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

Abstract

Behavioral symptoms are an important feature of Huntington's disease and contribute to impairment in quality of life. The Movement Disorder Society commissioned the assessment of the clinimetric properties of rating scales in Huntington's disease to make recommendations regarding their use, following previously used standardized criteria. A systematic literature search was conducted to identify the scales used to assess behavioral symptoms in Huntington's disease. For the purpose of this review, 7 behavioral domains were deemed significant in Huntington's disease: irritability, anxiety, depression, apathy, obsessive-compulsive behaviors, psychosis, and suicidal ideation. We included a total of 27 behavioral rating scales, 19 of which were of a single behavioral domain and the remaining 8 scales included multiple behavioral domains. Three rating scales were classified as "recommended" exclusively for screening purposes: the Irritability Scale for irritability, the Beck Depression Inventory-II, and the Hospital Anxiety and Depression Scale for depression. There were no "recommended" scales for other purposes such as diagnosis, severity, or change in time or to treatment. The main challenges identified for assessment of behavioral symptoms in Huntington's disease are the co-occurrence of multiple behavioral symptoms, the particular features of a behavioral symptom in Huntington's disease, and the need to address stage- and disease-specific features, including cognitive impairment and lack of insight. The committee concluded that there is a need to further validate currently available behavioral rating scales in Huntington's disease to address gaps in scale validation for specific behavioral domains and purpose of use. © 2016 International Parkinson and Movement Disorder Society.

Bupropion for the treatment of apathy in Huntington’s disease: A multicenter, randomised, double-blind, placebo-controlled, prospective crossover trial

“…In the case of the AES, the poor differentiation between the answers 2 (“slightly characteristic”) and 3 (“somewhat characteristic”) on the four point Likert scale may reduce sensitivity. It is noteworthy that the validation of the AES in HD is still incomplete [41]. Our trial adds important data for this process.…”

“…We chose the informant version of the AES, the AES-I score, as the primary outcome variable since we assumed that HD patients rate their symptoms of apathy less severe than their caregivers or physicians [40, 41]. In HD, like in other neuropsychiatric diseases with frontal pathology, lack of insight into symptoms and functional capacities is common [42, 43].…”

Rating Scales for Movement Disorders With Sleep Disturbances: A Narrative Review

“…A IPMDS task force rated the UHDRS behavioral section (UHDRS-b) as a “suggested” scale for assessing severity of and screening for behavioral symptoms in patients with HD ( 46 ). The UHDRS also has a version for advanced patients (UHDRS-FAP), with satisfactory internal consistency and inter-rater reliability ( 47 ).…”

Neuropsychiatric symptoms and premanifest Huntington's disease

“…We read with interest the article titled "Rating Scales for Behavioral Symptoms in Huntington's Disease, Critique and Recommendations "by the Members of the Movement Disorder Society Committee on Rating Scales Development 1 and agree with the authors on most of them. In addition, we would like to point out that another important issue is to agree with the diagnostic criteria of what is called premanifest Huntington's disease (HD).…”

“…Michel Saenz-Farret, MD,1 Carlos Z uñiga-Ramirez, MD 1 Carolina Candelaria Ramirez-Gomez, MD2 Veronica Montilla-Uzcategui, MD 2 Federico Micheli, MD, PhD 2 * Disorders and Neurodegenerative Diseases Unit, Hospital Civil de Guadalajara "Fray Antonio Alcalde", Guadalajara, Mexico 2 Parkinson's Disease and Movement Disorders Unit, Department of Neurology, Hospital de Cl ınicas "Jose de San Mart ın," Buenos Aires, Argentina…”