It looks that the component of inflammasomes with interest in CMS pathogenesis is NLRP3 and its ability to recruit the inflammasome-adaptor protein ASC and secondary activation of caspase-1 and caspase-11, which become crucial steps in the process of pro-inflammatory cytokines interleukin (IL)-1β and IL-18 releasing and maturation, and has the ability to initiate apoptosis of cardiomyocytes, activation of cardiofibroblasts, and induction of myocardial fibrosis [31,32,33]. Here, NLRP3 is linked to congenital myasthenic syndrome.