Description Of Krabbe Disease

Krabbe disease is a disorder inherited from parents, which destroys the myelin coat of nerve cells throughout the nervous system and the process is called demyelination. Demyelination inhibits the transmission of nerve impulses and that hinders the communication of the brain and the body and that results in disability (Mayo Foundation for Medical Education and Research, 2016). Dr. Knud. H.Krabbe diagnosed this disorder in 1916 (Arizona Board of Regents, 2015). Krabbe disease is a body recessive disorder that results from lack of enzyme galactorebrosidase or simply GALC, which is necessary to break down and metabolize toxic substances in nerve tissues (YouTube, 2016). A lack in GALC results in toxic built-up for example psychosine which is a destructive toxin that accumulates in the brain, as it begins to build-up in the nerve tissue it destroys the myelin sheath which covers the nerves (YouTube, 20160.
Krabbe disease is also known as Globoid Cell Leukodystrophy, the name globoid cell Leukofystrophy arose from the characteristic pathology of Krabbe disease, where macrophage accumulates high level of galactolipids because of lack in GALC activity (Mayo Foundation for Medical Education and Research, 2016). Macrophages are specific type of cells which are differentiated normal cells, hence they are called globoid