(Refer: Nelson’ss textbook of pediatrics, 19th edition, pg no: 2071-2072) Krabbe disease (KD)/globoid cell leukodystrophyAutosomal recessive neurodegenerative disorder. Characterized by severe myelin loss and the presence of globoid bodies in the white matter. Deficiency of lysosomal enzyme galactocerebroside β- galactosidase (galactosylceramidase). Accumulation of galactocerebroside causes activation of an alternative catabolic pathway removes a fatty acid from this molecule, generating galactosylphingosine. In patients with KD, galactocerebroside cannot be metabolized during the normal turnover or myelin because of deficiency of galactocerebroside β-galactosidase. Because oligodendroglial cells are responsible for the elaboration of myelin, their loss results in myelin breakdown, thus producing additional galactocerebroside and causing a vicious circle of myelin destruction.