Cognitive change in patients with Huntington disease on the Repeatable Battery for the Assessment of Neuropsychological Status

Beglinger, Leigh J.; Duff, Kevin; Allison, J.; Theriault, Danielle; O’Rourke, Justin J.F.; Leserman, Anne; Paulsen, Jane S.

doi:10.1080/13803390903313564

Cited by 41 publications

(33 citation statements)

References 39 publications

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“…The total score for each item is obtained by multiplying frequency by severity. The UHDRS has been used in numerous studies of HD and randomized, controlled trials, either using the total score or specific item domains . A cutoff score of ≥6 for the presence of depressed mood was reported using the corresponding item of the UHDRS‐b .…”

Section: Scales Assessing Multiple Behavioral Domainsmentioning

confidence: 99%

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

et al. 2016

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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.

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Section: Scales Assessing Multiple Behavioral Domainsmentioning

confidence: 99%

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

et al. 2016

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“…Huntington disease (HD) is a phenotypically heterogeneous disease characterized by choreiform and dystonic movements [1], bradykinesia [2–3], cognitive decline [4], and psychiatric comorbidities [5]. Balance and gait impairments [6–7], recurrent falls [8–9], and functional decline and disability [10] are common manifestations of the disease.…”

Section: Introductionmentioning

confidence: 99%

Deficits in stepping response time are associated with impairments in balance and mobility in people with Huntington disease

Goldberg

Schepens

Feely

et al. 2010

Journal of the Neurological Sciences

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Huntington disease (HD) is a disorder characterized by chorea, dystonia, bradykinesia, cognitive decline and psychiatric comorbidities. Balance and gait impairments, as well as falls, are common manifestations of the disease. The importance of compensatory rapid stepping to maintain equilibrium in older adults is established, yet little is known of the role of stepping response times (SRTs) in balance control in people with HD. SRTs and commonly-used clinical measures of balance and mobility were evaluated in fourteen symptomatic participants with HD, and nine controls at a university mobility research laboratory. Relative and absolute reliability, as well as minimal detectable change in SRT were quantified in the HD participants. HD participants exhibited slower SRTs and poorer dynamic balance, mobility and motor performance than controls. HD participants also reported lower balance confidence than controls. Deficits in SRT were associated with low balance confidence and impairments on clinical measures of balance, mobility, and motor performance in HD participants. Measures of relative and absolute reliability indicate that SRT is reliable and reproducible across trials in people with HD. A moderately low percent minimal detectable change suggests that SRT appears sensitive to detecting real change in people with HD. SRT is impaired in people with HD and may be a valid and objective marker of disease progression.

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“…Test-retest reliability for the RBANS is adequate in geriatric samples (Duff et al, 2005). Although initially developed as a screening measure for dementia, the validity of the RBANS has been demonstrated in a variety of neuropsychological disorders (e.g., dementia, stroke, Parkinson’s disease, multiple sclerosis, Huntington’s disease, traumatic brain injury, depression) (Beatty, 2004; Beatty et al, 2003; Beglinger et al, 2009; Dickerson et al, 2004; Duff, Beglinger, Theriault, Allison, & Paulsen, 2009; Hobson, Hall, Humphreys-Clark, Schrimsher, & O’Bryant, 2009; Pachet, 2007; Paradiso, Duff, Vaidya, Hoth, & Mold, 2009; Wilde, 2006). …”

Section: Methodsmentioning

confidence: 99%

The RBANS Effort Index: Base Rates in Geriatric Samples

Duff

Spering

O’Bryant

et al. 2011

Applied Neuropsychology

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The Effort Index (EI) of the RBANS was developed to assist clinicians in discriminating patients who demonstrate good effort from those with poor effort. However, there are concerns that older adults might be unfairly penalized by this index, which uses uncorrected raw scores. Using five independent samples of geriatric patients with a broad range of cognitive functioning (e.g., cognitively intact, nursing home residents, probable Alzheimer's disease), base rates of failure on the EI were calculated. In cognitively intact and mildly impaired samples, few older individuals were classified as demonstrating poor effort (e.g., 3% in cognitively intact). However, in the more severely impaired geriatric patients, over one third had EI scores that fell above suggested cut-off scores (e.g., 37% in nursing home residents, 33% in probable Alzheimer's disease). In the cognitively intact sample, older and less educated patients were more likely to have scores suggestive of poor effort. Education effects were observed in 3 of the 4 clinical samples. Overall cognitive functioning was significantly correlated with EI scores, with poorer cognition being associated with greater suspicion of low effort. The current results suggest that age, education, and level of cognitive functioning should be taken into consideration when interpreting EI results and that significant caution is warranted when examining EI scores in elders suspected of having dementia. (Randolph, 1998) were quite rare in an outpatient clinical sample. Based on this observation, they created an embedded Effort Index (EI) for the RBANS using inversely weighted scores from these two subtests (Digit Span and List Recognition), with higher EI scores reflecting poorer effort. Using various cutoffs (>0, >3), the EI yielded good discrimination between actual cases of traumatic brain injury and three "malingering" groups (86 -96% classification accuracy). From these findings, the authors concluded that the RBANS EI is a sensitive marker for suspect effort though further validation was warranted. The sample used to develop the EI was predominantly comprised of individuals with traumatic brain injury, psychiatric illnesses, and other neurological diseases (e.g., cerebrovascular accident, epilepsy, multiple sclerosis). Although some individuals with dementia were included in this sample, cases with advanced disease that required 24-hour supervision were excluded. KeywordsOne concern with the EI is that it utilizes raw scores from these two subtests, which might inflate the level of "suspect" effort in older adults, who tend to get lower raw scores than younger adults. Indeed, elevated levels of "suspect" effort, based on RBANS EI scores, were observed in a small sample of medically ill geriatric patients (Hook, Marquine, & Hoelzle, 2009). In that study, 31% of the sample was identified as having questionable effort using the recommended EI cutoffs (Silverberg, Wertheimer, & Fichtenberg, 2007). As cognitive impairment increased, so did the concerns about poor effort...

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Cognitive change in patients with Huntington disease on the Repeatable Battery for the Assessment of Neuropsychological Status

Cited by 41 publications

References 39 publications

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

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

Deficits in stepping response time are associated with impairments in balance and mobility in people with Huntington disease

The RBANS Effort Index: Base Rates in Geriatric Samples

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