Sudanophilic leukodystrophy with increased hexosamine content

Abstract

The case of a 44-year-old man who had shown a progressive deterioration of intellect accompanied by increasing spasticity and finally blindness is described in terms of diffuse cerebral sclerosis. Large symmetrical areas of demyelinization were found in the parieto-occipi-tal lobes with an accumulation of PAS-positive material throughout the lesion and sudanophilic material at its border. Chemical analysis showed a quantitative increase in hexosamine content throughout the cerebral lesions with increased cholesterol esters only at the border. Increased hexosamine content is not necessarily associated with meta-chromatic leukoencephalopathy. This case is unusual in that histological-ly it belongs to the sudanophilic type of demyelinating disease while chemically it has features of both the sudanophilic and metachromatic types.