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Prion Dementia Without Characteristic Pathology Lancet 336:7-9, 21-221990., Collinge,J.,et al, 1990 See this aricle in Pubmed Article Abstract Gerstmann-Straussler syndrome(GSS)was diagnosed in a family with presenile dementia by prior 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 dementing condition, and the true prevalence of these diseases is likely to be underestimated. Screening by prior protein gene analysis will help to determine the full clinical and neuropathological phenotype in familial cases.This observation may be relevant to the assessment of possible transmission of above bovine spongiform encephalopathy to man.

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(click to filter results - removes previous filter) dementia dementia,familial dementia,presenile dementia,transmissible genetic linkage Gerstmann-Straussler-Scheinker disease molecular genetics neuropathology neuropathology,brain polymerase chain reaction prion disease

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