Article
Prion dementia without characteristic pathology.
Lancet (London, England) - 7 Jul 1990
Collinge J, Owen F, Poulter M, Leach M, Crow T J, Rossor M N, Hardy J, Mullan M J, Janota I, Lantos P L
Abstract excerpt
Gerstmann-Sträussler syndrome (GSS) was diagnosed in a family with presenile dementia by prion protein gene analysis. Extensive histological examination of the brain of an affected individual from this family showed no characteristic features of GSS or Creutzfeldt-Jakob disease (CJD). Thus "spongiform encephalopathy" (GSS or CJD) cannot always be excluded on neuropathological grounds in an individual dying of a...
Topics
- Alleles
- Alzheimer Disease
- Brain Chemistry
- DNA, Viral
- Humans
- Male
- Mutation
- Oligonucleotide Probes
- Pedigree
- Phenotype
- Polymerase Chain Reaction
- PrPSc Proteins