Executive functions in premanifest Huntington's disease

SC You, MD Geschwind, SJ Sha, A Apple… - Movement …, 2014 - Wiley Online Library
SC You, MD Geschwind, SJ Sha, A Apple, G Satris, KA Wood, ET Johnson, J Gooblar…
Movement Disorders, 2014Wiley Online Library
Background We investigated the viability of psychometrically robust executive function
measures as markers for premanifest Huntington's disease (HD). Methods Fifteen
premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive
function battery. This battery yields an overall executive composite score, plus working
memory, cognitive control, and fluency scores that are measured on psychometrically
matched scales. The scores were correlated with two disease markers, disease burden and …
Background
We investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).
Methods
Fifteen premanifest HD subjects and 42 controls were compared on the NIH EXAMINER executive function battery. This battery yields an overall executive composite score, plus working memory, cognitive control, and fluency scores that are measured on psychometrically matched scales. The scores were correlated with two disease markers, disease burden and striatal volumes, in the premanifest HD subjects.
Results
The premanifest HD subjects scored significantly lower on the working memory score. The executive composite positively correlated with striatal volumes, and the working memory score negatively correlated with disease burden. The cognitive control and fluency scores did not differ between the groups or correlate significantly with the disease markers.
Conclusions
The NIH EXAMINER executive composite and working memory scores are sensitive markers of cognitive dysfunction, striatal volume, and disease burden in premanifest HD. © 2013 International Parkinson and Movement Disorder Society
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