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

Published online by Cambridge University Press:  01 March 1997

FREDERICK W. UNVERZAGT ,
MARTIN R. FARLOW ,
JAMES NORTON ,
STEPHEN R. DLOUHY ,
KATHERINE YOUNG  and
BERNARDINO GHETTI
FREDERICK W. UNVERZAGT
Affiliation:
Department of Psychiatry, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202 Department of Medical and Molecular Genetics, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202
MARTIN R. FARLOW
Affiliation:
Department of Psychiatry, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202 Department of Neurology, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202
JAMES NORTON
Affiliation:
Department of Psychiatry, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202
STEPHEN R. DLOUHY
Affiliation:
Department of Medical and Molecular Genetics, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202 Department of Pathology and Laboratory Medicine, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202
KATHERINE YOUNG
Affiliation:
Department of Pathology and Laboratory Medicine, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202
BERNARDINO GHETTI
Affiliation:
Department of Psychiatry, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202 Department of Neurology, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202 Department of Medical and Molecular Genetics, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202 Department of Pathology and Laboratory Medicine, Division of Neuropathology, Indiana University School of Medicine, Indianapolis, IN 46202
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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.)

Keywords

Gerstmann-Sträussler-Scheinker diseaseDementiaNeuropsychological testingGenetic disorderCognition
Type
Research Article
Information
Journal of the International Neuropsychological Society , Volume 3 , Issue 2 , March 1997 , pp. 169 - 178
Copyright
© 1997 The International Neuropsychological Society

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