HD Insights™

Vol. 13 l Winter 2016

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20 HD Insights, Vol. 13 Editorial Board Members Ray Dorsey, MD — Editor, University of Rochester Donald Kennedy, PhD — Stanford University Nobuyuki Nukina, MD, PhD — Riken Brain Science Institute Rodrigo Osorio — Agrupación Chilena de Huntington Bernard Ravina, MD — Voyager Therapeutics Ralf Reilmann, MD, PhD — George Huntington Institute Sarah Tabrizi, MBChB, PhD — University College London Leslie Thompson, PhD — University of California Irvine Huntington Study Group Shari Kinel, JD — HSG Executive Director Liz McCarthy, BA — HSG Director, Special Programs Kristin Strazdins — HSG Accounting and Office Manager Stevan Ramirez — HSG Director, Finance & Operations Heather Hare — HSG Director, Communications & Outreach For more information on HD Insights, or to receive a free subscription, please visit us at www.hdinsights.org or email us at editor@hdinsights.org. Publication Staff Meredith A. Achey, BM — Deputy Editor Robin Taylor — Production Editor Martin Holmes — Technical Editor Lise Munsie, PhD — Scientific Content Reviewer David Kolko — Distribution Specialist Copyright © Huntington Study Group 2016. All rights reserved. These new sleep results in patients are in line with reports from transgenic rodent models of HD that report gradually worsening sleep quality from a very early stage, and together raise many interesting questions. What causes these early sleep disturbances? What is their significance in the early cognitive deficits and the onset and progression of the disease? These questions remain to be investigated. Finally, it remains to be established whether therapeutic sleep quality improvement could help premanifest and manifest patients with HD to reduce the cognitive symptoms or even slow down the disease process, as suggested by earlier studies performed in transgenic animal models of HD. 17,18 This study was supported by the CHDI Foundation (CHDI-RG50786). Sleep disturbances, cont... H D I N S I G H T S Contributors Roger A. Barker, MRCP, PhD, FMedSci Priya Hays, PhD Erika L. F. Holzbaur, PhD Baljit S. Khakh, PhD Carolin A. M. Koriath, MD Alpar S. Lazar, PhD Jong-Min Lee, PhD George McNally, BMedSc Davina J. Hensman Moss, BA, MBBS Lise Munsie, PhD Michael V. Sofroniew, MD, PhD Sarah J. Tabrizi, MBChB, PhD Yvette C. Wong, PhD 1 Scullin MK, Bliwise DL. Sleep, cognition, and normal aging: integrating a half century of multidisciplinary research. Perspect Psychol Psy. 2015;10(1):97-137. 2 Yaffe K, Falvey CM, Hoang T. Connections between sleep and cognition in older adults. Lancet Neurol. 2014;13(10):1017-1028. 3 Videnovic A, Lazar AS, Barker RA, Overeem S. 'The clocks that time us'-circadian rhythms in neurodegenerative disorders. Nat Rev Neurol. 2014;10(12):683-693. 4 Ju YE, Lucey BP, Holtzman DM. Sleep and Alzheimer disease pathology--a bidirectional relationship. Nat Rev Neurol. 2014;10(2):115-119. 5 Anderson KN, Bradley AJ. Sleep disturbance in mental health problems and neurodegenerative disease. Nat Sci Sleep. 2013;5:61-75. 6 Morton AJ. Circadian and sleep disorder in Huntington's disease. Exp Neurol. 2013;243:34-44. 7 Arnulf I, Nielsen J, Lohmann E, et al. Rapid eye movement sleep disturbances in Huntington disease. Arch Neurol. 2008;65(4):482-488. 8 Wiegand M, Möller A, Lauer C, et al. Nocturnal sleep in Huntington's disease. J Neurol. 1991;238(4):203-208. 9 Piano C, Losurdo A, Della Marca G, et al. Polysomnographic Findings and Clinical Correlates in Huntington Disease. A Cross-sectional Cohort Study. Sleep. 2015;38(9):1489-95. 10 Hansotia P, Wall R, Berendes J. Sleep disturbances and severity of Huntington's disease. Neurol. 1985;35(11):1672-1674. 11 Neutel D, Tchikviladze M, Charles P, et al. Nocturnal agitation in Huntington disease is caused by arousal-related abnormal movements rather than by rapid eye movement sleep behavior disorder. Sleep Med. 2015;16(6):754-759. 12 Morton AJ, Wood NI, Hastings MH, Hurelbrink C, Barker RA, Maywood ES. Disintegration of the sleep-wake cycle and circadian timing in Huntington's disease. J Neurosci. 2005;25(1):157-163. 13 Goodman AO, Rogers L, Pilsworth S, et al. Asymptomatic sleep abnormalities are a common early feature in patients with Huntington's disease. Curr Neurol Neurosci. 2011;11(2):211-217. 14 Aziz NA, Pijl H, Frolich M, et al. Systemic energy homeostasis in Huntington's disease patients. J Neurol Neurosur PS. 2010;81(11):1233-1237. 15 Goodman AO, Murgatroyd PR, Medina-Gomez G, et al. The metabolic profile of early Huntington's disease--a combined human and transgenic mouse study. Exp Neurol. 2008;210(2):691-698. 16 Lazar AS, Panin F, Goodman AO, et al. Sleep deficits but no metabolic deficits in premanifest Huntington's disease. Ann Neurol. 2015;78(4):630-648. 17 Pallier PN, Maywood ES, Zheng Z, et al. Pharmacological imposition of sleep slows cognitive decline and reverses dysregulation of circadian gene expression in a transgenic mouse model of Huntington's disease. J Neurosci. 2007;27(29):7869-7878. 18 Pallier PN, Morton AJ. Management of sleep/wake cycles improves cognitive function in a transgenic mouse model of Huntington's disease. Brain Res. 2009;1279:90-98.

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