Abstract
It is well known in Huntington's disease that cytosine-adenine-guanine expansion and age at study entry are predictive of the timing of motor diagnosis. The goal of this study was to assess whether additional motor, imaging, cognitive, functional, psychiatric, and demographic variables measured at study entry increased the ability to predict the risk of motor diagnosis over 12 years. One thousand seventy-eight Huntington's disease gene-expanded carriers (64% female) from the Neurobiological Predictors of Huntington's Disease study were followed up for up to 12 y (mean = 5, standard deviation = 3.3) covering 2002 to 2014. No one had a motor diagnosis at study entry, but 225 (21%) carriers prospectively received a motor diagnosis. Analysis was performed with random survival forests, which is a machine learning method for right-censored data. Adding 34 variables along with cytosine-adenine-guanine and age substantially increased predictive accuracy relative to cytosine-adenine-guanine and age alone. Adding six of the common motor and cognitive variables (total motor score, diagnostic confidence level, Symbol Digit Modalities Test, three Stroop tests) resulted in lower predictive accuracy than the full set, but still had twice the ...Continue Reading
References
Feb 11, 2000·Neurology·K MarderI Shoulson
Jun 9, 2005·The American Journal of Clinical Nutrition·Ann M GabaCarol N Boozer
Jan 24, 2007·Biometrical Journal. Biometrische Zeitschrift·Thomas A Gerds, Martin Schumacher
Mar 29, 2007·Seminars in Neurology·Francis O Walker
Dec 22, 2007·Journal of Neurology, Neurosurgery, and Psychiatry·J S PaulsenUNKNOWN Predict-HD Investigators and Coordinators of the Huntington Study Group
Jun 24, 2009·American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics : the Official Publication of the International Society of Psychiatric Genetics·Douglas R LangbehnUNKNOWN and the PREDICT-HD Investigators of the Huntington Study Group
Jul 14, 2010·Movement Disorders : Official Journal of the Movement Disorder Society·Jane S PaulsenUNKNOWN PREDICT-HD Investigators of the Huntington Study Group
May 24, 2011·Lancet Neurology·David W WeirBlair R Leavitt
Aug 23, 2011·American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics : the Official Publication of the International Society of Psychiatric Genetics·Ying ZhangUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Aug 24, 2011·Current Neurology and Neuroscience Reports·Jane S Paulsen
Oct 25, 2011·Science·John Bohannon
Mar 28, 2012·Journal of Neurology, Neurosurgery, and Psychiatry·Deborah Lynn HarringtonUNKNOWN PREDICT-HD Investigators of the Huntington Study Group
May 2, 2012·Genomics·Xi Chen, Hemant Ishwaran
Jun 14, 2012·Movement Disorders : Official Journal of the Movement Disorder Society·Tamara PringsheimNathalie Jette
Oct 24, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Jeffery D LongUNKNOWN Researchers of the PREDICT-HD Huntington's Study Group
Dec 12, 2013·European Journal of Human Genetics : EJHG·Nancy R DowningUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Jan 1, 2010·Future Neurology·Jane S Paulsen
Mar 13, 2014·Nature Reviews. Neurology·Christopher A RossSarah J Tabrizi
May 6, 2014·Frontiers in Aging Neuroscience·Jane S PaulsenUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Aug 22, 2014·Movement Disorders : Official Journal of the Movement Disorder Society·Jane S Paulsen, Jeffrey D Long
Dec 3, 2014·Lancet Neurology·Jane S PaulsenUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Mar 5, 2015·European Journal of Human Genetics : EJHG·Ji-In KimUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Citations
Oct 7, 2016·Frontiers in Neurology·Jennifer Ashley CiarochiUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Jan 4, 2017·Human Brain Mapping·Joseph J ShafferHans J Johnson
Nov 29, 2016·Movement Disorders : Official Journal of the Movement Disorder Society·Jeffrey D LongCristina Sampaio
Jun 23, 2018·Brain Sciences·Jennifer A CiarochiUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Jan 31, 2020·Biostatistics·Unkyung LeeTanya P Garcia
Jul 8, 2016·Annals of Clinical and Translational Neurology·Tanya SimuniUNKNOWN PPMI Investigators
Sep 2, 2017·Scientific Reports·José M Lezcano-ValverdeLuis Rodriguez-Rodriguez
Apr 14, 2018·Frontiers in Neurology·Jingyu LiuUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Nov 18, 2018·BMC Medical Research Methodology·Jeffrey D Long, James A Mills
Jan 16, 2019·Movement Disorders : Official Journal of the Movement Disorder Society·Jane S PaulsenYing Zhang
Jun 7, 2017·Journal of Huntington's Disease·Kan LiUNKNOWN PREDICT-HD Investigators of the Huntington Study Group
Jul 1, 2019·Journal of Huntington's Disease·Fan LiUNKNOWN PREDICT-HD and ENROLL-HD Investigators of the Huntington Study Group
Apr 2, 2019·Journal of Huntington's Disease·Jennifer A CiarochiUNKNOWN PREDICT-HD Investigators and Coordinators of the Huntington Study Group
Jan 29, 2020·Scientific Reports·Pablo PoloseckiGuillermo A Cecchi
Nov 22, 2020·Scientific Reports·C Y Daniel LeeX William Yang
May 6, 2020·Clinical Parkinsonism & Related Disorders·Emilia M GattoClaudia Perandones
Aug 21, 2018·American Journal of Human Genetics·Jeffrey D LongJames F Gusella
Feb 27, 2017··Milan SonkaSatyananda Kashyap