Burrill Bernard Crohn at Mount Sinai Hospital in 1907. He was promoted to head of gastroenterology in 1920, the same year he joined the staff of Columbia University. Working with colleagues at Mount Sinai Hospital, Crohn identified fourteen patients whose symptoms and intestinal abnormalities discovered at surgery, while consistent with each other, did not fit any previously identified disease. Crohn, along with Leon Ginzburg and Gordon Oppenheimer, prepared the classic paper describing this new condition. This paper was read at a professional meeting in May 1932 and published in the "Journal of the American Medical Association" in October 1932. The title of the
Krabbe disease may be caused by a lack of active saposin A (a derivative of prosaposin). The buildup of unmetabolized lipids adversely affects the growth of the nerve's protective myelin sheath (the covering that insulates many nerves) resulting in demyelination and severe progressive degeneration of motor skills. As part of a group of disorders known as leukodystrophies, Krabbe disease results from the imperfect growth and development of myelin. GALC deficiency also results in a build-up of a glycosphingolipid called psychosine, which is toxic to oligodendrocytes, a type of non-neuronal cell found in the nervous system, collectively termed neuroglia. The disease may be