Motor changes in presymptomatic Huntington disease gene carriers

Eric Siemers, Tatiana Foroud, Daniel J. Bill, Jeffrey Sorbel, James A. Norton, M. E. Hodes, Gwendolyn Niebler, P. Michael Conneally, Joe C. Christian

Research output: Contribution to journalArticle

60 Citations (Scopus)

Abstract

Objectives: To determine whether changes in motor function and reaction time are present in presymptomatic individuals carrying the Huntington disease (HD) allele. Design: A case-control, double-blind study comparing asymptomatic at-risk subjects, with or without the HD allele, and subjects clinically determined to have early manifest HD. Setting: The Department of Medical and Molecular Genetics at Indiana University School of Medicine, Indianapolis. Participants: We studied 383 patients at risk for HD. Each subject was asymptomatic by self-report. Measures: Genotype for the HD allele was determined by polymerase chain reaction testing. A battery of 8 physiological tests measuring speed of movement and reaction time was performed with a computer-driven system. Results: Following neurologic examination, 17 of the 120 gene carriers (GCs) had symptoms sufficient for a clinical diagnosis of manifest HD. The remaining 103 GCs were designated presymptomatic GCs. When the non-GCs were compared with the presymptomatic GCs (1-way analysis of covariance and the Fisher protected t test), results on 3 of the 8 physiological tests movement time, movement time with decision, and auditory reaction time-were different. Additionally, the number of trinucleotide (CAG) repeats significantly correlated with test performance for movement time with decision and visual reaction time with decision when both the entire group of GCs and the presymptomatic GCs alone were considered. Conclusion: These results suggest that subtle subclinical changes in motor function are present in presymptomatic individuals who have inherited the HD allele.

Original languageEnglish
Pages (from-to)487-492
Number of pages6
JournalArchives of Neurology
Volume53
Issue number6
StatePublished - Jun 1996

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Asymptomatic Diseases
Huntington Disease
Genes
Alleles
Trinucleotide Repeats
Computer Systems
Neurologic Examination
Medical Genetics
Gene
Carrier
Double-Blind Method
Self Report
Molecular Biology
Genotype
Medicine
Polymerase Chain Reaction
Allele
Reaction Time

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Siemers, E., Foroud, T., Bill, D. J., Sorbel, J., Norton, J. A., Hodes, M. E., ... Christian, J. C. (1996). Motor changes in presymptomatic Huntington disease gene carriers. Archives of Neurology, 53(6), 487-492.

Motor changes in presymptomatic Huntington disease gene carriers. / Siemers, Eric; Foroud, Tatiana; Bill, Daniel J.; Sorbel, Jeffrey; Norton, James A.; Hodes, M. E.; Niebler, Gwendolyn; Conneally, P. Michael; Christian, Joe C.

In: Archives of Neurology, Vol. 53, No. 6, 06.1996, p. 487-492.

Research output: Contribution to journalArticle

Siemers, E, Foroud, T, Bill, DJ, Sorbel, J, Norton, JA, Hodes, ME, Niebler, G, Conneally, PM & Christian, JC 1996, 'Motor changes in presymptomatic Huntington disease gene carriers', Archives of Neurology, vol. 53, no. 6, pp. 487-492.
Siemers E, Foroud T, Bill DJ, Sorbel J, Norton JA, Hodes ME et al. Motor changes in presymptomatic Huntington disease gene carriers. Archives of Neurology. 1996 Jun;53(6):487-492.
Siemers, Eric ; Foroud, Tatiana ; Bill, Daniel J. ; Sorbel, Jeffrey ; Norton, James A. ; Hodes, M. E. ; Niebler, Gwendolyn ; Conneally, P. Michael ; Christian, Joe C. / Motor changes in presymptomatic Huntington disease gene carriers. In: Archives of Neurology. 1996 ; Vol. 53, No. 6. pp. 487-492.
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