The condition known as amyotonia congenita, first described by Oppenheim in 19001 is frequently referred to as Oppenheim's disease. In 1903, Batten2 described the same condition without reference to Oppenheim's work, under the name of infantile type of myopathy. Kundt,3 Collier and Wilson,4 Rothman,5 Chene,6 Griffith,77 Cassirer8 and Thorspeken9 have all made valuable contribution to the literature in this subject. Marburg,10 Kaumheimer11 and Foot12 have made exhaustive studies of the histologic changes in the muscles and in the nervous systems. Until recently about 136 cases have been reported in the literature, but in many of these the diagnosis has been questioned by other contributors. Of this number thirty cases were reported by Americans, twenty-six by Italians, thirty-three by Germans, twenty-one by Englishmen, seventeen by Frenchmen, three by Belgians, two by the Dutch, one case by a Swiss, and