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

Neurology

Volumn 89, Issue 24, 2017, Pages 2495-2502

  Schobel, Scott A ^a   Palermo, Giuseppe ^a   Auinger, Peggy ^b   Long, Jeffrey D ^c   Ma, Shiyang ^b   Khwaja, Omar S ^a   Trundell, Dylan ^a   Cudkowicz, Merit ^d   Hersch, Steven ^d   Sampaio, Cristina ^e   Dorsey, E Ray ^b   Leavitt, Blair R ^f   Kieburtz, Karl D ^b   Sevigny, Jeffrey J ^a   Langbehn, Douglas R ^c   Tabrizi, Sarah J ^g  

^a F HOFFMANN LA ROCHE LTD   (Switzerland)

^b UNIVERSITY OF ROCHESTER   (United States)

^c UNIVERSITY OF IOWA   (United States)

^d MASSACHUSETTS GENERAL HOSPITAL   (United States)

^e CHDI MANAGEMENT CHDI FOUNDATION   (United States)

^f UNIVERSITY OF BRITISH COLUMBIA   (Canada)

^g UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

ADULT; ARTICLE; BEHAVIOR DISORDER ASSESSMENT; BRAIN ATROPHY; BRAIN SIZE; CIRCLE TRACING; CLINICAL OUTCOME; COGNITIVE DEFECT; CONTROLLED STUDY; DISEASE ASSOCIATION; DISEASE EXACERBATION; EMOTION RECOGNITION; FEMALE; FOLLOW UP; FORCE; FUNCTIONAL DISEASE; GRIP FORCE; HAND STRENGTH; HUMAN; HUNTINGTON CHOREA; LONGITUDINAL STUDY; MAJOR CLINICAL STUDY; MALE; MIDDLE AGED; MOTOR DYSFUNCTION; NEUROLOGIC DISEASE ASSESSMENT; OBSERVATIONAL STUDY; PRIORITY JOURNAL; PROBLEM BEHAVIORS ASSESSMENT; RECOGNITION; SIGNAL NOISE RATIO; TEST RETEST RELIABILITY; TONGUE FORCE; UNIFIED HUNTINGTON DISEASE RATING SCALE; ANALOGS AND DERIVATIVES; APATHY; EMOTION; FACIAL RECOGNITION; MOTOR PERFORMANCE; NEUROPSYCHOLOGICAL TEST; PATHOPHYSIOLOGY; PERCEPTION; PROSPECTIVE STUDY; PSYCHOLOGY; RANDOMIZED CONTROLLED TRIAL (TOPIC); REPRODUCIBILITY; STROOP TEST;

UBIDECARENONE; UBIQUINONE; VITAMIN;

ADULT; APATHY; COGNITIVE DYSFUNCTION; DISEASE PROGRESSION; EMOTIONS; FACIAL RECOGNITION; FEMALE; HUMANS; HUNTINGTON DISEASE; LONGITUDINAL STUDIES; MALE; MIDDLE AGED; MOTOR SKILLS; NEUROPSYCHOLOGICAL TESTS; PROSPECTIVE STUDIES; RANDOMIZED CONTROLLED TRIALS AS TOPIC; REPRODUCIBILITY OF RESULTS; SIGNAL-TO-NOISE RATIO; SOCIAL PERCEPTION; STROOP TEST; UBIQUINONE; VITAMINS;

EID: 85039840959     PISSN: 00283878     EISSN: 1526632X     Source Type: Journal    
DOI: 10.1212/WNL.0000000000004743     Document Type: Article

References (24)

1. Ross CA, Tabrizi SJ. Huntington's disease: from molecular pathogenesis to clinical treatment. Lancet Neurol 2011; 10:83-98.
2. Unified Huntington's Disease Rating Scale: reliability and consistency: Huntington Study Group. Mov Disord 1996; 11:136-142.
3. Huntington Study Group. A randomized, placebocontrolled trial of coenzyme Q10 and remacemide in Huntington's disease. Neurology 2001;57:397-404.
4. Tabrizi SJ, Scahill RI, Owen G, et al. Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month observational data. Lancet Neurol 2013;12:637-649.
5. Dorsey ER, Beck CA, Darwin K, et al. Natural history of Huntington disease. JAMA Neurol 2013;70:1520-1530.
6. McGarry A, McDermott M, Kieburtz K, et al. A randomized, double-blind, placebo-controlled trial of coenzyme Q10 in Huntington disease. Neurology 2017;88:152-159.
7. Elkum N, Shoukri MM. Signal-to-noise ratio (SNR) as a measure of reproducibility: design, estimation, and application. Health Serv Outcomes Res Methodol 2008;8:119-133.
8. Zhang Y, Long JD, Mills JA, et al. Indexing disease progression at study entry with individuals at-risk for Huntington disease. Am J Med Genet B Neuropsychiatr Genet 2011; 156B:751-763.
9. Shrout PE, Fleiss JL. Intraclass correlations: uses in assessing rater reliability. Psychol Bull 1979;86:420-428.
10. Yi Q, Panzarella T. Estimating sample size for tests on trends across repeated measurements with missing data based on the interaction term in a mixed model. Control Clin Trials 2002;23:481-496.
11. Kremer B, Clark CM, Almqvist EW, et al. Influence of lamotrigine on progression of early Huntington disease: a randomized clinical trial. Neurology 1999;53:1000-1011.
12. Landwehrmeyer GB, Dubois B, de Yebenes JG, et al. Riluzole in Huntington's disease: a 3-year, randomized controlled study. Ann Neurol 2007;62:262-272.
13. Shoulson I, Odoroff C, Oakes D, et al. A controlled clinical trial of baclofen as protective therapy in early Huntington's disease. Ann Neurol 1989;25:252-259.
14. NCT02006472. PRIDE HD clinical trial (Pridopidine, Teva Pharmaceuticals). Available at: ClinicalTrials.gov. Accessed August 21, 2017.
15. NCT02197130. Amaryllis clinical trial (PDE10A inhibitor; Pfizer). Available at: ClinicalTrials.gov. Accessed August 21, 2017.
16. NCT02101957. Cysteamine clinical trial (Raptor Pharmaceuticals). Available at: ClinicalTrials.gov. Accessed August 21, 2017.
17. NCT02215616. LEGATO HD trial (Laquinimod, Teva Pharmaceuticals). Available at: ClinicalTrials.gov. Accessed August 21, 2017.
18. Huntington Study Group, Frank S, Testa CM, et al. Effect of deutetrabenazine on chorea among patients with Huntington disease: a randomized clinical trial. JAMA 2016;316:40-50.
19. Huntington Study Group. A randomized, double-blind, placebo-controlled trial of pridopidine in Huntington's disease. Mov Disord 2013;28:1407-1415.
20. Stout JC, Queller S, Baker KN, et al. HD-CAB: a cognitive assessment battery for clinical trials in Huntington's disease 1,2,3. Mov Disord 2014;29:1281-1288.
21. Stout JC, Jones R, Labuschagne I, et al. Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease. J Neurol Neurosurg Psychiatry 2012;83:687-694.
22. Salem L, Saleh N, Youssov K, et al. The most appropriate primary outcomes to design clinical trials on Huntington's disease: meta-analyses of cohort studies and randomized placebo-controlled trials. Fundam Clin Pharmacol 2014; 28:700-710.
23. Hughes CP, Berg L, Danziger WL, Coben LA, Martin RL. A new clinical scale for the staging of dementia. Br J Psychiatry 1982;140:566-572.
24. Fahn S, Elton R; Members of the UPDRS Development Committee. Recent developments in Parkinson's disease. Macmillan Health Care Inf 1987;2:153-163, 293-304.