Sleep disturbances and severity of Huntington's disease

Hansotia, Phiroze; Wall, Richard A.; Berendes, J

doi:10.1212/wnl.35.11.1672

Cited by 97 publications

(52 citation statements)

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“…Mild stage patients reportedly have no clinical sleep disturbance, but do have mild PSG abnormalities, with increased interspersed wakefulness and a longer time to first REM episode [3,61]. We have also seen something similar in unpublished data from eight patients in the early stages of the disease.…”

Section: Polysomnography In Huntington's Diseasesupporting

confidence: 71%

“…To date, sleep studies in HD patients provide evidence of a progressively worsening sleep disorder [123], which appears to be independent of CAG repeat length [3,61]. Mild stage patients reportedly have no clinical sleep disturbance, but do have mild PSG abnormalities, with increased interspersed wakefulness and a longer time to first REM episode [3,61].…”

Section: Polysomnography In Huntington's Diseasementioning

confidence: 99%

“…Patients with moderate disease also experience increased time in Stages 1 and 2 of sleep [61] and reduced SWS and REM sleep [61,157,177]. REM sleep percentages have been shown to decrease with disease severity [3] and, in contrast to other neurodegenerative diseases, HD patients show a higher density of sleep spindles compared to healthy control subjects [36,157,177].…”

Section: Polysomnography In Huntington's Diseasementioning

confidence: 99%

“…REM sleep percentages have been shown to decrease with disease severity [3] and, in contrast to other neurodegenerative diseases, HD patients show a higher density of sleep spindles compared to healthy control subjects [36,157,177]. Moderately severe HD patients experience impaired initiation of sleep with increased sleep onset latency [177], as well as impaired maintenance of sleep with increased nocturnal awakenings or arousals and a high percentage of wakefulness after sleep onset [61,157,177]. Thus, HD patients experience reduced sleep efficiency [19,61,100,157,177].…”

Section: Polysomnography In Huntington's Diseasementioning

confidence: 99%

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How vital is sleep in Huntington’s disease?

Goodman

Barker

2010

J Neurol

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Huntington's disease (HD) is a fatal neurodegenerative disease caused by an abnormal expansion of a CAG repeat in exon 1 of the HD gene on chromosome 4. The disease runs a debilitating and progressive course with an average survival of 15-25 years after disease onset. HD patients classically develop involuntary movements including chorea, as well as progressive cognitive and psychiatric disturbances, although a number of other features have also been reported, including changes in sleep and circadian rhythms; it is this latter area that forms the focus of this review.

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Section: Polysomnography In Huntington's Diseasesupporting

confidence: 71%

Section: Polysomnography In Huntington's Diseasementioning

confidence: 99%

Section: Polysomnography In Huntington's Diseasementioning

confidence: 99%

Section: Polysomnography In Huntington's Diseasementioning

confidence: 99%

See 2 more Smart Citations

How vital is sleep in Huntington’s disease?

Goodman

Barker

2010

J Neurol

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“…Nevertheless, given that abnormal movements are a core feature of the disease in the majority of HD patients, a means of objectively measuring these movements would be ideal in helping to monitor the progression of the disease as well as assessing the success of potential future symptomatic and neuroprotective treatments. In addition by recording over a 48 hour period, it may also be able to provide useful information on sleep-wake activity in this condition, which is of relevance given that abnormalities in sleep have been reported in some patients with HD [5,12,20].…”

Section: Introductionmentioning

confidence: 99%

The use of the Actiwatch–Neurologica® system to objectively assess the involuntary movements and sleep–wake activity in patients with mild–moderate Huntington’s disease

Hurelbrink¹,

Lewis

Barker

2005

J Neurol

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Huntington's disease (HD) is a neurodegenerative disorder characterised by cognitive, psychiatric and motor abnormalities including a range of involuntary movements. Currently, assessment of these movements involves the use of subjective rating scales such as the Unified Huntington's Disease Rating Scales (UHDRS) for bradykinesia and maximal dystonia and chorea, without any objective measures. As new therapies emerge, it is critical that an objective means of evaluating these abnormal movements is developed and we have investigated the use of a wrist-worn activity monitor, the Actiwatch-Neurologica, to determine whether these movements can be measured. In addition, this activity monitor and subjective reports were used to objectively measure the degree of sleep disruption in these same HD patients. Eight patients with mild-moderate HD and 8 age- and sex-matched control subjects wore the monitor for a period of 48 hours and recorded in a diary whether they were asleep or awake for each hour over the 2-day period. Assessment of various movement parameters revealed that HD patients exhibited significantly greater total and maximum activity levels and spent longer performing high acceleration movements while they were awake compared with controls. During sleep, patients not only showed significantly more activity and spent more time making high acceleration movements, but they also made significantly more movements than control subjects. These results demonstrate that the Actiwatch-Neurologica activity monitor can be used to objectively assess movements in HD patients during periods of high activity as well as during sleep.

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Rapid Eye Movement Sleep Disturbances in Huntington Disease

Arnulf

Nielsen²,

Lohmann³

et al. 2008

Arch Neurol

197

146

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Background: Sleep disorders including insomnia, movements during sleep, and daytime sleepiness are common but poorly studied in Huntington disease (HD).Objective: To evaluate the HD sleep-wake phenotype (including abnormal motor activity during sleep) in patients with various HD stages and the length of CAG repeats. Because a mild hypocretin deficiency has been found in the brains of some patients with HD (hereinafter referred to as HD patients), we also tested the HD patients for narcolepsy. Design and Patients:Twenty-five HD patients (including 2 premanifest carriers) underwent clinical interview, nighttime video and sleep monitoring, and daytime multiple sleep latency tests. Their results were compared with those of patients with narcolepsy and control patients. Results: The HD patients had frequent insomnia, earlier sleep onset, lower sleep efficiency, increased stage 1 sleep, delayed and shortened rapid eye movement (REM) sleep, and increased periodic leg movements. Three HD patients (12%) had REM sleep behavior disorders. No sleep abnormality correlated with CAG repeat length. Reduced REM sleep duration (but not REM sleep behavior disorders) was present in premanifest carriers and patients with very mild HD and worsened with disease severity. In contrast to narcoleptic patients, HD patients had no cataplexy, hypnagogic hallucinations, or sleep paralysis. Four HD patients had abnormally low (Ͻ8 minutes) daytime sleep latencies, but none had multiple sleeponset REM periods. Conclusions: The sleep phenotype of HD includes insomnia, advanced sleep phase, periodic leg movements, REM sleep behavior disorders, and reduced REM sleep but not narcolepsy. Reduced REM sleep may precede chorea. Mutant huntingtin may exert an effect on REM sleep and motor control during sleep.

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Sleep disturbances and severity of Huntington's disease

Cited by 97 publications

References 0 publications

How vital is sleep in Huntington’s disease?

How vital is sleep in Huntington’s disease?

The use of the Actiwatch–Neurologica® system to objectively assess the involuntary movements and sleep–wake activity in patients with mild–moderate Huntington’s disease

Rapid Eye Movement Sleep Disturbances in Huntington Disease

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