Myasthenia Gravis and Other Myasthenic Syndromes
In this group of disorders transmission at the motor end-plates is abnormal. There are a number of possible abnormalities in motor endings which might result in inadequate neuromuscular transmission. These include abnormal synthesis of acetylcholine (ACh), defective release of ACh quanta, abnormalities in the neuromuscular cleft itself, abnormal post-synaptic ACh receptors and abnormal breakdown of ACh because of defective cholinesterase. In a number of congenital myasthenic syndromes discrete abnormalities in various components of the post-synaptic ACh receptor have been found, and in the Lambert—Eaton myasthenic syndrome release of ACh vesicles is abnormal (Table 12.1).
KeywordsMEPP Amplitude Decremental Response Congenital Myasthenic Syndrome Myasthenic Crisis Repetitive Nerve Stimulation
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