Dictionary > Flatau-schilder disease

Flatau-schilder disease

Flatau-schilder disease –>
Schilder’s disease
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 characterised 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, asymmetrical area of myelin destruction, sometimes involving an entire cerebral hemisphere, and typically with extension across the corpus callosum. 2) The leukodystrophies.
Synonym: encephalitis periaxialis diffusa, flatau-schilder disease.


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