department of neurology, the 2nd affiliated hospital, kunming medical college yinfengqiong
DESCRIPTION
concept ( 1 ) 急性炎症性脱髓鞘性多发性神经病 (acute inflammatory demyelinating polyneuropathies, AIDP) 又称格林 - 巴利综合征 (Guillain-Barré Syndrome GBS)TRANSCRIPT
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Department of Neurology, The 2nd affiliated hospital, kunming Medical College
Yinfengqiong
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急性炎症性脱髓鞘性多发性神经病(acute inflammatory demyelinating
polyneuropathies, AIDP)
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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)
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concept(2)
pathological feature is demyelination( 脱髓鞘 ) of peripheral nerve
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concept(3) clinical feature is limbs symmetric
f laccid paralysis , peripheral anesthesia
Grave patients die of respiratory failure ( 呼吸衰竭 )
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气管插管 trachea cannula
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Etiology and pathogenesis(1)
The precise cause is unclear GBS often follows minor
infective illness or inoculation
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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
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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
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Segmental demyelination( 节段性多发性髓鞘脱失 )
pathologyNervr cell
axonal
muscle
Myelin sheath
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Clinical features(1)
GBS Prodrome It often follows 1-4 weeks after a
respiratory infection or diarrhea( 腹泻 ).
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Clinical features(2) Weakness(paralysis): Most often sy
mptomatic in legs Distribution: Proximal + Distal; Symmetric( 对称的 ) flaccid ( 弛缓性的 )
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Clinical features(3)
Severity: Quadriplegia ( 四肢瘫 )in 30%; Bedbound another 30% Respiratory failure( 呼吸衰竭 ) fatal factor cause death
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Clinical features(4)
Sensory( 感觉 ): ususlly less marked than motor symptoms. Paraesthesias( 外周感觉障碍 ):
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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
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Clinical features(6)
Cranial nerve( 颅神经 ): Ⅶ, ,ⅨⅩ facial weakness is present in 50
% of cases.
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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
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summary clinical feature : limbs symme
tric f laccid paralysis , peripheral anesthesia
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Clinical features(8)
Clinical classification( 分类 )) AIDP AMAN AMSAN Fisher syndrome Unclassifiable GBS
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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 )
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Investigations Eletrophysiologic studies( 电生理 ) marked slowing of motor and senso
ry conduction velocity,
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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 )
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Diagnostic criteria for GBS(1)
Required for diagnosis Progressive weakness of more than
one limb. Distal areflexia with proximal arefl
exia or hyporeflexia.
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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.
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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.
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Differential diagnosis Hypokalemic periodic paralysis
(Hopp) Poliomyelitis Myasthenia gravis(MG)
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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.
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Treatment Sometimes antibiotic is necessar
y for preventing respiratory tract’s infection.
肺活量 <20﹣25ml/kg, 动脉氧分压 <70mmHg
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Treatment Symptomatic therapy( 对 症 治 疗 ):
The aim is to prevent such complications as respiratory failure or vascular collapse.
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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
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气管插管 trachea cannula
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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
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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
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TreatmentCorticosteroids( 皮质类固醇 ): it ha
s not been successful in acute GBS and can bring about adverse outcome.
Rehabilitation( 康复 )
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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.
.
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Prognosis The prognosis is poorer when
there is evidence of preceding CJ infection