神经肌接头病-重症肌无力Lambert-Eaton 综合征
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Neuromusuclar Junction (NMJ) Physiology
the nerve AP reaches the nerve terminal which inflated and without myelin and leads to the opening of calcium channel and release of ACh into the synaptic cleft by exocytosis.
Epidemiology
It is the prototypic neuromuscular disorders with an incidence of 80-200 per million and prevalence about 500 per million.
In China, it is estimated that 0.6 million people were diagnosed as MG and most of them lives in the South of China.
Neuromusuclar Junction (NMJ)
Physiology
1/3 of the ACh diffuses rapidly to the postsynaptic membrane and binds to the AChRs, leading to opening of the AChR-associated cation channel and depolarization called the end-plate potential (EPP).
Etiology
The autoimmune origin of MG is proposed long before it was established in 1973 by the direct evidence provided by Patrick and Lindstrom, who have immunized rabbit with affinity-purified Torpedo AChR with CFA and reproduced the animal models representing human MG (EAMG).
It had been a life-threatening disease before 1970’s, though nowadays the incidence of death has been greatly reduced to about 0.2%.
Myasthenia Gravis (MG)
If the EPP exceeds certain threshold, voltage gated sodium channel at the postsynaptic membrane are opened.
This generates the muscle action potential (CMAP) that propagates along the muscle fiber and activates contraction.
Neuromusuclar Junction (NMJ) Physiology
Another 1/3 of the ACh is hydrolyzed by cholinesterase (ChE).
The remaining 1/3 of the ACh is recaptured by the presynaptic membrane.
Although the primary antiself event being unclear, thymus appears to be the place where it initiates.
The general opinion is that virus infection or other nonspecific factors invades the thymus in genetically predisposed individuals leading to the development of MG.
The AChR is the autoantigen.
Myasthenia Gravis (MG)
Etiology
The presence of anti-AChR Abs can be demonstrated in 80%-90% of MG patients.
There is a 3:1 female-male ratio for patients who develop MG in early adult life.
Overall, the above makes MG fulfills the criteria for autoimmune diseases.
Myasthenia Gravis (MG)
Etiology
Most of the patients with MG have abnormalities in the thymus, e.g. thymic hyperplastic or thymoma.
acquired MG is an antibody and complementmediated, T cell-dependent autoimmune disease leading to a defect in neuromuscular transmission.
Myasthenia gravis ( MG)
重症肌无力 (Myasthenia Gravis, MG)
概念 病因及发病机制 病理 临床表现 诊断及鉴别诊断 治疗
Myasthenia gravis (MG)
Definition
MG was originated from Latin, meaning very severe Leabharlann Baidueakness.
the nerve AP reaches the nerve terminal which inflated and without myelin and leads to the opening of calcium channel and release of ACh into the synaptic cleft by exocytosis.
Epidemiology
It is the prototypic neuromuscular disorders with an incidence of 80-200 per million and prevalence about 500 per million.
In China, it is estimated that 0.6 million people were diagnosed as MG and most of them lives in the South of China.
Neuromusuclar Junction (NMJ)
Physiology
1/3 of the ACh diffuses rapidly to the postsynaptic membrane and binds to the AChRs, leading to opening of the AChR-associated cation channel and depolarization called the end-plate potential (EPP).
Etiology
The autoimmune origin of MG is proposed long before it was established in 1973 by the direct evidence provided by Patrick and Lindstrom, who have immunized rabbit with affinity-purified Torpedo AChR with CFA and reproduced the animal models representing human MG (EAMG).
It had been a life-threatening disease before 1970’s, though nowadays the incidence of death has been greatly reduced to about 0.2%.
Myasthenia Gravis (MG)
If the EPP exceeds certain threshold, voltage gated sodium channel at the postsynaptic membrane are opened.
This generates the muscle action potential (CMAP) that propagates along the muscle fiber and activates contraction.
Neuromusuclar Junction (NMJ) Physiology
Another 1/3 of the ACh is hydrolyzed by cholinesterase (ChE).
The remaining 1/3 of the ACh is recaptured by the presynaptic membrane.
Although the primary antiself event being unclear, thymus appears to be the place where it initiates.
The general opinion is that virus infection or other nonspecific factors invades the thymus in genetically predisposed individuals leading to the development of MG.
The AChR is the autoantigen.
Myasthenia Gravis (MG)
Etiology
The presence of anti-AChR Abs can be demonstrated in 80%-90% of MG patients.
There is a 3:1 female-male ratio for patients who develop MG in early adult life.
Overall, the above makes MG fulfills the criteria for autoimmune diseases.
Myasthenia Gravis (MG)
Etiology
Most of the patients with MG have abnormalities in the thymus, e.g. thymic hyperplastic or thymoma.
acquired MG is an antibody and complementmediated, T cell-dependent autoimmune disease leading to a defect in neuromuscular transmission.
Myasthenia gravis ( MG)
重症肌无力 (Myasthenia Gravis, MG)
概念 病因及发病机制 病理 临床表现 诊断及鉴别诊断 治疗
Myasthenia gravis (MG)
Definition
MG was originated from Latin, meaning very severe Leabharlann Baidueakness.