The Trail Making Test in prodromal Huntington disease: Contributions of disease progression to test performance

Abstract

We examined the Trail Making Test (TMT) in a sample of 767 participants with prodromal Huntington disease (prodromal HD) and 217 healthy comparisons to determine the contributions of motor, psychiatric, and cognitive changes to TMT scores. Eight traditional and derived TMT scores were also evaluated for their ability to differentiate prodromal participants closer to estimated age of diagnosis from those farther away and prodromal individuals from healthy comparisons. Results indicate that motor signs only mildly affected Part A, and psychiatric symptoms did not affect either part. Tests of perceptual processing, visual scanning, and attention were primarily associated with Part A, and executive functioning (response inhibition, set-shifting), processing speed, and working memory were associated with Part B. Additionally, TMT scores differentiated between healthy comparisons and prodromal HD individuals as far as 9–15 years before estimated diagnosis. In participants manifesting prodromal motor signs and psychiatric symptoms, the TMT primarily measures cognition and is able to discriminate between groups based on health status and estimated time to diagnosis.

Keywords:

• Huntington disease
• Cognition
• Motor
• Psychiatric
• Neurodegenerative

Acknowledgments

This research is supported by the National Institutes for Health, National Institute of Neurological Disorders and Stroke (NS40068), and CHDI Foundation, Inc. We thank the PREDICT-HD sites, the study participants, and the National Research Roster for Huntington Disease Patients and Families.

Predict-HD Investigators, Coordinators, Motor Raters, and Cognitive Raters are listed in the Appendix.