Abstract
The neuromuscular junction (NMJ) is uniquely sensitive to the effects of neurotoxins. Unlike the blood-brain barrier, which protects the brain and spinal cord, and the blood-nerve barrier, which protects peripheral nerve, there are no barriers to protect the NMJ from the deleterious effects of these agents. Several forms of neurotoxins are directed against the NMJ. Many occur as natural substances of plants or animals, other result from the actions of widely prescribed pharmaceutical compounds, and still others are environmental hazards. In nearly all instances of NMJ neurotoxicity, there is a reduction in the safety factor of neuromuscular transmission by one of several mechanisms. These neurotoxins may affect either the presynaptic or the postsynaptic elements of the NMJ. The clinical features of these neurotoxins are quite varied; many have associated toxicity of other parts of the central, peripheral, or autonomic nervous systems. Many will have other systemic effects as well. While feared as the purveyor of morbidity and mortality, many of these neurotoxins have led to significant advances in our understanding of the molecular mechanisms of pharmacology and physiology and their associated diseases. For example, the recognition that α-bungarotoxin binds to the acetylcholine receptor (AChR) advanced our knowledge of the diagnosis and treatment of myasthenia gravis (MG) (1). Worldwide, the most common neurotoxicity of the NMJ results from envenomation. Of more concern to the clinical neurologist are those situations that result from the direct effects of various pharmacologic agents routinely used in the practice of medicine that produce significant aberrations of neuromuscular transmission in susceptible individuals. The potential for environmental intoxication has been limited by the stringent regulation of federal and international regulatory agencies.
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Howard, J.F. (2003). Toxic Neuromuscular Transmission Disorders. In: Kaminski, H.J. (eds) Myasthenia Gravis and Related Disorders. Current Clinical Neurology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-341-5_15
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