The first German family with autosomal dominant Emery-Dreifuss syndrome (EDS) is 
described, with electrophysiologic and myopathologic results providing evidence 
of a primary neurogenic disease. According to classification of the scapulo 
peroneal syndrome without cardiomyopathy, we conclude that there are two 
variants of EDS: one myopathic, the other neurogenic in origin. Therefore, the 
term Emery-Dreifuss muscular dystrophy should be avoided. Instead, each case of 
EDS should be classified as myopathic or neurogenic with X chromosome recessive 
or autosomal dominant inheritance.