Krabbe Leukodystrophy Syndrome: A rare, progressive
and lethal disease of the central nervous system leading to sudanophilic
cerebral sclerosis, metachromatic leukodystrophy, and adrenoleukodystrophy.
Affects mostly children and characterized by adrenal atrophy and diffuse
central demyelination. Presents with progressive dementia, spasticity,
cortical blindness, deafness, hemiplegia, quadriplegia, ataxia, pyramidal
signs, retrobulbar neuritis, and pseudobulbar palsy. Seizures. Onset in late
childhood. Most die within a few months after onset.