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 ◽  
...  
2013 ◽  
Vol 28 (12) ◽  
pp. 1717-1723 ◽  
Author(s):  
Katia Youssov ◽  
Guillaume Dolbeau ◽  
Patrick Maison ◽  
Marie-Françoise Boissé ◽  
Laurent Cleret de Langavant ◽  
...  

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

2004 ◽  
Vol 62 (4) ◽  
pp. 977-982 ◽  
Author(s):  
Vitor Tumas ◽  
Sarah Teixeira Camargos ◽  
Paulo Samandar Jalali ◽  
Adriano de Paula Galesso ◽  
Wilson Marques Jr

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.


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 ◽  
...  

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 ◽  
...  

2011 ◽  
Vol 23 (6) ◽  
pp. 315-317
Author(s):  
Witold Soltan ◽  
Emilia Sitek ◽  
Hubert Wichowicz ◽  
Dariusz Wieczorek ◽  
Jaroslaw Slawek

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.


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