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Neurology. 2017 Dec 12;89(24):2495-2502. doi: 10.1212/WNL.0000000000004743. Epub 2017 Nov 15.

Motor, cognitive, and functional declines contribute to a single progressive factor in early HD.

Collaborators (384)

Adams J, Jog M, Hyson C, Furtado S, Duker A, Armstrong M, Lachner C, Fernandez H, Okun MS, Shprecher D, Cartwright M, Loy C, Jiminez-Shahed J, Robottom B, Suter G, Moore T, Forsyth J, Hurt A, Belden J, Price K, Erickson D, Nickels B, Thompson MM, Cole L, Megens J, Makaji E, Masse SL, Malarick K, Mook L, Maya S, Bender A, Meyer J, Turakhia P, Harwood K, Duderstadt K, Lenarz S, Hamerlinck J, Edern P, Hickey C, Owens A, Gibbons C, Gray C, Jaglin JA, Janko K, Lawrence H, Estes B, Hayward B, Johnson A, Gode A, Huet G, Romero-Kersh B, Wernette K, Sullivan E, Guyot J, Konkle J, O'Neill C, King P, Martin A, Bautista J, Mans N, Griffith J, Surles E, Halton S, Palao A, Padron N, Elliott K, Oelke L, Butterfield L, Perry-Trice P, Wyne S, Pantella C, Tainsh L, Sperin E, Evans S, Gartner M, Duffy A, Kristof P, Niles L, Rainone S, Wernle A, Clouse R, Cines M, Dustin K, Deeley M, Merritt S, Ferreri H, McMurray A, Sheinberg A, Hughes-Gay M, Ladonna K, Chung E, Lee J, Goldstein R, Esposito L, Scoglio N, Grana M, Zimmerman C, Blasucci LM, Thayer W, Hawkins J, Koch J, Hunt V, Bargoil J, Scott I, Tempkin T, Stewart L, Hayes E, Galea D, Belmar B, Richardson K, Hunter C, Jimenez E, Quesada M, Levy W, Blenke A, Gauger L, Stoner J, Klimek ML, Sommerfeld B, Eglow M, Regan K, Neefus E, Malikowski M, Radam T, Zomok M, Lannon M, Agramonte R, Hartlein J, Nickerson C, Gibson S, Monari EH, Swartz C, Smith-Bova A, Jones R, Wielinski C, Graham S, Psych DGD, Clin Neuropsych JMM, Koroshetz W, McDermott M, Beal MF, Greenamyre JT, Ross CA, Shoulson I, Cudkowicz ME, Sexton P, Skeuse C, Rosas D, Jenkins B, Rosenblatt A, Sherr M, Hersch S, Cellar J, Guttman M, Brown C, Como PG, Marshall F, DeMarcaida JA, Zimmerman C, Marder K, Moskowitz C, Polanco C, Beltre J, Shannon K, Jaglin JA, Niederman F, Ashizawa T, Hunter C, Hunter C, Hauser R, Walker A, Gauger L, Hahn MA, Delgado H, Dawson T, Siemers E, Seeberger L, Dingmann C, Dubinsky R, Gray C, Weiner WJ, Rodriguez-Batemen D, Albin RL, Wernette K, Harrison M, Rost-Ruffner E, Paulsen J, Rodnitzky RL, Dobson J, Vining L, Suchowersky O, Pantella C, Saint-Hilaire MH, Furtado J, Martin W, Wieler M, Walker F, Hunt V, Raymond L, Almqvist E, Rubin A, Blair B, Marek K, Caplan K, Baker D, Brocht A, Casaceli C, Orme C, Rudolph A, Rumfola L, Schifitto G, Shinaman A, Sulimowicz K, Watts A, Eapen S, Zhang L, Tariot P, Fahn S, Hall W, Koller W, Gorell J, Woolson R, Shoulson I, Gusella J, Foroud T, Van Kammen DP, Marshall F, Hickey C, Marder K, Frucht S, Moskowitz C, PaulaWasserman RC, Muratori L, Louis E, Shannon K, Jaglin J, Jankovic J, Palao A, Harrison M, Davis R, Dietrich S, Singer C, Quesada M, Hersch S, Rosas D, Tanev K, Malarick K, McInnis R, Sanchez-Ramos J, Kostyk S, Doucette N, Paulsen J, Uc E, Rodnitzky R, Leserman A, Vik S, Perlmutter J, Tabbal S, Schmidt A, Barton S, Ross C, Dorsey R, Nucifora F, Dubinsky R, Dubinsky H, Broyles J, Suchowersky O, Klimek ML, Jones R, Testa C, Sperin E, Testa C, Jennings D, Higgins D, Molho E, Adams J, Frank S, Saint-Hilaire M, Diggin M, Furtado S, Christine O'Neill F, Quaid K, Wesson M, Zauber SE, LeDoux M, Raymond L, Leavitt B, Decolongon J, Perlman S, Corey-Bloom J, Peavy G, Kumar R, Segro V, Erickson D, McCusker E, Griffith J, Loy C, Wheelock V, Tempkin T, Martin A, Nance M, Kang U, Mallonee W, Suter G, Revilla F, Drazinic C, Fitzpatrick MJ, Duff K, Scott B, Robottom B, Chiu E, Yastrubetskaya O, Churchyard A, Greenamyre TJ, Lucarelli N, Agarwal P, Antonijevic I, DeLaRosa J, Panegyres P, Coleman A, Oakes D, Beck C, Robertson S, Holt D, Eaton K, Lindsay P, Deuel L, MacDonald M, Foroud T, Dorsey R, Giuliano J, Vetter L, Suchowersky O, Beck C, Oakes D, Tabrizi SJ, Durr A, Leavitt B, Roos RA, Borowsky B, Landwehrmeyer B, Frost C, Johnson H, Langbehn D, Craufurd D, Feilmann R, Stout JC, Campbell A, Campbell M, Labuschagne I, Milchman C, Coleman CA, Santos RD, Decolongon J, Sturrock A, Jauff Ret FC, Justo D, Lehericy S, Marelli C, Nigaud K, Valabregue R, Bechtel GN, Bohlen S, van den Bogaard NSJA, Dumas EM, van der Grond J, Hart EP', Arran UN, Callaghan J, Stopford C, Cash DM, Crawford H, Fox NC, Hobbs NZ, Lahiri N, Malone I, Read J, Say MJ, Whitehead D, Wild E, Jones R, Axelson UE, Queller S, Campbell C.

Author information

1
From F. Hoffman-La Roche, Ltd (S.A.S., G.P., O.S.K., D.T., J.J.S.), Roche Innovation Center, Basel, Switzerland; University of Rochester (P.A., S.M., E.R.D., K.D.K.), NY; University of Iowa (J.D.L., D.R.L.), Iowa City; Massachusetts General Hospital and Harvard Medical School (M.C., S.H.), Boston; CHDI Management/Foundation (C.S.), Princeton, NJ; University of British Columbia, Vancouver, Canada (B.R.L.); and University College London (S.J.T.), UK. scott.schobel@roche.com.
2
From F. Hoffman-La Roche, Ltd (S.A.S., G.P., O.S.K., D.T., J.J.S.), Roche Innovation Center, Basel, Switzerland; University of Rochester (P.A., S.M., E.R.D., K.D.K.), NY; University of Iowa (J.D.L., D.R.L.), Iowa City; Massachusetts General Hospital and Harvard Medical School (M.C., S.H.), Boston; CHDI Management/Foundation (C.S.), Princeton, NJ; University of British Columbia, Vancouver, Canada (B.R.L.); and University College London (S.J.T.), UK.

Abstract

OBJECTIVE:

To identify an improved measure of clinical progression in early Huntington disease (HD) using data from prospective observational cohort studies and placebo group data from randomized double-blind clinical trials.

METHODS:

We studied Unified Huntington Disease Rating Scale (UHDRS) and non-UHDRS clinical measures and brain measures of progressive atrophy in 1,668 individuals with early HD followed up prospectively for up to 30 to 36 months of longitudinal clinical follow-up.

RESULTS:

The results demonstrated that a composite measure of motor, cognitive, and global functional decline best characterized clinical progression and was most strongly associated with brain measures of progressive corticostriatal atrophy.

CONCLUSIONS:

Use of a composite motor, cognitive, and global functional clinical outcome measure in HD provides an improved measure of clinical progression more related to measures of progressive brain atrophy and provides an opportunity for enhanced clinical trial efficiency relative to currently used individual motor, cognitive, and functional outcome measures.

PMID:
29142089
PMCID:
PMC5729794
DOI:
10.1212/WNL.0000000000004743
[Indexed for MEDLINE]
Free PMC Article

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