Huntington disease (HD) is an autosomal dominant neurodegenerative disease caused by expansion of the trinucleotide cytosine-adenine-guanine (CAG) in the firs exon of the Huntingtin (HTT) gene. It is well-known that HD manifests as a triad of clinical symptoms in motor, cognition, and psychiatric. Currently, there is no effective treatment for HD patients. The faculty in the Department of Biostatistics have been actively engaged in a large 12-year prospective observational study at 32 sites in six countries including USA, Canada, Germany, Australia, Spain, and UK, PREDICT-HD, funded by NIH/NIDDS, to (i). predict the HD manifestation for prodromal HD subjects; (ii). lead the development of statistical disease progression models for understanding the HD progression; (iii). facilitate a strategy to enrich the sample for conducting disease-modifying intervention trials.
- Zhang, Y., Zhou, J., Gehl, CR., Long, JD., Johnson, H., Magnotta, VA., Sewell, D., Shannon, K., and Paulsen, JS. (2021). Mild cognitive impairment as an early landmark in Huntington’s disease. Frontier of Neurology-Movement Disorder. doi:10.3389/fneur.2021.678652.
- Paulsen, J., Lourens, S., Kieburtz, K., and Zhang, Y. (2019). Sample enrichment for clinical trials to show delay of onset in Huntington disease. Movement Disorder. 34(2): 274-280.
- Lourens, S., Zhang, Y., Long, JD., and Paulsen, LS. (2017). Analysis of longitudinal censored semicontinuous data with application to the study of executive dysfunction: The Tower Task. Statistical Methods for Medical Research. 26(2): 865-897.
- Liu, D., Long, JD., Zhang, Y., Raymond, LA., Marder, K., Rosser, A., McCukser, EA., Mills, JA., and Paulsen, JS. (2015). Motor onset and diagnosis in Huntington disease using the diagnostic confidence level. Journal of Neurology. 262: 2691-2698.
- Paulsen, JS., Long, JD., Ross, CA., Harrington, DL., Erwin, CJ., Williams, JK., Westervelt, KH., Johnson, HJ., Aylward, EH., Zhang, Y., Bockholt, HJ., Barker, RA., and the PREDICT-HD investigators and Coordinators of the Huntington Study Group. (2014). Prediction of manifest of Huntington disease with clinical and imaging measures: A 12-year prospective observation study. The Lancet-Neurology. 13: 1193-1201.
- Biglan, KM., Zhang, Y., Long, JD., Geschwind, M., Kang, GA., Killoran, A., Lu, W., McCusker, E., Mills, JA., Raymond, LA., Testa, C., Wojcieszek, J., Paulsen, JS., and the PREDICT-HD investigators of the Huntington Study Group. (2013). Refining the diagnosis of Huntington Disease: The PREDICT-HD study. Frontier in Aging Neuroscience. 5:12. doi:10.3389/fnagi.2013.00012.
- Harrington, D., Smith, M., Zhang, Y., Carlozzi, N., Paulsen, JS., and the PREDICT-HD investigators and Coordinators of the Huntington Study Group. (2012). Cognitive domains that predict time to diagnosis in prodromal Huntington disease. Journal of Neurology, Neurosurgery, and Psychiatry. 83: 612-619.
- Zhang, Y., Long, JD., Mills, JA., Warner, JH., Lu, W., Paulsen, JS., and the PREDICT-HD investigators and Coordinators of the Huntington Study Group. (2011). Indexing disease progression at study entry with individuals at-risk for Huntington disease. American Journal of Medical Genetics, Part B: Neuropsychiatric Genetics. 156(7): 751-763.