In contrast to this GLDN‐associated lethal congenital arthrogryposis and congenital myopathy Compton‐North, resulting from homozygous CNTN1 variants, are only associated with a severe phenotype, leading to death early in life, mostly after the first days, almost independent of the type of variant.15, 22. The gene discussed is CNTN1; the disease is congenital myopathy with cores.