A three-sister sibship of Gerstmann-Sträussler-Scheinker disease with a CJD phenotype.

  title={A three-sister sibship of Gerstmann-Str{\"a}ussler-Scheinker disease with a CJD phenotype.},
  author={C. Majt{\'e}nyi and Paul Brown and Larisa Cerven{\'a}kov{\'a} and Lev G. Goldfarb and Jun Tateishi},
  volume={54 11},
OBJECTIVE To describe a rare phenotypic variant of P102L Gerstmann-Sträussler-Scheinker disease (GSS). BACKGROUND Classic GSS is characterized by an early age at onset, prominent cerebellar signs with a slowly evolving dementia, and a neuropathology including multifocal PrP-positive plaques and variable but usually modest spongiform change. METHODS Clinical, neuropathologic, immunohistochemical, and molecular genetic analysis of three sisters in a Hungarian family was performed. RESULTS… CONTINUE READING
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