Skip to main content
SpringerLink
Log in

Asymptomatic Sleep Abnormalities Are a Common Early Feature in Patients with Huntington’s Disease

  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Huntington’s disease (HD) is a fatal neurodegenerative disease characterized by motor, cognitive, and psychiatric disturbance. In this article, we used polysomnography, actigraphy and a variety of validated questionnaires to ascertain the extent to which sleep changes are identifiable and measurable in mild stage HD, and importantly, to see whether patients are negatively impacted by the changes in their sleep. We found significant differences in sleep architecture and sleep efficiency in patients compared with controls using polysomnography. However, patient scores on the Functional Outcomes of Sleep Questionnaire, Medical Outcomes of Sleep Scale, and Epworth Sleepiness Scale were not significantly different to controls. These results suggest that although marked changes in sleep architecture are present in early HD and can be detected using polysomnography, patients do not necessarily recognize or report these abnormalities.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. The Huntington’s Disease Collaborative Research Group. A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell 1993; 72:971–983.

    Article  Google Scholar 

  2. Foroud T, Gray J, Ivashina J, et al. Differences in duration of Huntington’s disease based on age at onset. J Neurol Neurosurg Psychiatry 1999;66:52–6.

    Article  PubMed  CAS  Google Scholar 

  3. Bamford KA, Caine ED, Kido DK, et al. A prospective evaluation of cognitive decline in early Huntington’s disease: functional and radiographic correlates. Neurology 1995;45:1867–73.

    PubMed  CAS  Google Scholar 

  4. Shiwach R. Psychopathology in Huntington’s disease patients. Acta Psychiatr Scand 1994;90:241–6.

    Article  PubMed  CAS  Google Scholar 

  5. Bruyn GW, von Wolferen WJ. Pathogenesis of Huntington’s chorea. Lancet 1973;1:1382.

    Article  PubMed  CAS  Google Scholar 

  6. Hansotia P, Wall R, Berendes J. Sleep disturbances and severity of Huntington’s disease. Neurology 1985;35:1672–4.

    PubMed  CAS  Google Scholar 

  7. Petit D, Gagnon JF, Fantini ML, et al. Sleep and quantitative EEG in neurodegenerative disorders. J Psychosom Res 2004;56:487–96.

    Article  PubMed  Google Scholar 

  8. • Arnulf I, Nielsen J, Lohmann E, et al. Rapid eye movement sleep disturbances in Huntington disease. Arch Neurol 2008;65:482–8. The majority of studies on sleep in HD disease were published over 10 years ago. This is the most recent study published to date and provides evidence to suggest that not only is REM sleep affected in the disease but that some of these changes may actually precede chorea.

    Article  PubMed  Google Scholar 

  9. Iakhno NN. Disorders of nocturnal sleep in Huntington chorea. Zh Nevropatol Psikhiatr Im S S Korsakova 1985;85:340–6.

    PubMed  CAS  Google Scholar 

  10. Silvestri R, Raffaele M, De Domenico P, et al. Sleep features in Tourette’s syndrome, neuroacanthocytosis and Huntington’s chorea. Neurophysiol Clin 1995;25:66–77.

    Article  PubMed  CAS  Google Scholar 

  11. Mena-Segovia J, Cintra L, Prospero-Garcia O, et al. Changes in sleep-waking cycle after striatal excitotoxic lesions. Behav Brain Res 2002;136:475–81.

    Article  PubMed  Google Scholar 

  12. Wiegand M, Moller AA, Lauer CJ, et al. Nocturnal sleep in Huntington’s disease. J Neurol 1991;238:203–8.

    Article  PubMed  CAS  Google Scholar 

  13. • Goodman AO, Barker RA. How vital is sleep in Huntington’s disease? J Neurol 2010. This paper provides a thorough review of the literature published to date on sleep in HD.

  14. Van Someren EJ, Swaab DF, Colenda CC, et al. Bright light therapy: improved sensitivity to its effects on rest-activity rhythms in Alzheimer patients by application of nonparametric methods. Chronobiol Int 1999;16:505–18.

    Article  PubMed  Google Scholar 

  15. Rechtschaffen A, Kales A. A manual of standardized terminology, technique and scoring system for sleep stages of human sleep. Los Angeles: Brain Information Service, 1968.

    Google Scholar 

  16. Sleep-related breathing disorders in adults: recommendations for syndrome definition and measurement techniques in clinical research. The Report of an American Academy of Sleep Medicine Task Force. Sleep 1999;22:667–89.

  17. Zucconi M, Ferri R, Allen R, et al. The official World Association of Sleep Medicine (WASM) standards for recording and scoring periodic leg movements in sleep (PLMS) and wakefulness (PLMW) developed in collaboration with a task force from the International Restless Legs Syndrome Study Group (IRLSSG). Sleep Med 2006;7:175–83.

    Article  PubMed  Google Scholar 

  18. Littner MR, Kushida C, Wise M et al. Practice parameters for clinical use of the multiple sleep latency test and the maintenance of wakefulness test. Sleep 2005;28:113–121.

    PubMed  Google Scholar 

  19. Carskadon MA, Dement WC, Mitler MM et al. Guidelines for the multiple sleep latency test (MSLT): a standard measure of sleepiness. Sleep 1986;9,519–524.

    PubMed  CAS  Google Scholar 

  20. Johns MW. A new method for measuring daytime sleepiness: the Epworth sleepiness scale. Sleep 1991;14:540–5.

    PubMed  CAS  Google Scholar 

  21. Johns MW. Reliability and factor analysis of the Epworth Sleepiness Scale. Sleep 1992;15:376–81.

    PubMed  CAS  Google Scholar 

  22. Ellenbogen JM. Cognitive benefits of sleep and their loss due to sleep deprivation. Neurology 2005;64:E25–E27.

    PubMed  Google Scholar 

  23. Foster RG, Wulff K. The rhythm of rest and excess. Nat Rev Neurosci 2005;6:407–14.

    Article  PubMed  CAS  Google Scholar 

  24. Laureys S, Peigneux P, Perrin F, et al. Sleep and motor skill learning. Neuron 2002;35:5–7.

    Article  PubMed  CAS  Google Scholar 

  25. Schernhammer ES, Laden F, Speizer FE, et al. Night-shift work and risk of colorectal cancer in the nurses’ health study. J Natl Cancer Inst 2003;95:825–8.

    Article  PubMed  Google Scholar 

  26. Sephton S, Spiegel D. Circadian disruption in cancer: a neuroendocrine-immune pathway from stress to disease? Brain Behav Immun 2003;17:321–8.

    Article  PubMed  CAS  Google Scholar 

  27. Spiegel K, Leproult R, Van Cauter E. Impact of sleep debt on metabolic and endocrine function. Lancet 1999;354:1435–9.

    Article  PubMed  CAS  Google Scholar 

  28. Spiegel K, Tasali E, Penev P, et al. Brief communication: Sleep curtailment in healthy young men is associated with decreased leptin levels, elevated ghrelin levels, and increased hunger and appetite. Ann Intern Med 2004;141:846–50.

    PubMed  Google Scholar 

  29. Taheri S, Lin L, Austin D, et al. Short sleep duration is associated with reduced leptin, elevated ghrelin, and increased body mass index. PLoS Med 2004;1:e62.

    Article  PubMed  Google Scholar 

  30. 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;27:7869–78.

    Article  PubMed  CAS  Google Scholar 

  31. • Pallier PN, Morton AJ. Management of sleep/wake cycles improves cognitive function in a transgenic mouse model of Huntington’s disease. Brain Res 2009;1279:90–8. This paper illustrates the profound beneficial effects associated with improving sleep and circadian rhythms in the R6/2 transgenic mouse model of HD. The implication associated with this study is that treating patients with the disease may well result in a significant improvement in cognitive performance and other symptoms.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgment

We wish to thank all of our participating patients and control subjects for their help. This work was supported by donations from patients and their families. All research carried out was given full ethical approval from the Cambridge Ethics Committee.

Disclosure

No potential conflicts of interest relevant to this article were reported.

Author information

Authors and Affiliations

  1. Cambridge Centre for Brain Repair, Department of Clinical Neurosciences, Addenbrooke’s Hospital, Hills Road, Cambridge, CB2 0QQ, United Kingdom

    Anna O. G. Goodman & Roger A. Barker

  2. Respiratory Support and Sleep Centre, Papworth Hospital, Papworth Everard, Cambridge, CB23 8RE, United Kingdom

    Lorraine Rogers, Samantha Pilsworth & John M. Shneerson

  3. Department of Pharmacology, University of Cambridge, Tennis Court Road, Cambridge, CB2 1PD, United Kingdom

    Catherine J. McAllister & A. Jennifer Morton

  4. Cambridge Centre for Brain Repair, E. D. Adrian Building, Forvie Site, Robinson Way, Cambridge, CB2 0PY, United Kingdom

    Anna O. G. Goodman

Authors
  1. Anna O. G. Goodman
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lorraine Rogers
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Samantha Pilsworth
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Catherine J. McAllister
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. John M. Shneerson
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A. Jennifer Morton
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Roger A. Barker
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Anna O. G. Goodman.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Goodman, A.O.G., Rogers, L., Pilsworth, S. et al. Asymptomatic Sleep Abnormalities Are a Common Early Feature in Patients with Huntington’s Disease. Curr Neurol Neurosci Rep 11, 211–217 (2011). https://doi.org/10.1007/s11910-010-0163-x

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11910-010-0163-x

Keywords

Search

Navigation