Exploring the Validity of the Short Version of the Problem Behaviours Assessment (PBA-s) for Huntington’s disease: A Rasch Analysis

McNally, George; Rickards, Hugh; Horton, Mike; Craufurd, David

doi:10.3233/jhd-150164

Cited by 35 publications

(23 citation statements)

References 34 publications

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“…improved PBA-s validity. 26 In our study, scores of 1 (slightly present, questionable) and 2 (mild or present but not a problem) were combined to reflect mild symptoms and scores of 3 (moderate, symptom causing problem) and 4 (severe or almost intolerable) were considered severe symptoms. By taking the percentage of individuals who had a severity score of 0 for each behavioral symptom and subtracting it from 1, the frequency at which all symptoms were experienced, irrespective of severity, was determined.…”

Section: Discussionmentioning

confidence: 97%

“…The PBA-s offers a reliable assessment of behavioral symptoms with an estimated interrater reliability of 0.82 (95% CI = 0.65-1.00) for severity scores and 0.73 (95% CI = 0.47-1.00) for frequency scores. [24][25][26] A wide range of behavioral symptoms were reported by most subjects by the time of ENROLL-HD matriculation with apathy, anxiety, irritability and depression reported most frequently. Previous studies have also found apathy to be the most common behavioral symptom over the course of HD, with depression and irritability also common.…”

Section: Discussionmentioning

confidence: 99%

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Age of onset and behavioral manifestations in Huntington's disease: An Enroll‐HD cohort analysis

et al. 2020

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Huntington's disease is associated with motor, cognitive and behavioral dysfunction. Behavioral symptoms may present before, after, or simultaneously with clinical disease manifestation. The relationship between age of onset and behavioral symptom presentation and severity was explored using the Enroll-HD database. Manifest individuals (n = 4469) were initially divided into three groups for preliminary analysis:

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Section: Discussionmentioning

confidence: 97%

Section: Discussionmentioning

confidence: 99%

Age of onset and behavioral manifestations in Huntington's disease: An Enroll‐HD cohort analysis

et al. 2020

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“…In this case, higher scores in TMT B-A and Stroop interference indicate worse performance, while for verbal fluency and SDMT poorer performance corresponds to lower scores. Psychiatric measures included the Depression and Anxiety subscales of the short version of the Problem Behavioural Assessment (PBA-s (McNally et al, 2015)), multiplying frequency by severity, with higher scores indicating more severe symptoms, as well as the Spanish version of the sensitivity to punishment and sensitivity to reward questionnaire (SPSRQ (Torrubia et al, 2001)), with higher scores indicating more sensitivity, and the short Lille apathy scale (Sockeel et al, 2006), with higher scores indicating more severity. Due to time constrains, some of the behavioural variables were not acquired in some participants.…”

Section: Methodsmentioning

confidence: 99%

Specific patterns of brain alterations underlie distinct clinical profiles in Huntington's disease

García-Gorro

Llera

Martínez‐Horta

et al. 2019

NeuroImage: Clinical

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Huntington's disease (HD) is a genetic neurodegenerative disease which involves a triad of motor, cognitive and psychiatric disturbances. However, there is great variability in the prominence of each type of symptom across individuals. The neurobiological basis of such variability remains poorly understood but would be crucial for better tailored treatments. Multivariate multimodal neuroimaging approaches have been successful in disentangling these profiles in other disorders. Thus we applied for the first time such approach to HD. We studied the relationship between HD symptom domains and multimodal measures sensitive to grey and white matter structural alterations. Forty-three HD gene carriers (23 manifest and 20 premanifest individuals) were scanned and underwent behavioural assessments evaluating motor, cognitive and psychiatric domains. We conducted a multimodal analysis integrating different structural neuroimaging modalities measuring grey matter volume, cortical thickness and white matter diffusion indices – fractional anisotropy and radial diffusivity. All neuroimaging measures were entered into a linked independent component analysis in order to obtain multimodal components reflecting common inter-subject variation across imaging modalities. The relationship between multimodal neuroimaging independent components and behavioural measures was analysed using multiple linear regression. We found that cognitive and motor symptoms shared a common neurobiological basis, whereas the psychiatric domain presented a differentiated neural signature. Behavioural measures of different symptom domains correlated with different neuroimaging components, both the brain regions involved and the neuroimaging modalities most prominently associated with each type of symptom showing differences. More severe cognitive and motor signs together were associated with a multimodal component consisting in a pattern of reduced grey matter, cortical thickness and white matter integrity in cognitive and motor related networks. In contrast, depressive symptoms were associated with a component mainly characterised by reduced cortical thickness pattern in limbic and paralimbic regions. In conclusion, using a multivariate multimodal approach we were able to disentangle the neurobiological substrates of two distinct symptom profiles in HD: one characterised by cognitive and motor features dissociated from a psychiatric profile. These results open a new view on a disease classically considered as a uniform entity and initiates a new avenue for further research considering these qualitative individual differences.

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“…In our opinion, these cases are not equal; hypothesis is that it is more likely that severity – rather than frequency – of apathy is related to the neurodegenerative process in HD. McNally et al ( McNally et al, 2015 ) have also pointed out in their re-evaluation of the PBA-s that using the product score might statistically not be appropriate as it is not a ratio scale. For future research, it would be of interest to further evaluate the use of the different PBA-s scores.…”

Section: Discussionmentioning

confidence: 99%

Apathy and atrophy of subcortical brain structures in Huntington's disease: A two-year follow-up study

Baake

Coppen

Duijn

et al. 2018

NeuroImage: Clinical

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BackgroundHuntington's disease (HD) is characterized by motor and behavioral symptoms, and cognitive decline. HD gene carriers and their caregivers report the behavioral and cognitive symptoms as the most burdensome. Apathy is the most common behavioral symptom of HD and is related to clinical measures of disease progression, like functional capacity. However, it is unknown whether apathy is directly related to the neurodegenerative processes in HD.ObjectiveThe aim is to investigate whether an association between atrophy of subcortical structures and apathy is present in HD, at baseline and after 2 years follow-up.MethodVolumes of 7 subcortical structures were measured using structural T1 MRI in 171 HD gene carriers of the TRACK-HD study and apathy was assessed with the Problem Behaviors Assessment-Short, at baseline and follow-up visit. At baseline, logistic regression was used to evaluate whether volumes of subcortical brain structures were associated with the presence of apathy. Linear regression was used to assess whether subcortical atrophy was associated with the degree of apathy at baseline and with an increase in severity of apathy over time.ResultsAt baseline, smaller volume of the thalamus showed a higher probability of the presence of apathy in HD gene carriers, but none of the subcortical structures was associated with the degree of apathy. Over time, no association between atrophy of any subcortical structures and change in degree of apathy was found.ConclusionThe presence of apathy is associated with atrophy of the thalamus in HD, suggesting that apathy has an underlying neural cause and might explain the high incidence of apathy in HD. However, no association was found between atrophy of these subcortical structures and increase in severity of apathy over a 2-year time period.

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Exploring the Validity of the Short Version of the Problem Behaviours Assessment (PBA-s) for Huntington’s disease: A Rasch Analysis

Abstract: 240 words Manuscript: 5017 wordsManuscript Reference Number: 15-164 (1 st revised submission) All authors have contributed the work, agree with the presented findings, the work has not been published before nor is being considered for publication in another journal. Correspondence address

Cited by 35 publications

References 34 publications

Age of onset and behavioral manifestations in Huntington's disease: An Enroll‐HD cohort analysis

Age of onset and behavioral manifestations in Huntington's disease: An Enroll‐HD cohort analysis

Specific patterns of brain alterations underlie distinct clinical profiles in Huntington's disease

Apathy and atrophy of subcortical brain structures in Huntington's disease: A two-year follow-up study

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