Changes in nocturnal sleep in Huntington's and Parkinson's disease

Emser, W.; Brenner, M.; Stober, T.; Schimrigk, К.

doi:10.1007/bf00314313

Cited by 94 publications

(62 citation statements)

References 12 publications

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“…Wiegand (28) demonstrated polysomnographic findings similar to other dementing illnesses including decreased slow-wave sleep, prolonged sleep latency, and decreased sleep efficiency. As have other authors (29), this study found a higher density of sleep spindles in HD subjects compared to control subjects. Mr. K., in case 2, suffered from chronic insomnia.…”

Section: Discussionsupporting

confidence: 75%

Early Psychiatric Manifestations of Huntington's Disease: More Than Just a Movement Disorder

Leroi¹,

Michalon²

1997

DMJ

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Huntington's disease (HD), a neurodegenerative condition of the central nervous system, poses a major challenge for clinicians. The clinical presentation is variable and consists of cognitive, psychiatric and motor symptoms. The psychiatric manifestations often appear many years before the movement disorder. Lack of recognition of the psychiatric mani-festations may lead to delays in diagnosis and multidisciplinary interventions. The aim of this article is to highlight the psychiatric aspects of HD, particularly focusing on the early presenta-tion. Two cases of HD with prominent psychiatric symptoms are used to illustrate the preva-lence of these aspects of the disease. In the absence of treatment directly influencing the course of HD, psychiatrists remain central to the multidisciplinary team diagnosing and treating this debilitating condition.Huntington's disease (HD), which was first described by George Huntington in 1872, is a neurodegenerative disorder which presents as a syndrome of motor abnormalities, cognitive decline and neuropsychiatric symptoms. HD has an autosomal dominant pattern of in-heritance with complete penetrance. The gene for the disease was identified in March, 1993 (1,2), enabling predictive and prenatal testing for those at risk of inheriting the disease (3). The disease affects 5 to 8 per 100,000 in North America (4,5). The average age of onset is be-tween 35 and 40 years of age, however, juve-nile forms of the disease can result in symp-toms as early as age 4 (6).The course of HD is progressive and is marked by increasing motor difficulties, in-cluding chorea, dystonia and athetosis, as well as bradykinesia, dysarthria, dysphagia, and urinary incontinence (7). Death results an average of 17 years after onset of the disease and is caused by cardiac and pulmonary complica-tions, suicide, nutritional deficiencies and self-destructive behaviours (8).The HD gene (referred to as IT15), found on the short arm of chromosome 4, partly consists of an unstable triplicate repeat (CAG) sequence. The number of triplicate repeats is higher (more than 38 repeats) in those with HD than in the normal population, which has 11-38 repeats. The mRNA transcribed from the HD gene can be found in all organs of an affected person and codes for a protein which has been called "huntingtin". The relationship between this protein and the development of the pathological changes in HD is still unclear, although several studies have attempted to define the neurophysiology and neuropathology. Neuropathology reveals early and promi-nent degeneration of the caudate nucleus. Later cell loss occur in the globus pallidus, subthalamic nucleus, nucleus accumbens, cerebellum and parts of the cortex (9). Within the caudate nucleus, abnormalities of small-to-medium sized spiny neurons result in loss of the inhibitory neurotransmitter gamma aminobutyric acid (GABA) as well as substance P and enkephalins. There is relative sparing of somatostatin and dopamine (10).HD is generally considered a "neurologic" illness, falling ...

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Section: Discussionsupporting

confidence: 75%

Early Psychiatric Manifestations of Huntington's Disease: More Than Just a Movement Disorder

Leroi¹,

Michalon²

1997

DMJ

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“…This finding is consistent with our and other groups' prior findings (Emser et al, 1988;Christensen et al, 2014a;Latreille et al, 2015), but contradicts those of other studies (Happe et al, 2004). According to Braak et al (2003), the neurodegenarative progress in PD shows a progressive ascending course starting from the brain stem and spreading to additional brain structures.…”

Section: Discussionsupporting

confidence: 35%

“…Reduced sleep spindle (SS) density and activity have been identified in patients with PD and iRBD (Puca et al, 1973;Myslobodsky et al, 1982;Emser et al, 1988;Comella et al, 1993;Christensen et al, 2014a;Latreille et al, 2015). SS are generated by a complex interaction involving thalamic, limbic, and cortical areas.…”

Section: Introductionmentioning

confidence: 99%

Sleep spindle alterations in patients with Parkinson's disease

et al. 2015

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“…Sleep pattern was found to be normal in patients of Huntington’s disease when compared to controls and SSDs were significantly increased in these patients [20]. Piano et al [21] had documented fragmented sleep, increased periodic limb movements, sleep apnea and daytime sleepiness in Huntington’s disease.…”

Section: Discussionmentioning

confidence: 99%

Assessment of Sleep Spindle Density among Genetically Positive Spinocerebellar Ataxias Types 1, 2, and 3 Patients

Seshagiri

Botta²,

Sasidharan³

et al. 2018

Ann Neurosci

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Objective: The effect of thalamic degeneration in patients with spinocerebellar ataxias (SCA) and sleep spindle (SS) abnormalities has not been studied so far, although there is a strong association between these disorders. This study was done to evaluate and compare the SS densities (SSDs) of genetically proven autosomal dominant SCA1, SCA2 and SCA3 patients with controls. Methods: Prospectively and genetically confirmed cases of SCA and controls were recruited. Patients were assessed clinically, were evaluated with sleep questionnaires and an overnight polysomnography was performed. SSDs were analyzed using neuroloop gain plugin of Polyman version 1.15 software. Results: Eighteen patients of SCA1 (n = 6), SCA2 (n = 5), SCA3 (n = 7) and 6 controls were recruited in our study. The mean age of SCA1 patients was 39.2 ± 5.4, of SCA2 patients was 30.8 ± 9.5 and of SCA3 patients was 35.4 ± 6.4 years. The mean duration of illness in SCA1 was 4.7 ± 1.7 years, in SCA2 it was 4.3 ± 4.4 years and in SCA3 it was 5 ± 2.3 years. The median SSD values (percentage loop gain) during stage 2 of non-rapid eye movement sleep were 16.9% in SCA1, 0% in SCA2, 1.2% in SCA3 and 59.5% in controls. There was a significant difference in SSD values in SCA2 (p = 0.04), SCA3 (p = 0.02) patients and controls. Conclusion: SSDs were significantly decreased in patients with SCA, which is a novel finding. This is likely due to the “thalamic switch” disruption, observed as reduced SSDs in SCA2 and SCA3. Sleep spindle deficits could act as one of the biomarkers of ongoing neurodegeneration in the thalamic circuitry of SCA patients.

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Changes in nocturnal sleep in Huntington's and Parkinson's disease

Cited by 94 publications

References 12 publications

Early Psychiatric Manifestations of Huntington's Disease: More Than Just a Movement Disorder

Early Psychiatric Manifestations of Huntington's Disease: More Than Just a Movement Disorder

Sleep spindle alterations in patients with Parkinson's disease

Assessment of Sleep Spindle Density among Genetically Positive Spinocerebellar Ataxias Types 1, 2, and 3 Patients

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