Funding agencies: This study was supported by the NINDS (HHSN271200623661C [to Joel H. Kramer]), the UC Discovery Research and Development Grant (ITLBIO 178688), the NIA (K23AG037566 [to Katherine L. Possin], R01AG032289 [to Joel H. Kramer], P50AG023501 [to Bruce L. Miller]), the Larry L. Hillblom Foundation (2007/2I [to Bruce L. Miller]), the Hellman Family Foundation, the Michael J. Homer Family Fund, the Taube/Koret Center, and the Huntington's Disease Society of America (made possible with a gift from the James E. Bashaw Family).
Executive functions in premanifest Huntington's disease
Article first published online: 27 DEC 2013
© 2013 International Parkinson and Movement Disorder Society
Volume 29, Issue 3, pages 405–409, March 2014
How to Cite
You, S. C., Geschwind, M. D., Sha, S. J., Apple, A., Satris, G., Wood, K. A., Johnson, E. T., Gooblar, J., Feuerstein, J. S., Finkbeiner, S., Kang, G. A., Miller, B. L., Hess, C. P., Kramer, J. H. and Possin, K. L. (2014), Executive functions in premanifest Huntington's disease. Mov. Disord., 29: 405–409. doi: 10.1002/mds.25762
Relevant conflicts of interest/financial disclosures: Nothing to report.
Full financial disclosures and author roles may be found in the online version of this article.
- Issue published online: 17 MAR 2014
- Article first published online: 27 DEC 2013
- Manuscript Accepted: 6 NOV 2013
- Manuscript Revised: 27 OCT 2013
- Manuscript Received: 1 JUL 2013
- Huntington's disease;
- executive function;
- working memory;
- striatal volume;
- disease burden
We investigated the viability of psychometrically robust executive function measures as markers for premanifest Huntington's disease (HD).
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.
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.
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