Definition: term used to describe at least two separate disorders described by Schilder: 1) Diffuse sclerosis or encephalitis periaxialis diffusa; a nonfamilial disorder affecting primarily children and young adults and characterized by progressive dementia, visual disturbances, deafness, pseudobulbar palsy, and hemiplegia or quadriplegia. Most patients die within a few years of onset; pathologically, there is a large, asymmetric area of myelin destruction, sometimes involving an entire cerebral hemisphere, and typically with extension across the corpus callosum. 2) The leukodystrophies.
Synonym(s): encephalitis periaxialis diffusa
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