An infant presents with irritability, increased tone of extremities and recurrent seizures.  Tissue examination reveals globoid cells in parenchyma around blood vessels.  What is the most probable diagnosis?
Taysach’s disease
Krabbe disease
Adrenoleukodystrophy
Kanavan’s disease
(Refer: Nelson’ss textbook of pediatrics, 19th edition, pg no: 2071-2072)
Krabbe disease (KD)/globoid cell leukodystrophy

Autosomal 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.