Data from: Pearls & Oy-sters: challenging diagnosis of Gerstmann-Sträussler-Scheinker disease: clinical and imaging findings

Min Ju Kang, Jeewon Suh, Seong Soo An, SangYun Kim & Young Ho Park
PEARLS >Gerstmann-Straussler-Scheinker disease (GSS) is a rare prion disease characterized by cerebellar ataxia with progressive cognitive decline. >GSS is caused by a mutation within the prion protein gene (PRNP), which commonly exhibits autosomal dominant inheritance pattern. However, a significant portion of previously reported cases showed no family history of the disease, and GSS may also occur through de novo mutation of PRNP. OY-STERS >GSS is clinically heterogeneous and has no characteristic features on imaging. GSS...

Registration Year

  • 2018

Resource Types

  • Dataset


  • Seoul National University Bundang Hospital
  • Gachon University