Electrophysiology of neuromuscular junction disease: an appreciation of the contributions of Edward H. Lambert.

As the muscle fiber action potential is "all or none" and is not of constant voltage recorded in volume from remote electrodes, there are serious limitations on assessment of the "safety factor" for neuromuscular transmission by stimulus-response recording from surface electrodes, or by single fiber EMG. Lambert's use of biopsied intercostal muscle with microelectrode recording of spontaneous and evoked end-plate potentials has permitted more accurate analysis of transmission disorders. His personal and collaborative studies on experimental myasthenia gravis, congenital myasthenic syndromes, amyotrophic lateral sclerosis, and the Lambert-Eaton myasthenic syndrome are reviewed. It is suggested that studies of acetylcholine receptor agonists require reevaluation as indicating inadequacies of present models.