Rating scales for cognition in Huntington's disease: Critique and recommendations

2017 ◽  
Vol 33 (2) ◽  
pp. 187-195 ◽  
Author(s):  
Tiago A. Mestre ◽  
Anne-Catherine Bachoud-Lévi ◽  
Johan Marinus ◽  
Julie C. Stout ◽  
Jane S. Paulsen ◽  
...  
2017 ◽  
Vol 32 (3) ◽  
pp. 482-482
Author(s):  
Tiago A. Mestre ◽  
Erik van Duijn ◽  
Aileen M. Davis ◽  
Anne‐Catherine Bachoud‐Lévi ◽  
Monica Busse ◽  
...  

2018 ◽  
Vol 5 (2) ◽  
pp. 111-117 ◽  
Author(s):  
Tiago A. Mestre ◽  
Maria João Forjaz ◽  
Philipp Mahlknecht ◽  
Francisco Cardoso ◽  
Joaquim J. Ferreira ◽  
...  

2020 ◽  
Vol 60 (8) ◽  
pp. 1051-1060
Author(s):  
Wan Sun ◽  
Di Zhou ◽  
John H. Warner ◽  
Douglas R. Langbehn ◽  
Guenther Hochhaus ◽  
...  

2009 ◽  
Vol 25 (1) ◽  
pp. 91-96 ◽  
Author(s):  
Jennifer De Souza ◽  
Lisa A. Jones ◽  
Hugh Rickards

2016 ◽  
Vol 31 (10) ◽  
pp. 1466-1478 ◽  
Author(s):  
Tiago A. Mestre ◽  
Erik van Duijn ◽  
Aileen M. Davis ◽  
Anne-Catherine Bachoud-Lévi ◽  
Monica Busse ◽  
...  

2020 ◽  
Vol 9 (3) ◽  
pp. 291-302
Author(s):  
David Isaacs ◽  
Jessie S. Gibson ◽  
Jeffrey Stovall ◽  
Daniel O. Claassen

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.


2014 ◽  
Vol 26 (5) ◽  
pp. 298-306 ◽  
Author(s):  
Angelica Kloberg ◽  
Radu Constantinescu ◽  
Marie Karin Lena Nilsson ◽  
Maria Lizzie Carlsson ◽  
Arvid Carlsson ◽  
...  

ObjectiveTo evaluate the safety (primary objective) and efficacy (secondary objective) of (−)-OSU6162 in Huntington’s disease (HD).MethodsIn a double-blind, cross-over trial, patients with HD were randomly assigned to start treatment on either (−)-OSU6162 or placebo. After 4 weeks, those patients who initially received active drug were switched to placebo for another 4 weeks, and vice versa. During the first week the (−)-OSU6162 dose was 15 mg twice daily, during the second week 30 mg twice daily, and during the last 2 weeks 45 mg twice daily. Motor, cognitive, mental and social functions were rated by the clinical investigator or by self-assessment, using established rating scales.ResultsFifteen patients fulfilling inclusion and exclusion criteria completed the study. (−)-OSU6162 was well tolerated by all patients and no adverse effects were observed. (−)-OSU6162 treatment significantly improved the Short Form 36 Vitality score, mainly due to an improvement of the individual item ‘worn-out’ (VT3). In addition, an improvement of depressive symptoms was found using Beck Depression Inventory. In contrast to a general trend of improvement in several non-motor variables only small and non-significant differences between (−)-OSU6162 and placebo were found regarding motor functions.Conclusions(−)-OSU6162 offers promise for the treatment of HD, as a drug with good tolerability, capable of improving the patients’ experienced non-motor functions such as energy and mood and thus alleviating symptoms of great importance for their quality of life.


2018 ◽  
Vol 5 (4) ◽  
pp. 361-372 ◽  
Author(s):  
Tiago A. Mestre ◽  
Monica Busse ◽  
Aileen M. Davis ◽  
Lori Quinn ◽  
Filipe B. Rodrigues ◽  
...  

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