{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T00:36:42Z","timestamp":1761007002214,"version":"build-2065373602"},"reference-count":17,"publisher":"Wiley","issue":"14","license":[{"start":{"date-parts":[[2013,10,18]],"date-time":"2013-10-18T00:00:00Z","timestamp":1382054400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2013,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:p>Treatment effect in Huntington disease (HD) clinical trials has relied on primary outcome measures such as total motor score or functional rating scales. However, these measures have limited sensitivity, particularly in pre\u2010 to early stages of the disease. We performed a systematic review of HD clinical studies to identify endpoints that correlate with disease severity. Using standard HD keywords and terms, we identified 749 published studies from 1993 to 2011 based on the availability of demographic, biochemical, and clinical measures. The average and variability of each measure was abstracted and stratified according to pre\u2010far, pre\u2010close, early, mild, moderate, and severe HD stages. A fixed\u2010effect meta\u2010analysis on selected variables was conducted at various disease stages. A total of 1,801 different clinical variables and treatment outcomes were identified. Unified Huntington Disease Rating Scale (UHDRS) Motor, UHDRS Independence, and Trail B showed a trend toward separation between HD stages. Other measures, such as UHDRS Apathy, Verbal Fluency, and Symbol Digit, could only distinguish between pre\u2010 and early stages of disease and later stages, whereas other measures showed little correlation with increasing HD stages. Using cross\u2010sectional data from published HD clinical trials, we have identified potential endpoints that could be used to track HD disease progression and treatment effect. Longitudinal studies, such as TRACK\u2010HD, are critical for assessing the value of potential markers of disease progression for use in future HD therapeutic trials. A list of variables, references used in this meta\u2010analysis, and database is available at <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.cmmt.ubc.ca\/research\/investigators\/leavitt\/publications\">http:\/\/www.cmmt.ubc.ca\/research\/investigators\/leavitt\/publications<\/jats:ext-link>. \u00a9 2013 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.25663","type":"journal-article","created":{"date-parts":[[2013,10,18]],"date-time":"2013-10-18T12:22:51Z","timestamp":1382098971000},"page":"1987-1994","source":"Crossref","is-referenced-by-count":8,"title":["A systematic review and meta\u2010analysis of clinical variables used in Huntington disease research"],"prefix":"10.1002","volume":"28","author":[{"given":"Sonia","family":"Franciosi","sequence":"first","affiliation":[{"name":"Center for Molecular Medicine and Therapeutics and Department of Medical Genetics Child and Family Research Institute, University of British Columbia Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yaein","family":"Shim","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Therapeutics and Department of Medical Genetics Child and Family Research Institute, University of British Columbia Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Margaret","family":"Lau","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Therapeutics and Department of Medical Genetics Child and Family Research Institute, University of British Columbia Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael R.","family":"Hayden","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Therapeutics and Department of Medical Genetics Child and Family Research Institute, University of British Columbia Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Blair R.","family":"Leavitt","sequence":"additional","affiliation":[{"name":"Center for Molecular Medicine and Therapeutics and Department of Medical Genetics Child and Family Research Institute, University of British Columbia Vancouver British Columbia Canada"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,10,18]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2007.128728"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870110204"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.870130120"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.63.6.883"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(09)70170-X"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(11)70070-9"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(11)70263-0"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.29.1.1"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.2004.00241.x"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(10)70276-3"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1037\/1082-989X.3.4.486"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.22601"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.23337"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0b013e3182020123"},{"key":"e_1_2_7_16_1","first-page":"108","volume-title":"Evaluation of Biomarkers and Surrogate Endpoints in Chronic Disease. 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