scholarly journals Apathy Associated With Impaired Recognition of Happy Facial Expressions in Huntington’s Disease

2019 ◽  
Vol 25 (05) ◽  
pp. 453-461 ◽  
Author(s):  
Katherine Osborne-Crowley ◽  
Sophie C. Andrews ◽  
Izelle Labuschagne ◽  
Akshay Nair ◽  
Rachael Scahill ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Emotion Recognition ◽  
Huntington’S Disease ◽  
Facial Expressions ◽  
Rating Scale ◽  
Recognition Task ◽  
Control Group ◽  
Short Version ◽  
Disease Rating ◽  
Patient Groups

AbstractObjectives: Previous research has demonstrated an association between emotion recognition and apathy in several neurological conditions involving fronto-striatal pathology, including Parkinson’s disease and brain injury. In line with these findings, we aimed to determine whether apathetic participants with early Huntington’s disease (HD) were more impaired on an emotion recognition task compared to non-apathetic participants and healthy controls. Methods: We included 43 participants from the TRACK-HD study who reported apathy on the Problem Behaviours Assessment – short version (PBA-S), 67 participants who reported no apathy, and 107 controls matched for age, sex, and level of education. During their baseline TRACK-HD visit, participants completed a battery of cognitive and psychological tests including an emotion recognition task, the Hospital Depression and Anxiety Scale (HADS) and were assessed on the PBA-S. Results: Compared to the non-apathetic group and the control group, the apathetic group were impaired on the recognition of happy facial expressions, after controlling for depression symptomology on the HADS and general disease progression (Unified Huntington’s Disease Rating Scale total motor score). This was despite no difference between the apathetic and non-apathetic group on overall cognitive functioning assessed by a cognitive composite score. Conclusions: Impairment of the recognition of happy expressions may be part of the clinical picture of apathy in HD. While shared reliance on frontostriatal pathways may broadly explain associations between emotion recognition and apathy found across several patient groups, further work is needed to determine what relationships exist between recognition of specific emotions, distinct subtypes of apathy and underlying neuropathology. (JINS, 2019, 25, 453–461)

Disgust and Happiness Recognition Correlate with Anteroventral Insula and Amygdala Volume Respectively in Preclinical Huntington's Disease

2007 ◽  
Vol 19 (7) ◽  
pp. 1206-1217 ◽  
Author(s):  
C. M. Kipps ◽  
A. J. Duggins ◽  
E. A. McCusker ◽  
A. J. Calder
Keyword(s):  
Basal Ganglia ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Facial Expressions ◽  
Rating Scale ◽  
Critical Role ◽  
Recognition Task ◽  
Anterior Insula ◽  
Neural Basis ◽  
Amygdala Volume

Patients with Huntington's disease (HD) can show disproportionate impairments in recognizing facial signals of disgust, but the neural basis of this deficit remains unclear. Functional imaging studies have implicated the anterior insula in the ability to recognize disgust, but have identified other structures as well, including the basal ganglia. In view of variable insula and basal ganglia volume changes in HD, we used voxel-based morphometry to map regional variations in gray matter (GM) volume in participants carrying the mutation for HD, and correlated this with their performance on a test of facial emotion recognition for six basic emotions (disgust, fear, anger, happiness, sadness, surprise). The volume of the anteroventral insula was strongly correlated with performance on the disgust recognition task. The amygdala volume (bilaterally) correlated with the ability to recognize happy facial expressions. There was marked specificity of the regional correlations for the emotion involved. Recognition of other emotion expressions, or more general cognitive or motor performance as measured by a standardized rating scale, did not correlate with regional brain volume in this group. Control participants showed no effect for any measure. The strong linear correlations for disgust and happiness recognition imply direct involvement of the anterior insula in disgust appreciation, and a similar role for the amygdala in recognizing happy facial expressions. The absence of a significant correlation with the basal ganglia suggests a less critical role for these structures in disgust recognition than has previously been suggested. The findings also highlight the role of neurodegenerative diseases combined with statistical imaging techniques in elucidating the brain basis of behavior and cognition.

scholarly journals Internal consistency of a Brazilian version of the unified Huntington's disease rating scale

2004 ◽  
Vol 62 (4) ◽  
pp. 977-982 ◽  
Author(s):  
Vitor Tumas ◽  
Sarah Teixeira Camargos ◽  
Paulo Samandar Jalali ◽  
Adriano de Paula Galesso ◽  
Wilson Marques Jr
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Internal Consistency ◽  
Negative Correlation ◽  
Clinical Characteristics ◽  
Rating Scale ◽  
Functional Evaluation ◽  
Brazilian Version ◽  
Disease Rating ◽  
High Degree

We evaluated the reliability of a translated Brazilian version of the Unified Huntington's Disease Rating Scale (UHDRS) to establish the reproducibility of the scale in a population that differs substantially from that on which the scale was originally validated. After a training period with the video and guidelines requested from the Huntington Study Group, we applied the UHDRS, except for the cognitive tests, to a group of 21 Brazilian patients with a molecular diagnosis of Huntington's disease (HD). We found a high degree of internal consistency of the motor section of the UHDRS (Cronbach's alpha= 0.841). There was a negative correlation between the total motor score and the functional assessment, the independence scale and the functional capacity. There was a positive correlation between these 3 scales of functional evaluation and a negative correlation between the age of onset of the disease and the number of CAG repeats. The behavioral scale and disease duration were not correlated with any factor. The clinical characteristics of this sample of patients as described by the UHDRS were roughly similar to those reported in the original validation studies and the correlations described were similar to those reported previously. We conclude that the Brazilian version of the UHDRS is reliable and valid to study patients with HD in the Brazilian setting, that this sample of Brazilian patients had clinical characteristics similar to those observed in other world regions, as expected, and that the clinical training method used for the application of the UHDRS was effective to insure a high degree of clinical reproducibility.

scholarly journals The unified huntington's disease rating scale for advanced patients: Validation and follow-up study

2013 ◽  
Vol 28 (14) ◽  
pp. 1995-2001 ◽  
Author(s):  
Katia Youssov ◽  
Guillaume Dolbeau ◽  
Patrick Maison ◽  
Marie-Françoise Boissé ◽  
Laurent Cleret de Langavant ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Follow Up Study ◽  
Disease Rating

scholarly journals Dietary Intake, Mediterranean Diet Adherence and Caloric Intake in Huntington’s Disease: A Review

Nutrients ◽  
2020 ◽  
Vol 12 (10) ◽  
pp. 2946
Author(s):  
Christiana C. Christodoulou ◽  
Christiana A. Demetriou ◽  
Eleni Zamba-Papanicolaou
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Mediterranean Diet ◽  
Rating Scale ◽  
Age Of Onset ◽  
Caloric Intake ◽  
Diet Adherence ◽  
Mediterranean Diet Adherence ◽  
Disease Rating ◽  
The Mediterranean

Decades of research and experimental studies have investigated Huntington’s disease (HD), a rare neurodegenerative disease. Similarly, several studies have investigated whether high/moderate adherence to the Mediterranean Diet and specific macro and micronutrients can decrease cognitive loss and provide a neuroprotective function to neurons. This review systematically identifies and examines studies that have investigated Mediterranean Diet adherence, micro- and macronutrients, supplementation and caloric intake in people with HD, in order to identify if dietary exposures resulted in improvement of disease symptoms, a delay in age of onset or if they contributed to an earlier age of onset in people with HD. A systematic search of PubMed, Directory of open access journal and HubMed was performed independently by two reviewers using specific search terms criteria for studies. The identified abstracts were screened and the studies were included in the review if they satisfied predetermined inclusion criteria. Reference screening of included studies was also performed. A total of 18 studies were included in the review. A few studies found that patients who had high/moderate adherence to Mediterranean Diet showed a slight improvement in their Unified Huntington’s Disease Rating Scale and Total Functional Capacity. In addition, people with HD who had high Mediterranean Diet adherence showed an improvement in both cognitive and motor scores and had a better quality of life compared to patients who had low Mediterranean Diet adherence. Furthermore, a few studies showed that supplementation with specific nutrients, such as triheaptanoin, L-acetyl-carnitine and creatine, had no beneficial effect on the patients’ Unified Huntington’s Disease Rating Scale score. A few studies suggest that the Mediterranean Diet may confer a motor and cognitive benefit to people with HD. Unfortunately, there was little consistency among study findings. It is important for more research to be conducted to have a better understanding of which dietary exposures are beneficial and may result delaying age of onset or disease progression in people with HD.

Feasibility and Efficacy of Brief Computerized Training to Improve Emotion Recognition in Premanifest and Early-Symptomatic Huntington’s Disease

2017 ◽  
Vol 23 (4) ◽  
pp. 314-321 ◽  
Author(s):  
Clare L. Kempnich ◽  
Dana Wong ◽  
Nellie Georgiou-Karistianis ◽  
Julie C. Stout
Keyword(s):  
Huntington's Disease ◽  
Sample Size ◽  
Emotion Recognition ◽  
Huntington’S Disease ◽  
Training Group ◽  
Control Group ◽  
Significant Group ◽  
Training Time ◽  
Training Tool ◽  
Computerized Training

AbstractObjectives: Deficits in the recognition of negative emotions emerge before clinical diagnosis in Huntington’s disease (HD). To address emotion recognition deficits, which have been shown in schizophrenia to be improved by computerized training, we conducted a study of the feasibility and efficacy of computerized training of emotion recognition in HD. Methods: We randomly assigned 22 individuals with premanifest or early symptomatic HD to the training or control group. The training group used a self-guided online training program, MicroExpression Training Tool (METT), twice weekly for 4 weeks. All participants completed measures of emotion recognition at baseline and post-training time-points. Participants in the training group also completed training adherence measures. Results: Participants in the training group completed seven of the eight sessions on average. Results showed a significant group by time interaction, indicating that METT training was associated with improved accuracy in emotion recognition. Conclusions: Although sample size was small, our study demonstrates that emotion recognition remediation using the METT is feasible in terms of training adherence. The evidence also suggests METT may be effective in premanifest or early-symptomatic HD, opening up a potential new avenue for intervention. Further study with a larger sample size is needed to replicate these findings, and to characterize the durability and generalizability of these improvements, and their impact on functional outcomes in HD. (JINS, 2017, 23, 314–321)

scholarly journals Unified Huntington's disease rating scale for advanced patients: Validation and follow-up study

2013 ◽  
Vol 28 (12) ◽  
pp. 1717-1723 ◽  
Author(s):  
Katia Youssov ◽  
Guillaume Dolbeau ◽  
Patrick Maison ◽  
Marie-Françoise Boissé ◽  
Laurent Cleret de Langavant ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Follow Up Study ◽  
Disease Rating

scholarly journals Avaliação funcional e motora da forma juvenil da doença de Huntington: relato de caso

2020 ◽  
Vol 27 (1) ◽  
pp. 78
Author(s):  
Guilherme Wilson Souza Silveira ◽  
Dionatan Costa Rodrigues ◽  
Clarissana Araújo Botaro ◽  
Luciana De Andrade Agostinho
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Disease Rating

Introdução: A doença de Huntington (DH) é uma desordem neurodegenerativa autossômica dominante e rara, com comprometimento motor, cognitivo e comportamental. O início dos sintomas precoces, com menos de 20 anos, caracteriza a forma infanto-juvenil da DH. O acompanhamento da evolução da doença é importante e permite determinar as limitações funcionais e motoras de acordo com a progressão de cada caso. Objetivo: Relatar um caso juvenil da doença de Huntington associando o genótipo e o fenótipo. Métodos: A escala Unified Huntington’s Disease Rating Scale (UHDRS), validada em português, foi aplicada por um profissional capacitado, utilizando as seções: Avaliação do Estado Funcional (FAS), Escala de Capacidade Funcional Total (TFC) e Escala de Independência (IS). Resultados: O indivíduo investigado é do sexo masculino com início dos sintomas motores aos 18 anos. Em 2019, o jovem tinha 12 anos de tempo dedoença e foi diagnosticado com DH, com alelos em heterozigose com 19 e 53 repetições CAG. As avaliações realizadas com a escala UHDRS associadas à avaliação funcional e motora, obtiveram os seguintes escores: UHDRS FAS = 18 pontos, UHDRS TFC =12, UHDRS IS = 90%e UHDRS TMS = 57 pontos. Conclusão: Pela variabilidade clínica associada à forma juvenil, o paciente deve ter tratamento personalizado, condizente com o comprometimento motor, funcional e comportamental apresentado. A UHDRS é escala mais indicada para avaliar indivíduos afetados pela DH, principalmente pelo alto grau de consistência e confiabilidade clínica.

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