A rare, progressive, and lethal disease of the central nervous system
that 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 patients die within few months after onset.