Sudanophilic Cerebral Sclerosis

The disorder seems to begin rarely in early infancy. However, the paucity of myelin in the cerebral hemispheres during the first 4 to 6 months of life would make histopathologic classification on the basis of myelin breakdown difficult at this stage. Progression is usually subacute in pace. Cortical blindness is often a conspicuous feature. Sibs may show great differences in the site of the lesion, age of onset, and rate of progression (Meyer and Pilkington, 1936). All cases reported as familial Schilder disease are probably in fact sudanophilic cerebral sclerosis, Krabbe disease (245200), or metachromatic leukoencephalopathy (250100). If the term is to be preserved at all, its use should be confined to sudanophilic cerebral sclerosis. (The neurologic disorder in adrenoleukodystrophy is also referred to as Schilder disease (300100).)