J Neuropsychiatry Clin Neurosci 1995; 7:239-242
Copyright © 1995 by American Neuropsychiatric Association
The dementia of Gerstmann-Straussler-Scheinker syndrome: clinical variability demonstrated by two case reports
CG Lyketsos and M Kraus
Neuropsychiatry and Memory Group, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Two cases of Gerstmann-Straussler-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.
