The Impact of Anosognosia on Clinical and Patient-Reported Assessments of Psychiatric Symptoms in Huntington’s Disease

2020 ◽  
Vol 9 (3) ◽  
pp. 291-302
Author(s):  
David Isaacs ◽  
Jessie S. Gibson ◽  
Jeffrey Stovall ◽  
Daniel O. Claassen
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Discriminant Validity ◽  
Rating Scales ◽  
Rating Scale ◽  
Psychiatric Symptoms ◽  
Self Report ◽  
Depression And Anxiety ◽  
Patient Reported ◽  
The Impact

Background: Psychiatric symptoms are widely prevalent in Huntington’s disease (HD) and exert greater impact on quality of life than motor manifestations. Despite this, psychiatric symptoms are frequently underrecognized and undertreated. Lack of awareness, or anosognosia, has been observed at all stages of HD and may contribute to diminished patient self-reporting of psychiatric symptoms. Objective: We sought to evaluate the impact of anosognosia on performance of commonly used clinical rating scales for psychiatric manifestations of HD. Methods: We recruited 50 HD patients to undergo a formal psychiatrist evaluation, the Problem Behavior Assessment-Short Form (PBA-s), and validated self-report rating scales for depression, anxiety, and anger. Motor impairment, cognitive function, and total functional capacity were assessed as part of clinical exam. Patient awareness of motor, cognitive, emotional, and functional capacities was quantified using the Anosognosia Rating Scale. Convergent validity, discriminant validity, classification accuracy, and anosognosia effect was determined for each psychiatric symptom rating scale. Results: Anosognosia was identified in one-third of patients, and these patients underrated the severity of depression and anxiety when completing self-report instruments. Anosognosia did not clearly influence self-reported anger, but this result may have been confounded by the sub-optimal discriminant validity of anger rating scales. Conclusion: Anosognosia undermines reliability of self-reported depression and anxiety in HD. Self-report rating scales for depression and anxiety may have a role in screening, but results must be corroborated by provider and caregiver input when anosognosia is present. HD clinical trials utilizing patient-reported outcomes as study endpoints should routinely evaluate participants for anosognosia.

F57 Psychiatric symptoms in huntington’s disease: correlations between interview and self-report measures in the capit-hd2 beta-testing study

2018 ◽  
Author(s):  
Isobel McMillan ◽  
Duncan McLauchlan ◽  
Monica Busse ◽  
Anne-Catherine Bachoud-Lévi ◽  
Ralf Reilmann ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Psychiatric Symptoms ◽  
Self Report ◽  
Beta Testing

Dysphagia characteristics in Huntington’s disease patients: insights from the Fiberoptic Endoscopic Evaluation of Swallowing and the Swallowing Disturbances Questionnaire

CNS Spectrums ◽  
2018 ◽  
Vol 24 (04) ◽  
pp. 413-418 ◽  
Author(s):  
Yael Manor ◽  
Yael Oestreicher-Kedem ◽  
Alona Gad ◽  
Jennifer Zitser ◽  
Achinoam Faust-Socher ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Oropharyngeal Dysphagia ◽  
Case Series ◽  
Solid Food ◽  
Self Report ◽  
Endoscopic Evaluation ◽  
Retrospective Case ◽  
Tertiary Referral Center

BackgroundHuntington’s disease (HD) is a neurodegenerative disease characterized by increasing dysphagia as the disease progresses. Specific characteristics of the HD dysphagia are not well defined.ObjectiveTo characterize the swallowing disturbances of HD patients, to evaluate the feasibility of Fiberoptic Endoscopic Evaluation of Swallowing (FEES) in assessing dysphagia in HD patients, and to discern the relation between FEES findings and patients’ self-report on dysphagia symptoms and swallowing related quality of life (SWAL-QOL).MethodA retrospective case series in a tertiary referral center. All recruited HD patients underwent Bed Side Swallowing Evaluation (BSE), FEES, the Unified Huntington’s Disease Rating Scale (UHDRS), and the Montreal Cognitive Assessment (MoCA). All completed the Swallowing Disturbances Questionnaire (SDQ) and the SWAL-QOL questionnaire.ResultsFourteen HD patients were recruited. All were able to complete the FEES study. The FEES demonstrated delayed swallowing reflex, solid food residues, and pre/post swallowing spillage in most patients (50%, 53.5%, 83.3%, and 87.5%, respectively). The mean SDQ score was 13.2. Significant correlations were found between the SWAL-QOL fear of eating score; the SDQ oral, pharyngeal, and total scores; and the FEES parameters of pureed and solid food bolus flow time. Significant correlations were also found between the total UHDRS score, the volitional cough score, and the SWAL-QOL disease burden score.ConclusionHD patients exhibit prominent unique oropharyngeal dysphagia features that may serve as a marker of disease progression. The FEES and the SDQ are valuable tools for detecting these features in HD patients with swallowing disturbance.

Abnormal gait and bradykinesia in the preclinical phase of Huntington's disease – psychogenic movement disorder?

2011 ◽  
Vol 23 (6) ◽  
pp. 315-317
Author(s):  
Witold Soltan ◽  
Emilia Sitek ◽  
Hubert Wichowicz ◽  
Dariusz Wieczorek ◽  
Jaroslaw Slawek
Keyword(s):  
Huntington's Disease ◽  
Movement Disorder ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Psychiatric Symptoms ◽  
Preclinical Phase ◽  
Abnormal Gait ◽  
Genetic Status ◽  
Psychogenic Movement Disorder

Soltan W, Sitek E, Wichowicz H, Wieczorek D, Slawek J. Abnormal gait and bradykinesia in the preclinical phase of Huntington's disease – psychogenic movement disorder?Objective: Psychiatric symptoms may occur in individuals at risk of Huntington's disease (HD) regardless of their genetic status. Psychopathological symptomatology is attributed to both genetic and environmental factors. In case of asymptomatic gene carriers, psychiatric symptoms may precede involuntary movements.Methods: We report the first case with abnormal gait and bradykinesia in preclinical adult HD. A 33-year-old woman blind to her mother's HD diagnosis and her own genetic status developed motor slowing and gait disturbance. The symptoms withdrew due to counselling and antidepressant medications. Subsequently, she was informed her own and her mother's genetic testing results, but 2-year follow-up did not reveal the onset of choreic movements, cognitive deterioration or depressive symptoms in the patient. Personality assessment (Minnesota Multiphasic Personality Inventory) and neurological examination results are presented, accompanied by 2-year follow-up data. Follow-up examination included Unified Huntington's Disease Rating Scale (motor, behaviour and function), Beck Depression Inventory, Hamilton Depression Rating Scale and neuropsychological assessment (trail-making test, Stroop test, verbal fluency trials, symbol digit modalities test, digit span, serial seven subtraction, Hopkins verbal learning test and nine-hole peg test).Conclusion: Motor abnormalities in individuals at risk of HD may be of psychogenic origin. It is a matter of debate if this psychogenic reaction presented as hypokinetic syndrome may be a result of choreic movements of her mother (hyperkinetic syndrome) and depression or if this psychogenic reaction represents the preclinical psychiatric abnormalities in an asymptomatic gene carrier preceding the onset of the disease.

Predictors of Working Capacity Changes Related to Huntington’s Disease: A Longitudinal Study

2021 ◽  
pp. 1-8
Author(s):  
Kasper F. van der Zwaan ◽  
Milou Jacobs ◽  
Erik W. van Zwet ◽  
Raymund A.C. Roos ◽  
Susanne T. de Bot
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurodegenerative Disorder ◽  
Psychiatric Symptoms ◽  
Motor Dysfunction ◽  
Healthy Controls ◽  
Working Capacity ◽  
Predictive Values ◽  
Gene Carriers ◽  
The Impact

Background: Huntington’s disease (HD) is an inherited neurodegenerative disorder that is characterized by motor, cognitive, and psychiatric symptoms. Although 65%of HD expanded gene carriers report changes in employment as the first functional loss, little is known about the predictors leading to changes of working capacity. Given the impact on quality of life, understanding of these factors is of great clinical value. Objective: This study evaluates disease specific characteristics and their predictive value in loss of working capacity in HD. Methods: Longitudinal data was collected through the worldwide observational study (Enroll-HD), with 15,301 participants in total and 2,791 HD and healthy control participants meeting the inclusion criteria. Changes in working capacity were analyzed by means of a survival analysis. Predictive values of demographic factors and clinical characteristics were assessed for premanifest and manifest HD through Cox regressions. Results: HD expanded gene carriers, manifest and premanifest combined, had a 31%chance of experiencing changes in employment after three years, compared to 4%in healthy controls. Apathy was found to be the most crucial determinant of working capacity changes in premanifest HD, while executive and motor dysfunction play an important role in manifest HD. Conclusion: HD expanded gene carriers are more likely to lose working capacity compared to healthy controls. Disease progression, altered motor function, cognitive decline, and in an early stage of the disease apathetic symptoms are indicative of negative changes in working capacity. Clinicians should recognize that early disease related changes, especially apathy, can affect working capacity.

scholarly journals Impact of Caryolanemagnolol on Gait and Functional Mobility on Individuals with Huntington’s Disease

2015 ◽  
Vol 14 (9) ◽  
pp. 1713-1717
Author(s):  
Ran Du
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Negative Impact ◽  
Dynamic Balance ◽  
Functional Mobility ◽  
Stand Test ◽  
Mobility Test ◽  
Sit To Stand ◽  
The Impact

Purpose: To highlight the impact of caryolanemagnolol on gait measures in forward walking, balance and mobility measures, as well as in hand and forearm function measures.Methods: Twenty patients with Huntington’s disease (HD) on stable doses of caryolanemagnolol were evaluated after medication and again following resumption of medication. The improvement in spatiotemporal gait parameters was measured by GAITRite, balance and/or mobility using Tinetti mobility test (TMT), five times sit-to-stand test (5TSST), and six-condition Romberg tests, as well as the function of the hand and forearm by finger tapping and Luria tests on Huntington's disease rating scale (UHDRS) motor scale.Results: The results demonstrated significant improvement in the unified UHDRS motor scores, Tinetti mobility test (TMT) total and balance subscale scores, and the five times sit-to-stand test when oncaryolanemagnolol compared to off-caryolanemagnolol. Spatiotemporal gait measures, the six condition Romberg test, and UHDRS hand and forearm function items remained unaffected on caryolanemagnolol treatment. Improved TMT and 5TSST performance when on drug indicates that caryolanemagnolol use may improve balance and functional mobility in individuals with HD.Conclusions: Caryolanemagnolol improves chorea and functional mobility by improving dynamic balance in individuals without any negative impact on motor function in walking and transfers.Keywords: Huntington's disease, Caryolanemagnolol, Gait measures, Choreic movements, Mobility tests

Predictors of Caregiver Burden in Huntington’s Disease

2021 ◽  
Author(s):  
Danielle C Hergert ◽  
Cynthia R Cimino
Keyword(s):  
Huntington's Disease ◽  
Cognitive Functioning ◽  
Huntington’S Disease ◽  
Caregiver Burden ◽  
Rating Scale ◽  
Age Of Onset ◽  
Executive Dysfunction ◽  
South Florida ◽  
Self Report ◽  
Younger Age

Abstract Objective Huntington’s disease (HD) is a genetic neurodegenerative condition that is characterized by cognitive, motor, and psychiatric dysfunction. The purpose of this study was to explore which disease characteristics influence caregiver burden in HD. Methods Fifty participants with HD and 50 of their caregivers participated in the study at the University of South Florida. Participants were administered a neuropsychological battery, the Unified Huntington’s Disease Rating Scale (UHDRS) motor exam, and the Frontal Systems Behavior Scale (FrSBe) self-report. Caregivers completed the Caregiving Appraisal Scale and the FrSBe family-report. Results There were significant correlations between caregiver burden and caregiver age and sex, UHDRS motor scores, cognitive functioning, and self and caregiver-reported FrSBe scores. The significant variables were entered into a regression model and explained 63.1% of the variance in caregiver burden scores. Caregiver age, cognitive functioning, and caregiver-reported FrSBe scores continued to be significant predictors of caregiver burden, whereas the other variables were no longer significant. Conclusions There were significant relationships between caregiver burden, cognitive functioning, and frontally mediated behaviors, but not motor scores. The results suggest that possible interventions for caregivers may include education to caregivers on how to cope with apathy/executive dysfunction and cognitive decline. Caregiver age was associated with burden, with younger age being associated with increased burden when controlling for symptom severity. This has implications for this population in that HD typically has a younger age of onset than other neurodegenerative diseases and therefore, these caregivers may be particularly at risk for caregiver burden.

Validation of self-report depression rating scales in Huntington's disease

2009 ◽  
Vol 25 (1) ◽  
pp. 91-96 ◽  
Author(s):  
Jennifer De Souza ◽  
Lisa A. Jones ◽  
Hugh Rickards
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scales ◽  
Self Report

The Neuropsychiatry of Huntington Disease-Like 2: A Comparison with Huntington’s Disease

2020 ◽  
Vol 9 (4) ◽  
pp. 325-334
Author(s):  
Aline Ferreira-Correia ◽  
Amanda Krause ◽  
David G. Anderson
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Huntington Disease ◽  
Rating Scale ◽  
Psychiatric Symptoms ◽  
African Ancestry ◽  
Autosomal Dominant Disorder ◽  
Cross Sectional ◽  
Triplet Repeat Expansion ◽  
Cross Sectional Design

Background: Huntington Disease-Like 2 (HDL2) is a rare autosomal dominant disorder caused by an abnormal CAG/CTG triplet repeat expansion on chromosome 16q24. The symptoms of progressive decline in motor, cognitive and psychiatric functioning are similar to those of Huntington’s disease (HD). The psychiatric features of the HDL2 have been poorly characterized. Objective: To describe the neuropsychiatric features of HDL2 and compare them with those of HD. Methods: A blinded cross-sectional design was used to compare the behavioural component of the Unified Huntington’s Disease Rating Scale (UHDRS) in participants with HDL2 (n = 15) and HD (n = 13) with African ancestry. Results: HDL2 patients presented with psychiatric symptoms involving mood disturbances and behavioural changes that were not significantly different from those in the HD group. Duration of disease and motor performance correlated (p < 0.001) with the Functional Capacity score and the Independence score of the UHDRS. HD patients reported movement dysfunction as the first symptom more frequently than HDL2 Patients (p < 0.001). Conclusion: The psychiatric phenotype of HDL2 is similar to that of HD and linked to motor decline and disease duration. Psychiatric symptoms seem more severe for HDL2 patients in the early stages of the disease.

Diagnostic Accuracy of the Conners’ Adult ADHD Rating Scale in a Postsecondary Population

2016 ◽  
Vol 23 (14) ◽  
pp. 1829-1837 ◽  
Author(s):  
Allyson G. Harrison ◽  
Sylvia Nay ◽  
Irene T. Armstrong
Keyword(s):  
Adult Adhd ◽  
Discriminant Validity ◽  
Rating Scales ◽  
Rating Scale ◽  
False Negative ◽  
False Negative Rate ◽  
Self Report ◽  
Postsecondary Students ◽  
Main Method ◽  
Screening Measure

Objective: Clinicians frequently rely upon the results of self-report rating scales when making the diagnosis of ADHD; however, little research exists regarding the ability of self-report measures to accurately differentiate ADHD from other disorders. Method: This present study investigated the ability of the Conners’ Adult ADHD Rating Scale (CAARS) to discriminate between 249 postsecondary students with carefully diagnosed ADHD and 507 clinical controls. Results: The overall discriminant validity of the CAARS was 69%, and it had an unacceptably high false positive and false negative rate. At lower prevalence rates, a high score on the CAARS has only a 22% chance of accurately identifying individuals with ADHD. Conclusion: Although the CAARS is an adequate screening measure, it should not be the main method by which a diagnosis is made, as it frequently misidentifies individuals with other psychological complaints as having ADHD. Implications for clinical practice are discussed.

Crisis Occurrence and Resolution in Patients with Severe and Persistent Mental Illness

Crisis ◽  
2005 ◽  
Vol 26 (4) ◽  
pp. 160-169 ◽  
Author(s):  
Paul S. Links ◽  
Rahel Eynan ◽  
Jeffrey S. Ball ◽  
Aiala Barr ◽  
Sean Rourke
Keyword(s):  
Mental Illness ◽  
Suicide Ideation ◽  
Rating Scale ◽  
Psychiatric Symptoms ◽  
Self Report ◽  
Community Treatment ◽  
Persistent Mental Illness ◽  
Crisis Events ◽  
Symptom Distress Scale ◽  
Distress Scale

Abstract. Assertive community treatment appears to have limited impact on the risk of suicide in persons with severe and persistent mental illness (SPMI). This exploratory prospective study attempts to understand this observation by studying the contribution of suicidality to the occurrence of crisis events in patients with SPMI. Specifically, an observer-rated measure of the need for hospitalization, the Crisis Triage Rating Scale, was completed at baseline, crisis occurrence, and resolution to determine how much the level of suicidality contributed to the deemed level of crisis. Second, observer-ratings of suicidal ideation, the Modified Scale for Suicide Ideation, and psychopathology and suicidality, Brief Psychiatric Rating Scale, were measured at baseline, crisis occurrence, and resolution. A self-report measure of distress, the Symptom Distress Scale, was completed at baseline, crisis occurrence, and resolution. Finally, the patients' crisis experiences were recorded qualitatively to compare with quantitative measures of suicidality. Almost 40% of the subjects experienced crisis events and more than a quarter of these events were judged to be severe enough to warrant the need for hospitalization. Our findings suggest that elevation of psychiatric symptoms is a major contributor to the crisis occurrences of individuals with SPMI; although the risk of suicide may have to be conceived as somewhat separate from crisis occurrence.

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