Definition:
Krabbe disease (KD) is a rare disorder in which the myelin coating on the nerves breaks down, causing brain cells to be destroyed. The inherited disorder is caused by a lack of the enzyme galactocerebrosidase, which is needed for myelin metabolism. Mental and motor development are affected, and muscle weakness, deafness and blindness can also occur. There is currently no cure for the disease, which is most commonly found in infants. These children often die before age 2, but infusions of umbilical cord blood stem cells before onset or bone marrow transplant early in the disease can prolong life.
Also Known As: Krabbe Leukodystrophy, globoid cell leukodystrophy, Krabbe Syndrome
Alternate Spellings: Krabbe's, Krabbé
Common Misspellings: Crabby's
