A Systematic Review of Neuropsychiatric Symptoms and Functional Capacity in Huntington’s Disease

Sellers, Jessie; Ridner, Sheila H.; Claassen, Daniel O.

doi:10.1176/appi.neuropsych.18120319

Cited by 25 publications

(16 citation statements)

References 51 publications

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“…Greater levels of apathy have also been found to be related to less independence, increased motor impairment, and more clinician-rated behavioral problems (i.e., anger, irritability, and depression) (40). In addition, a recently published systematic review found that the most common significant factors associated with decreased functional capacity were depression and apathy (41). Given the prevalence of apathy among persons with HD reported in the literature and a recent study finding that apathy was an independent determinant of unemployment in HD mutation carriers (17), future research should include a measure of apathy in the methodology and analyses.…”

Section: Limitations and Future Researchmentioning

confidence: 99%

Alcohol Use, Mental Health, and Functional Capacity as Predictors of Workplace Disability in a Cohort With Manifest Huntington’s Disease

Goh

You

Perin

et al. 2020

JNP

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Objective: Huntington's disease (HD) is an inherited neurodegenerative disease involving motor, cognitive, psychiatric, and behavioral impairments that eventually affect work-role functioning. There is limited research regarding predictors of workplace disability in HD. The authors examined predictors of work impairment and disability in a cross-sectional cohort of employed persons with symptomatic HD participating in the worldwide Enroll-HD study.Methods: The study sample (N=316) comprised individuals with manifest HD and a CAG repeat length range between 39 and 60 and were currently engaged in paid full-or parttime employment. Univariate and multivariate logistic regression analyses identified predictors and the effect of all predictors in a fully adjusted model. Results:Of the sample, 20.3% reported missing work due to HD, 60.1% reported experiencing impairment while working due to HD, 79.1% reported having work-related activity impairment due to HD, and 60.8% reported impairment in overall work productivity due to HD. Individuals had 25% higher odds of missing work time if they had a higher level of functional impairment (odds ratio=0.76, 95% CI=0.64, 0.91) and had three times greater odds of missing work if they were current alcohol drinkers, compared with nondrinkers (odds ratio52.86, 95% CI51.62, 5.03). Individuals with lower self-perceived mental health were also 5% more likely to experience impairment at work due to HD. Motor impairment was not a strong predictor of workplace disability.Conclusions: These findings provide important new knowledge that can inform the development of strategies or targeted intervention trials to support persons with symptomatic HD to maintain their work roles.

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Section: Limitations and Future Researchmentioning

confidence: 99%

Alcohol Use, Mental Health, and Functional Capacity as Predictors of Workplace Disability in a Cohort With Manifest Huntington’s Disease

Goh

You

Perin

et al. 2020

JNP

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“…Future studies should include more patients, with a greater diversity of motor symptoms, followed longitudinally to confirm its sensitivity to change over time. A body of research [1][2][3][4][5][6][7] has demonstrated multiple abnormalities in the somatosensory system in patients with dystonia, suggesting that altered sensory processing may lead to abnormal sensorimotor integration and contribute to motor symptoms. [8][9][10] At a behavioral level, somatosensory temporal discrimination threshold (STDT) is increased in dystonia, reflecting a deficit of temporal sensory processing.…”

Section: Discussionmentioning

confidence: 99%

“…Given inherent ceiling effects, 2,3 the TFC may not effectively measure functional status in HD patients with minimal motor or cognitive symptoms. The effects of neuropsychiatric symptoms are not clearly reflected in TFC scores 4 . These latter symptoms are common in HD, evident in almost all patients, 5,6 and impact the quality of life and caregiver burden 7‐9 .…”

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confidence: 98%

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Measuring Functional Status in Huntington's Disease

et al. 2020

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Background Neuropsychiatric and cognitive symptoms account for substantial morbidity in Huntington's disease (HD), but their impact on functional status may not be captured using the Total Functional Capacity (TFC) scale. The objective of this study was to assess the impact of motor, cognitive, and neuropsychiatric symptoms on functional status in persons with HD, comparing two instruments. Methods Multiple regression analyses assessed the relationship between neuropsychiatric, cognitive, and motor symptoms and functional status as measured using TFC and Adult Functional Adaptive Behavior (AFAB) scales. Results Greater burden of neuropsychiatric (P = 0.017), cognitive (P = 0.001), and motor (P = 0.001) symptoms was associated with greater impairments to functional status as measured by the AFAB scale. Only motor symptoms were associated with TFC scores (P = 0.002). The 3 symptom domains explained more of the variance in AFAB than TFC scores (P = 0.016). Conclusions TFC may have limited applicability, particularly in early‐stage HD patients, as a measure of functional status. The AFAB scale can be used in HD studies as a more holistic measure of functional status. © 2020 International Parkinson and Movement Disorder Society

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“…Psychiatric symptoms are also present in many neurological disorders. For example, depression and anxiety are common in different neurodegenerative disorders such as Parkinson’s disorder (PD), Alzheimer’s disease (AD), and Huntington’s disease (PD), as well as after stroke and traumatic brain injury (Ayerbe et al, 2013 ; Arciniegas and Wortzel, 2014 ; van Duijn et al, 2014 ; Zhao et al, 2016 ; Seppi et al, 2019 ; Sellers et al, 2020 ).…”

Section: Introductionmentioning

confidence: 99%

The Unfolded Protein Response and Autophagy as Drug Targets in Neuropsychiatric Disorders

Srinivasan

Korhonen

Lindholm

2020

Front. Cell. Neurosci.

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Neurons are polarized in structure with a cytoplasmic compartment extending into dendrites and a long axon that terminates at the synapse. The high level of compartmentalization imposes specific challenges for protein quality control in neurons making them vulnerable to disturbances that may lead to neurological dysfunctions including neuropsychiatric diseases. Synapse and dendrites undergo structural modulations regulated by neuronal activity involve key proteins requiring strict control of their turnover rates and degradation pathways. Recent advances in the study of the unfolded protein response (UPR) and autophagy processes have brought novel insights into the specific roles of these processes in neuronal physiology and synaptic signaling. In this review, we highlight recent data and concepts about UPR and autophagy in neuropsychiatric disorders and synaptic plasticity including a brief outline of possible therapeutic approaches to influence UPR and autophagy signaling in these diseases.

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A Systematic Review of Neuropsychiatric Symptoms and Functional Capacity in Huntington’s Disease

Cited by 25 publications

References 51 publications

Alcohol Use, Mental Health, and Functional Capacity as Predictors of Workplace Disability in a Cohort With Manifest Huntington’s Disease

Alcohol Use, Mental Health, and Functional Capacity as Predictors of Workplace Disability in a Cohort With Manifest Huntington’s Disease

Measuring Functional Status in Huntington's Disease

The Unfolded Protein Response and Autophagy as Drug Targets in Neuropsychiatric Disorders

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