神经肌接头病-重症肌无力Lambert-Eaton 综合征PPT.pptVIP

Myasthenia Gravis (MG) Treatment thymectomy should be performed in patients under 60 years of age. usually leads to symptomatic benefit or remission However, its beneficial effect may not be evident immediately. 神经肌接头病 (Disorders of neuromuscular transmission)  重症肌无力 Lambert-Eaton 综合征 Dep. of Neurology The 2nd Hospital Harbin Medical University Neuromuscular Disorders  Definition The diseases of neuromuscular junction (NMJ) describes a sets of disease caused by circulating factors such as neurotoxins or autoantibodies which bind with high affinity to specific proteins at the NMJ and disturb the neuromuscular transmission. 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. 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). 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. Myasthenia gravis ( MG) 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. 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)Etiology The au