ABSTRACT

Myasthenia gravis (MG) is a disease of the neuromuscular junction. The symptoms and signs are the result of a fluctuating and partly permanent weakness of the voluntary muscles caused by a defect in neuromuscular transmission. Nonimmunological variants of myasthenia have been recognized, commonly referred to as congenital myasthenia although the first signs sometimes appear in infancy or adolescence. MG may be considered as a prototype of an autoimmune disease with an antibody mediated attack on surface receptors of transmitters. Although Willis’ description is that of a fluctuating weakness, a severe weakness of the extremities combined with normal ability to speak is unusual in myasthenia. The name “MG pseudo-paralytica” stems from F. Jolly who described the failure of sustained tetanic contraction by repetitive stimulation of the nerve. Jolly also suggested the use of veratrin and physiostigmine, which evoke “the opposite of a myasthenic, namely a myotonic reaction”.