Gerstmann-Sträussler-Scheinker disease- the dilemma of molecular and clinical correlations.

@article{Boellaard1999GerstmannStrusslerScheinkerDT,
  title={Gerstmann-Str{\"a}ussler-Scheinker disease- the dilemma of molecular and clinical correlations.},
  author={J. W. Boellaard and Paul Brown and Jun Tateishi},
  journal={Clinical neuropathology},
  year={1999},
  volume={18 6},
  pages={271-85}
}
Gerstmann-Sträussler-Scheinker disease (GSSD) is a hereditary as well as transmissible human prion disease, restricted to families carrying point mutations of the PRPN gene on chromosome 20. To date 7 different causative mutations have been found. In this review the results of molecular biology with regard to the clinical course are discussed. As the findings of the disorder are very variable, the clinical picture and the neuropathology are extensively reported. An attempt has been made to… CONTINUE READING

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