EAN 2021 | Reduced safety factor in the pathogenesis of autoimmune and congenital myasthenic syndromes

Hakan Cetin, MD, PhD, Medical University of Vienna, Vienna, Austria, discusses the reduction of the safety factor seen in myasthenic syndromes. Impairment of the safety factor, measured as the ratio of the endplate potential to the threshold potential necessary to trigger an action potential, leads to dysfunction in neuromuscular transmission. Various factors can lead to a reduced safety factor in different myasthenic syndromes. In autoimmune syndromes, antibodies directed against synaptic proteins are often seen, such as in Lambert-Eaton myasthenic syndrome and myasthenia gravis. The safety factor is also impacted in congenital syndromes via mutations in synaptic membrane proteins. Dr Cetin explains how these factors lead to a reduced safety factor and why identifying the specific defect at fault has important clinical implications. This interview took place during the European Academy of Neurology 2021 congress.