A familial spongiform encephalopathy. Transgenic mice with a mutant form of the PrP gene from patients with this syndrome develop degenerative brain disease that is similar, but not identical, to that caused by scrapie.
It is a more chronic cerebellar form of spongiform encephalopathy, producing a neurodegenerative condition that has morphological similarities to Creutzfeldt-Jakob syndrome. However, in this syndrome there is slower progression, signs of spinocerebellar ataxia, and the spongiosis is less pronounced.
(12 Jul 2000)
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