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 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

Unified Huntington's disease rating scale: A follow up

1998 ◽  
Vol 13 (6) ◽  
pp. 915-919 ◽  
Author(s):  
Sabine Siesling ◽  
Jeroen P. P. van Vugt ◽  
Koos A. H. Zwinderman ◽  
Karl Kieburtz ◽  
Raymund A. C. Roos
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Rating Scale ◽  
Disease Rating

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.

Disease stage and plasma levels of cytokines in Huntington's disease: A 2-year follow-up study

2017 ◽  
Vol 32 (7) ◽  
pp. 1103-1104 ◽  
Author(s):  
J.A. Bouwens ◽  
E. van Duijn ◽  
C.M. Cobbaert ◽  
R.A.C. Roos ◽  
R.C. van der Mast ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Plasma Levels ◽  
Disease Stage ◽  
Follow Up Study

Psychiatric Disorders in Huntington's Disease: A 2-Year Follow-up Study

2012 ◽  
Vol 53 (3) ◽  
pp. 220-229 ◽  
Author(s):  
W. Reedeker ◽  
R.C. van der Mast ◽  
E.J. Giltay ◽  
T.A.D. Kooistra ◽  
R.A.C. Roos ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Psychiatric Disorders ◽  
Huntington’S Disease ◽  
Follow Up Study

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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