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Neuropsychological function in patients with Gerstmann-Sträussler-Scheinker disease from the Indiana Kindred (F198S)

  • FREDERICK W. UNVERZAGT (a1) (a2), MARTIN R. FARLOW (a1) (a3), JAMES NORTON (a1), STEPHEN R. DLOUHY (a2) (a4), KATHERINE YOUNG (a4) and BERNARDINO GHETTI (a1) (a3) (a2) (a4)...

Abstract

Three patients with Gerstmann-Sträussler-Scheinker disease (GSS) caused by a serine-for-phenylalanine substitution at codon 198 of the prion protein gene (PRNP) were compared to 9 age- and education-matched non-mutation-carriers from the same large Indiana kindred (GSS–IK) on a comprehensive neuropsychological test battery. Clinically significant impairments in intelligence, secondary memory, attention and cognitive processing speed, executive ability, and manual motor skills were noted in 2 patients. The wide range and the severity of the cognitive deficits indicated generalized cerebral dysfunction consistent with global dementia. One patient, symptomatic for less than 1 year, had more selective deficits involving memory, motor skills, and verbal fluency, suggesting early subcortical involvement. (JINS, 1997, 3, 169–178.)

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