The Floppy Infant

In 1900, Oppenheim¹ described 8 patients who had a nonfamilial disease present at birth characterized by hypotonia, absent deep tendon reflexes, and muscular weakness, that gradually improved with age. Oppenheim postulated that this disease was the result of a temporary abnormality of muscle. Eight years later, Collier and Wilson² gave this "disease" the name "amyotonia congenita." Subsequently, because of Oppenheim's reputation on the Continent, almost all cases of hypotonia unexplainable by some other specific disease were designated as instances of "Oppenheim's disease" or "amyotonia congenita." Oppenheim had no pathological confirmation that the condition he described was different from other conditions causing hypotonia, and, in fact, subsequent investigations^3-4 have proved that the pathology of many cases of "amyotonia congenita" is the same as that of Werdnig-Hoffmann's disease, namely a degeneration of anterior horn cells of the spinal cord. It appears likely that various diseases have been grouped under