How vital is sleep in Huntington's disease?

Anna O G Goodman1, Roger A Barker

  • 1Cambridge Centre for Brain Repair, University of Cambridge, Cambridge, UK. aogr2@cam.ac.uk

Journal of Neurology
|March 25, 2010
PubMed

Insights

Huntington's disease (HD), a fatal neurodegenerative disorder, involves CAG repeat expansion. This review focuses on how HD impacts sleep and circadian rhythms, key aspects often overlooked in patients.

Area of Science:

  • Neuroscience
  • Genetics
  • Sleep Medicine

Background:

  • Huntington's disease (HD) is a fatal, inherited neurodegenerative disorder.
  • It stems from an abnormal CAG repeat expansion in the HD gene on chromosome 4.
  • HD typically presents with motor, cognitive, and psychiatric symptoms, but also affects sleep and circadian rhythms.

Purpose of the Study:

  • To review the impact of Huntington's disease on sleep and circadian rhythms.
  • To highlight the significance of these disruptions in the overall disease course.

Main Methods:

  • Literature review of studies on Huntington's disease and its effects on sleep and circadian patterns.
  • Analysis of reported symptoms and biological changes related to sleep.

Main Results:

  • Huntington's disease significantly disrupts sleep architecture and circadian timing.
  • Patients often experience insomnia, altered sleep duration, and daytime somnolence.
  • Changes in melatonin secretion and body temperature rhythms are frequently observed.

Conclusions:

  • Sleep and circadian rhythm disturbances are integral features of Huntington's disease progression.
  • Addressing these non-motor symptoms may improve patient quality of life.
  • Further research into the mechanisms and treatments for HD-related sleep issues is warranted.

Related Concept Videos

Huntington Disease l: Introduction01:21

Huntington Disease l: Introduction

Huntington disease or HD is a progressive, fatal neurodegenerative disorder inherited in an autosomal dominant pattern.PathophysiologyIt is caused by expansion of the CAG trinucleotide repeat in the HTT gene on chromosome 4 (4p16.3), producing an abnormal huntingtin protein with an expanded polyglutamine tract. This misfolded protein disrupts cellular function, leading to neuronal death. Normal alleles have ≤26 repeats, 27–35 are intermediate (risk of expansion), 36–39 show reduced penetrance,...
Understanding Sleep01:11

Understanding Sleep

Sleep, an essential biological state, involves significant reductions in physical activity, sensory awareness, and interaction with the environment. This complex physiological process is primarily regulated by specific brain regions, notably the hypothalamus and pons, which govern the sleep-wake cycle or circadian rhythm.
The circadian rhythm, a nearly 24-hour cycle, is deeply influenced by environmental light cues. Light exposure directly affects the hypothalamus, which in turn regulates...
Insufficient Sleep and Sleep Deprivation01:13

Insufficient Sleep and Sleep Deprivation

Insufficient sleep refers to not getting the recommended amount of sleep for optimal functioning, even if it's just slightly less than needed. Sleep insufficiency may occur due to lifestyle choices, such as staying up late for social events or work, resulting in routinely getting less sleep than required. For example, consistently sleeping 6 hours when the body needs 7-9 hours can lead to cumulative effects on health and well-being.
Sleep deprivation is a more severe form of sleep loss...
Substance Use Disorders Affecting Sleep01:24

Substance Use Disorders Affecting Sleep

Substance use disorders involve a pattern of using drugs more extensively than intended and continuing use despite harmful consequences. This includes legal substances like alcohol and nicotine, as well as illegal drugs. These disorders often involve both physical and psychological dependence, reflecting compulsive use of substances that significantly alter thoughts, feelings, and behaviors, contributing to a major public health issue.
Understanding the concepts of physical dependence,...
Sleep-Wake Cycles01:24

Sleep-Wake Cycles

Sleep is an essential physiological process vital to maintaining overall well-being. The reticular activating system (RAS), a network of neurons in the brainstem, regulates wakefulness and sleep. While it may seem passive, sleep consists of distinct cycles, each with its unique characteristics and functions. Two key sleep phases are non-rapid eye movement (NREM) and  rapid eye movement (REM).
NREM Sleep
NREM sleep comprises four progressive stages that seamlessly merge:
Narcolepsy01:07

Narcolepsy

Narcolepsy is a chronic sleep disorder characterized by pervasive, uncontrolled sleepiness and other sleep disturbances. One of its hallmark symptoms is an abrupt transition to REM sleep upon falling asleep, which causes symptoms typically associated with this phase to occur unexpectedly during wakefulness. These include the following symptoms, which typically last from a minute or two to half an hour.