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Publisher Summary Botulism is an acute neuroparalytic disease of humans and animals caused through the action of botulinum neurotoxins (BoNTs) acting at the neuromuscular junction (NMJ) of somatic nerves that innervate cranial and skeletal muscle. This results in the blockade of release of acetylcholine (ACh) with ensuing denervation and accompanying muscle paralysis and atrophy. Botulism is a presynaptic myasthenic neuromuscular syndrome exhibiting muscle weakness as its primary clinical sign. BoNTs are produced by heterogeneous group of clostridial bacteria that differ widely in genetic and metabolic characteristics. BoNTs are the most potent protein toxins and their toxicity depends on the route of entry into the human body. The diagnosis of botulism relies on the clinical findings that include prominent oculo-bulbar signs and laboratory detection of BoNT from appropriate specimens. The mainstay of treatment is intensive nursing care, with careful attention to respiratory failure, need for enteric feeding and cardiac arrest. Recovery from botulism is slow and tedious. The cellular mechanisms of BoNTs of activity were elucidated by new techniques and concepts, particularly advanced techniques for imaging tissue, electrophysiological methods, the theory of quantal release of acetylcholine at the synaptic membrane and genetic analyses, and structural analyses of the BoNTs. The pathophysiology of synaptic and postsynaptic effects from BoNT has been thoroughly studied in animal models. BoNTs affect synaptic activity of central neurons in tissue preparations at high doses or after direct intracranial injection. Motor systems throughout life within an organism have a dynamic capacity for adaptive remodeling and plasticity changes. |
