
急性脊髓炎（acute myelitis） The immediate problem is to differentiate GBS from acute spinal cord disease (marked by sensorimotor paralysis below a level on the trunk and sphincteric paralysis).

34 Clinical management General treatment 一般治疗 Immunotherapy 免疫治疗

35 General treatment 辅助呼吸 保持呼吸道通畅 密切观察，测肺活量20ml/kg→ICU必要时气管插管，使用呼吸器
预防呼吸道感染 翻身、拍背、稀化痰液、吸痰

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37 General treatment 预防并发症(prevention of complication) 坠积性肺炎 褥疮 血栓性静脉炎
防止肢体挛缩 尿路感染

38 General treatment 预防并发症(prevention of complication) 合理的正压通气、吸出分泌物
经常翻身，保持床单平整 皮下应用肝素 有临床指征时，应用广谱抗生素等

39 General treatment 对症处理 必要时心电监护 高血压—小剂量β受体阻滞剂 低血压—补液 心动过速—通常不需要治疗
心动过缓—阿托品 疼痛—卡马西平

40 Immunotherapy 机理 抑制免疫反应，去除致病因子对神经损害，使髓鞘有时间再生 方法 血浆置换 静脉注射免疫球蛋白 皮质醇激素治疗

41 Plasma exchange The usefulness of plasma exchange in the evolving phase of GBS. In patients who are treated within 2 weeks of onset, there is a reduction in the period of hospitalization in the length of time that the patient requires mechanical ventilation. However, when plasma exchange is delayed for 2 weeks or longer after the onset of the disease, the procedure has, with a few notable exceptions, been of little value. 起病2周的患者血浆置换能后缩短住院时间； 起病超过2周的患者血浆置换的效果不佳

42 Plasma exchange 血浆置换 机制：去除血浆中致病因子，可明显缩短病程，使用越早，疗效越好， 专用设备，价格昂贵
适用于急性进行性加重的GBS 用法：40ml/kg 禁忌症：严重感染， 心律失常、心功能不全， 凝血功能障碍

43 Intravenous immunoglobulin
静脉注射免疫球蛋白 尽早施行 用法：0.4g/(kg.d)×5天 禁忌症：免疫球蛋白过敏，先天性IgA缺乏 PE 和IVIG不必联合应用

44 Corticosteroids 皮质类固醇 有争议 理论上合理 研究表明无效 经验：青年人大剂量早期使用

45 Corticosteroids The value of corticosteroids in the treatment of GBS has been disputed for decades. Although corticosteroids can no longer recommended as routine treatment for acute GBS. We have observed a few instances in which the intravenous administration with high-close corticosteroids seemingly halted the progress of the disease. 糖皮质激素治疗 现在仍然存在争论 目前糖皮质激素已经不推荐为GBS的常规用药，但是研究表明大剂量冲击治疗能阻止疾病的进展

46 Prognosis Prognosis The majority of patients recover completely or nearly completely In about 10 percent of patients, the residual disability is pronounced 预后 大部分病人能完全或几乎完全康复，约10%的病人遗留残疾

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48 预后 80%患者恢复完全 死亡率大约5%(呼吸肌麻痹)

49 谢谢!