scholarly journals An item response analysis of the motor and behavioral subscales of the unified Huntington's disease rating scale in huntington disease gene expansion carriers

2011 ◽  
Vol 26 (5) ◽  
pp. 877-884 ◽  
Author(s):  
Anthony L. Vaccarino ◽  
Karen Anderson ◽  
Beth Borowsky ◽  
Kevin Duff ◽  
Joseph Giuliano ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Item Response ◽  
Huntington Disease ◽  
Rating Scale ◽  
Disease Gene ◽  
Response Analysis ◽  
Gene Expansion ◽  
Disease Rating ◽  
Huntington Disease Gene

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.

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.

scholarly journals Functional and motor response to low dose olanzapine in huntington's disease: case report

2004 ◽  
Vol 62 (4) ◽  
pp. 1092-1094 ◽  
Author(s):  
Jerson Laks ◽  
Marlos Rocha ◽  
Claudia Capitão ◽  
Romeu Côrtes Domingues ◽  
Giovanna Ladeia ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Daily Living ◽  
Motor Response ◽  
Low Dose ◽  
Rating Scale ◽  
Behavioral Impairment ◽  
Randomized Controlled ◽  
Disease Case ◽  
Disease Rating

Previous reports on the use of olanzapine in Huntington's disease (HD) used doses ranging from 10-30 mg. We report a case of HD with marked delusions and behavioral impairment assessed by the Unified Huntington's Disease Rating Scale at baseline and four months later treated with a low dose of olanzapine. The patient improved in motor, psychiatric and activity of daily living symptoms after four months of treatment. The response to a low dose of olanzapine in HD may be an indicator of efficacy in similar cases. Further randomized controlled trials can properly assess these findings.

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