急性炎症性脱髓鞘性多发性神经病 Acute Inflammatory Demyelinating Polyneuropathy, AIDP 王康 浙江大学医学院附属第一医院神经内科
急性炎症性脱髓鞘性多发性神经病 Acute Inflammatory Demyelinating Polyneuropathy, AIDP 王 康 浙江大学医学院附属第一医院神经内科
Introduction 又称吉兰一巴雷综合征 Symptoms nclude (Gui I lain-Barre Prickly, tngingse Syndrome,GBs),是以周围 Mucle 神经和神经根的脱髓鞘、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 自身免疫性疾病
Introduction ◼ 又称吉兰－巴雷综合征 (Guillain-Barré Syndrome,GBS )，是以周围 神经和神经根的脱髓鞘、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 自身免疫性疾病
历史回顾 a Landry 1859 10 patients with ascending paralysis ■ Guillain -Barre19162例 Guillain, Barre and strohl(1916) reported described two French soldiers with motor weakness. areflexia. and albuminocytological dissociation? in the cse蛋白细胞分离 是本病的特征
历史回顾 ◼ Landry 1859 10 patients with “ascending paralysis” ◼ Guillair-Barre 1916 2例 ◼ Guillain, Barré and strohl (1916) reported described two French soldiers with motor weakness, areflexia, and “albuminocytological dissociation” in the CSF 蛋白细胞分离 是本病的特征
历史回顾 In 1956. C miller fisher described a triad of acute op ohthalmoplegia, 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-Barre syndrome, and have different pathological subtypes
历史回顾 ◼ 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
流行病学 Worldwide incidence Incidence of typical GBS was between 0. 6 and 1.9/100 000 per year throughout the world China incidence incidence of 0 66 per 100 000 for all ages ■可发生于任何年龄,男女发病率相似,四季均可发病夏秋多见
流行病学 ◼ Worldwide incidence ◼ Incidence of typical GBS was between 0·6 and 1.9/100 000 per year throughout the world ◼ China incidence ◼ incidence of 0·66 per 100 000 for all ages ◼ 可发生于任何年龄，男女发病率相似，四季均可发病 夏秋多见
病理机制 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
病理机制 ◼ 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
Etiology Campylobacter Jejun Epstein-Barr Virus(EBv) Cytomegalovirus(CMV) ■HIV ■ Vaccinations 空肠肠弯曲菌
Etiology ◼ Campylobacter Jejuni ◼ Epstein-Barr Virus (EBV) ◼ Cytomegalovirus (CMV) ◼ HIV ◼ Vaccinations ◼ ··········· 空肠肠弯曲菌
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
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
临床表现 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
临床表现 ◼ 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
临床表现 Progressive ascending symmetrical weakness of the limbs Involvement of proximal and distal muscles Numbness and tingling in the hands and feet Back pa ain
◼ Progressive ascending symmetrical weakness of the limbs ◼ Involvement of proximal and distal muscles ◼ Numbness and tingling in the hands and feet ◼ Back pain 临床表现