Department of Neurology, The 2nd affiliated hospital, kunming Medical College

Yinfengqiong

急性炎症性脱髓鞘性多发性神经病(acute inflammatory demyelinating

polyneuropathies, AIDP)

concept (1)

急性炎症性脱髓鞘性多发性神经病 (acute inflammatory demyelinating poly

neuropathies, AIDP) 又称格林 - 巴利综合征 (Guillain-Barré Syndrome GBS)

concept(2)

pathological feature is demyelination( 脱髓鞘 ) of peripheral nerve

concept(3) clinical feature is limbs symmetric

f laccid paralysis ， peripheral anesthesia

Grave patients die of respiratory failure ( 呼吸衰竭 )

气管插管 trachea cannula

Etiology and pathogenesis(1)

The precise cause is unclear GBS often follows minor

infective illness or inoculation

Etiology and pathogenesis(2) Clinical and epidemiologic evidence s

uggest an association with preceding Campylobacter Jejuni(CJ) ( 空肠弯曲菌 )infection.

The pathogenesis resembles EAN Molecular mimicry

Etiology and pathogenesis(3)

病 原体 感 染pathogen infection

自身免疫性疾病

Autoim

mune disease

体液性免疫 humoral immunity

细胞性免疫Cellular immunity

Segmental demyelination( 节段性多发性髓鞘脱失 )

pathologyNervr cell

axonal

muscle

Myelin sheath

Clinical features(1)

GBS Prodrome It often follows 1-4 weeks after a

respiratory infection or diarrhea( 腹泻 ).

Clinical features(2) Weakness(paralysis): Most often sy

mptomatic in legs Distribution: Proximal + Distal; Symmetric( 对称的 ) flaccid ( 弛缓性的 )

Clinical features(3)

Severity: Quadriplegia ( 四肢瘫 )in 30%; Bedbound another 30% Respiratory failure( 呼吸衰竭 ) fatal factor cause death

Clinical features(4)

Sensory( 感觉 ): ususlly less marked than motor symptoms. Paraesthesias( 外周感觉障碍 ):

Clinical features(5) Sensory( 感觉 ):

Pain Loss( 感觉缺失 ): with classic glo

ve-and-stocking pattern ( 手套 -袜套型 ) of sensory loss

Clinical features(6)

Cranial nerve( 颅神经 ): Ⅶ, ,ⅨⅩ facial weakness is present in 50

% of cases.

Clinical features(7)呼吸肌麻痹respiratory paralys

is

肺部感染Lung infection

心力衰竭heart failure

main cause of death

summary clinical feature : limbs symme

tric f laccid paralysis ， peripheral anesthesia

Clinical features(8)

Clinical classification( 分类 )) AIDP AMAN AMSAN Fisher syndrome Unclassifiable GBS

Investigations

CSF( 脑脊液 ): a characteristic abnormality, with increased protein concentration but a normal cell count.

Protein-cell isolation( 蛋白 - 细胞分离 )

Investigations Eletrophysiologic studies( 电生理 ) marked slowing of motor and senso

ry conduction velocity,

Investigations evidence of denervation and axonal l

oss. F wave reflex is delayed or absen

t. Sural nerve biopsy( 腓肠神经活检 ): demyelination ( 脱髓鞘 )

Diagnostic criteria for GBS(1)

Required for diagnosis Progressive weakness of more than

one limb. Distal areflexia with proximal arefl

exia or hyporeflexia.

Diagnostic criteria for GBS(2)Supportive of diagnosis Progression for up to 4 weaks. Relatively symmetric deficits. Mild sensory involvement. Cranial nerve(especially )involvement.Ⅶ Recovery beginning within 4 weeks afte

r progression stops.

Diagnostic criteria for GBS(2)

Autonomic dysfunction. No fever at onset. Increased CSF protein after 1 week. CSF white blood cell count10/l. Nerve conduction slowing or block

by several weeks.

Differential diagnosis Hypokalemic periodic paralysis

(Hopp) Poliomyelitis Myasthenia gravis(MG)

Treatment Assisting respiration( 辅助呼吸 ): Patie

nts who are seve -rely affected are best managed in ICU where facilities are available for monitoring and assis- ted respiration if necessary.

Treatment Sometimes antibiotic is necessar

y for preventing respiratory tract’s infection.

肺活量 <20﹣25ml/kg, 动脉氧分压 <70mmHg

Treatment Symptomatic therapy( 对 症 治 疗 ):

The aim is to prevent such complications as respiratory failure or vascular collapse.

缺氧呼吸肌麻痹

咳嗽无力、排痰困难气管切开

预防感染保持呼吸道通畅

肺部感染

心力衰竭中枢性

周围性 死亡

病理与临床

气管插管 trachea cannula

Treatment Preventing complications( 防止并发症 ): 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

Treatment Etiological therapy( 病因治疗 ): Plasma exchange( 血浆置换 ) (plasmap

heresis) Intravenous immunoglobulin( 静 脉 注射免疫球蛋白 ): 0.4g /kg /d for 5 days

TreatmentCorticosteroids( 皮质类固醇 ): it ha

s not been successful in acute GBS and can bring about adverse outcome.

Rehabilitation( 康复 )

Prognosis The disorder is self-limiting,and impro

vement occurs over the weeks or months following onset. About 70-75% of patients recover completely, 25% areleft with mild neurologic deficits, and5%die, usually as a result of respiratoryfailure.

.

Prognosis The prognosis is poorer when

there is evidence of preceding CJ infection