13 research outputs found

    Hypokinesia in Huntington's Disease Co-occurs with Cognitive and Global Dysfunctioning

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    Besides chorea, hypokinesia is an important motor disturbance in Huntington's disease (HD) but its clinical, neuropsychiatric, and cognitive functioning correlates are largely unknown. This cross-sectional study investigates correlates of hypokinesia in HD and its effect on global functioning. Among 150 HD gene carriers, 96 patients were clinically motor manifest. Hypokinesia was assessed using the motor section of the Unified Huntington's Disease Rating Scale and global functioning was measured using the Total Functioning Capacity (TFC) scale. Neuropsychiatric measures included the Apathy Scale and the Composite International Diagnostic Interview for diagnosis of depression. The Mini Mental State Examination (MMSE) and a composite executive cognitive measure were used to assess global and executive cognitive functioning, respectively. Compared with 45 patients with no or mild hypokinesia, 51 patients with moderate to severe hypokinesia showed a significant difference in most clinical and neuropsychiatric variables and had worse cognitive functioning scores. However, using forward logistic regression analysis, poor executive cognitive functioning was the only independent correlate of hypokinesia (OR 7.33; 95% CI: 2.82-19.0; P < 0.001). Hypokinesia score was inversely associated with the TFC score (P < 0.001), also after adjusting for chorea, use of antipsychotics, apathy, and global and executive cognitive functioning. In conclusion, the presence of moderate to severe hypokinesia in HD patients co-occurs with executive cognitive dysfunction and adversely affects global functioning. (C) 2010 Movement Disorder SocietyStress-related psychiatric disorders across the life spa

    Correlates of Apathy in Huntington's Disease

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    The authors aim to study prevalence and clinical correlates of apathy in Huntington's disease. Apathy was defined as an Apathy Scale score >= 14 points in 152 Huntington's disease mutation carriers and 56 noncarriers. Correlates of apathy were analyzed cross-sectionally in mutation carriers using multivariable logistic regression analysis. Forty-nine (32%) Huntington's disease mutation carriers showed apathy compared to none of the noncarriers. After exclusion of 10 depressed patients, apathy was independently associated with male sex, worse global functioning, and higher use of neuroleptics and benzodiazepines. (The Journal of Neuropsychiatry and Clinical Neurosciences 2010; 22:287-294)Stress-related psychiatric disorders across the life spa

    INCIDENCE AND COURSE OF IRRITABILITY IN HUNTINGTON'S DISEASE

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    Stress-related psychiatric disorders across the life spa

    Irritability in Huntington's disease

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    Irritability is a frequent neuropsychiatric symptom in patients with Huntington's disease (HD). The Irritability Scale (IS) and the irritability factor of the Problem Behaviours Assessment (PBA) was used to assess irritability among 130 HD mutation carriers and 43 verified non-carriers. The IS was tested using receiver operating characteristic analysis against different cut-offs of the PBA irritability factor. A robust IS cut-off score of ≄14 points was found indicating that 45 (35%) of the 130 mutation carriers were irritable vs. 4 (9%) of the 43 non-carriers (P=0.001). The level of agreement between self-report and informant-report IS was of moderate strength (intraclass correlation=0.61). Using univariate and multivariate regression analyses, independent correlates of irritability were being married/living together (P=0.02), CAG repeat length (P=0.01), and use of benzodiazepines (P=0.008). Using the same model with the informant's irritability score, use of benzodiazepines was the only significant independent correlate of irritability (P=0.005). Irritability is a prominent symptom of HD and can be reliably assessed with the IS using a cut-off score ≄14 points. Although it is unclear whether benzodiazepine use causes irritability, or irritability leads to the prescription of benzodiazepines, clinical evaluation with respect to the use of benzodiazepines in HD warrants attention.Stress-related psychiatric disorders across the life spa

    Innovative approaches to environmental effects monitoring using an autonomous underwater vehicle

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    An overview is given of a project to develop autonomous underwater vehicle (AUV) technology for environmental effects monitoring (EEM) in the offshore oil and gas industry. This project is a joint venture between the Institute for Marine Dynamics of the National Research Council Canada (NRC-IMD) and the Ocean Engineering Research Centre at Memorial University of Newfoundland (MUN-OERC), with the support of several Canadian companies and universities. With the offshore oil and gas industry growing rapidly, it is important that new and innovative methods for EEM be considered. The paper reports on results from the project "Offshore Environmental Risk Engineering using Autonomous Underwater Vehicles" (OERE-AUV). The results include: (a) the development of a new general-purpose test-bed AUV called "C-SCOUT", (b) the planning for a series of sea trials using an existing vehicle to determine the effectiveness of an AUV to delineate a near-shore ocean outfall, (c) the characteristics and performance of several candidate sensors for EEM, and, (d) the development of hydrodynamic dispersion models for discharges into a marine environment. The ultimate application of the research is for the EEM of discharges of produced water, drilling cuttings and drilling muds from offshore oil and gas production facilities.NRC publication: Ye

    Gray matter vulnerabilities predict longitudinal development of apathy in Huntington's disease

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    Background: Apathy, a common neuropsychiatric disturbance in Huntington's disease (HD), is subserved by a complex neurobiological network. However, no study has yet employed a whole-brain approach to examine underlying regional vulnerabilities that may precipitate apathy changes over time. Objectives: To identify whole-brain gray matter volume (GMV) vulnerabilities that may predict longitudinal apathy development in HD. Methods: Forty-five HD individuals (31 female) were scanned and evaluated for apathy and other neuropsychiatric features using the short-Problem Behavior Assessment for a maximum total of six longitudinal visits (including baseline). In order to identify regions where changes in GMV may describe changes in apathy, we performed longitudinal voxel-based morphometry (VBM) on those 33 participants with a magnetic resonance imaging (MRI) scan on their second visit at 18 ± 6 months follow-up (78 MRI datasets). We next employed a generalized linear mixed-effects model (N = 45) to elucidate whether initial and specific GMV may predict apathy development over time. Results: Utilizing longitudinal VBM, we revealed a relationship between increases in apathy and specific GMV atrophy in the right middle cingulate cortex (MCC). Furthermore, vulnerability in the right MCC volume at baseline successfully predicted the severity and progression of apathy over time. Conclusions: This study highlights that individual differences in apathy in HD may be explained by variability in atrophy and initial vulnerabilities in the right MCC, a region implicated in action-initiation. These findings thus serve to facilitate the prediction of an apathetic profile, permitting targeted, time-sensitive interventions in neurodegenerative disease with potential implications in otherwise healthy populations
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