Abstract
Abstract Myotonia congenita (MG) and various ion channel myotonias all fall under the umbrella of nondystrophic myotonias (NDMs) and are distinct from myotonic dystrophy because they are caused by dysfunction of key skeletal muscle ion channels alone without any other systemic effects. NDMs are characterized by electrical myotonia, “defined as the prolongation of skeletal muscle relaxation time following sudden voluntary contraction or external mechanical stimulation.” Hyperexcitability of muscles is central to all of these disease states. The NDMs are divided into two groups: the chloride channelopathies (myotonia congenita) and the sodium channelopathies (paramyotonia congenita [PMC] and the potassium-aggravated myotonias [PAMs]). In contrast, acquired neuromyotonia is an autoimmune disease where approximately 40% of affected individuals have antibodies to voltage-gated potassium channels (VGKCs) that directly affect the neuromuscular junction.
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