The dementia of Gerstmann-Sträussler-Scheinker syndrome: clinical variability demonstrated by two case reports

J Neuropsychiatry Clin Neurosci. 1995 Spring;7(2):239-42. doi: 10.1176/jnp.7.2.239.

Abstract

Two cases of Gerstmann-Sträussler-Scheinker syndrome (GSS) are discussed in detail. These cases illustrate the variable clinical course and mostly cortical features of GSS-associated dementia. Given the availability of genetic tests that can now diagnose GSS, clinicians should consider these tests in cases where patients with dementia have extensive family histories or if the clinical course is atypical for Alzheimer's or vascular dementia.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Alzheimer Disease / diagnosis
  • Alzheimer Disease / psychology
  • Atrophy
  • Cerebral Cortex / pathology
  • Dementia, Vascular / diagnosis
  • Dementia, Vascular / psychology
  • Diagnosis, Differential
  • Diagnostic Imaging
  • Female
  • Gerstmann-Straussler-Scheinker Disease / diagnosis*
  • Gerstmann-Straussler-Scheinker Disease / genetics
  • Gerstmann-Straussler-Scheinker Disease / psychology
  • Humans
  • Male
  • Middle Aged
  • Neurologic Examination
  • Neuropsychological Tests