“Forgetting to Remember” in Huntington's Disease: A Study of Laboratory, Semi-Naturalistic, and Self-Perceptions of Prospective Memory

Nicoll, Diane R.; Pirogovsky, Eva; Woods, Steven Paul; Holden, H.; Filoteo, J. Vincent; Gluhm, Shea; Corey–Bloom, Jody; Gilbert, Paul E.

doi:10.1017/s1355617713001355

Cited by 14 publications

(7 citation statements)

References 45 publications

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“…Also, as Keefe et al (2016) indicate, performance-based functional capacity measures do not involve subjective judgments about one’s own abilities nor require informants, so they might help to reduce the burden on investigators and participants in future clinical studies. Moreover, the IAFAI and EcoKitchen results of the HP group seem to be in line with the findings of Nicoll et al (2014) in a study about prospective memory in HD, where a discrepancy between the participants’ performance-based and self-reported function was identified. This further highlights the importance of using objective measures to assess the functional and cognitive status of premanifest HD individuals, as the self-report methods to assess function are susceptible to bias and the classic cognitive tests often lack the sensitivity to subtle executive impairments and do not inform about their relevance to everyday performance.…”

Section: Discussionsupporting

confidence: 88%

“…Few studies have used or developed performance-based tools to assess everyday functioning in HD. Nicoll et al (2014) used the “Memory for Intentions Screening Test” as a standardized performance-based measure of prospective memory in HD and Sheppard et al (2017) used the “Advanced Finances Test” as a performance-based measure of the participants’ ability to manage finances. Both studies were done in semi-naturalistic settings (real materials and props handled in a laboratory) and resorted to observational methods to infer about the mild-moderate HD patients’ performance level.…”

Section: Introductionmentioning

confidence: 99%

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A Novel Ecological Approach Reveals Early Executive Function Impairments in Huntington’s Disease

et al. 2019

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Introduction: Impairments in executive functions are common in neurogenetic disorders such as Huntington’s disease (HD) and are thought to significantly influence the patient’s functional status. Reliable tools with higher ecological validity that can assess and predict the impact of executive dysfunction in daily-life performance are needed. This study aimed to develop and validate a novel non-immersive virtual reality task (“EcoKitchen”) created with the purpose of capturing cognitive and functional changes shown by HD carriers without clinical manifestations of the disease (Premanifest HD), in a more realistic setting. Materials and Methods: We designed a virtual reality task with three blocks of increasing executive load. The performance of three groups (Controls, CTRL; Premanifest HD individuals, HP; Early Manifest HD patients, HD) was compared in four main components of the study protocol: the EcoKitchen; a subjective (self-report) measure – “The Adults and Older Adults Functional Assessment Inventory (IAFAI)”; the “Behavioural Assessment of Dysexecutive Syndrome battery (BADS)”; and a conventional neuropsychological test battery. We also examined statistical associations between EcoKitchen and the other executive, functional and clinical measures used. Results: The HD group showed deficits in all the assessment methods used. In contrast, the HP group was only found to be impaired in the EcoKitchen task, particularly in the most cognitively demanding blocks, where they showed a higher number of errors compared to the CTRL group. Statistically significant correlations were identified between the EcoKitchen, measures of the other assessment tools, and HD clinical features. Discussion: The EcoKitchen task, developed as an ecological executive function assessment tool, was found to be sensitive to early deficits in this domain. Critically, in premanifest HD individuals, it identifies dysfunction prior to symptom onset. Further it adds a potential tool for diagnosis and management of the patients’ real-life problems.

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Section: Discussionsupporting

confidence: 88%

Section: Introductionmentioning

confidence: 99%

A Novel Ecological Approach Reveals Early Executive Function Impairments in Huntington’s Disease

et al. 2019

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“…The cognitive profile of manifest HD includes deficits in executive function, emotional processing and memory ( Ho et al, 2003; Henley et al, 2012; Tabrizi et al, 2013; Holl et al, 2013; Nicoll et al, 2014; Georgiou-Karistianis et al, 2013, 2014; Johnson et al, 2007; Stout et al, 2011; Begeti et al, 2013 ). In the prodromal phase the impairment is more subtle but abnormalities in psychomotor processing speed, verbal fluency and the recognition of negative emotions are common ( Tabrizi et al, 2013, 2012; Begeti et al, 2013 ).…”

Section: Introductionmentioning

confidence: 99%

The role of the amygdala during emotional processing in Huntington's disease: From pre-manifest to late stage disease

et al. 2015

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BackgroundDeficits in emotional processing can be detected in the pre-manifest stage of Huntington's disease and negative emotion recognition has been identified as a predictor of clinical diagnosis. The underlying neuropathological correlates of such deficits are typically established using correlative structural MRI studies. This approach does not take into consideration the impact of disruption to the complex interactions between multiple brain circuits on emotional processing. Therefore, exploration of the neural substrates of emotional processing in pre-manifest HD using fMRI connectivity analysis may be a useful way of evaluating the way brain regions interrelate in the period prior to diagnosis.MethodsWe investigated the impact of predicted time to disease onset on brain activation when participants were exposed to pictures of faces with angry and neutral expressions, in 20 pre-manifest HD gene carriers and 23 healthy controls. On the basis of the results of this initial study went on to look at amygdala dependent cognitive performance in 79 Huntington's disease patients from a cross-section of disease stages (pre-manifest to late disease) and 26 healthy controls, using a validated theory of mind task: “the Reading the Mind in the Eyes Test” which has been previously been shown to be amygdala dependent.ResultsPsychophysiological interaction analysis identified reduced connectivity between the left amygdala and right fusiform facial area in pre-manifest HD gene carriers compared to controls when viewing angry compared to neutral faces. Change in PPI connectivity scores correlated with predicted time to disease onset (r=0.45, p<0.05). Furthermore, performance on the “Reading the Mind in the Eyes Test” correlated negatively with proximity to disease onset and became progressively worse with each stage of disease.ConclusionAbnormalities in the neural networks underlying social cognition and emotional processing can be detected prior to clinical diagnosis in Huntington's disease. Connectivity between the amygdala and other brain regions is impacted by the disease process in pre-manifest HD and may therefore be a useful way of identifying participants who are approaching a clinical diagnosis. Furthermore, the “Reading the Mind in the Eyes Test” is a surrogate measure of amygdala function that is clinically useful across the entire cross-section of disease stages in HD.

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“…Impairment of executive functions can be debilitating and interferes with the ability to maintain everyday functioning (Burgess et al, 2006; Jurado & Rosselli, 2007). Deficits in executive functions can also interfere with other cognitive functions such as memory retrieval and prospective memory (Nicoll et al, 2014). Executive functions have been studied intensively in HD (Dumas et al, 2013; Paulsen, 2011).…”

Section: Introductionmentioning

confidence: 99%

Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington’s Disease Gene-expansion Carriers

Larsen

Vinther-Jensen

Gade

et al. 2015

J Int Neuropsychol Soc

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Executive functions (EF) and psychomotor speed (PMS) has been widely studied in Huntington's disease (HD). Most studies have focused on finding markers of disease progression by comparing group means at different disease stages. Our aim was to investigate performances on nine measures of EF and PMS in a group of premanifest and manifest HD-gene expansion carriers and to investigate which measures were most sensitive for assessment of individual patients by analyzing frequencies of impaired performances relative to healthy controls. We recruited HD gene-expansion carriers, 48 manifest and 50 premanifest and as controls 39 healthy gene-expansion negative individuals. All participants underwent neurological examination and neuropsychological testing with nine cognitive measures. The frequency of impairment was investigated using cutoff scores. In group comparisons the manifest HD gene-expansion carriers scored significantly worse than controls on all tests and in classification of individual scores the majority of scores were classified as probably impaired (10th percentile) or impaired (5th percentile) with Symbol Digit Modalities Test (SDMT) being the most frequently impaired. Group comparisons of premanifest HD gene-expansion carriers and healthy controls showed significant differences on SDMT and Alternating fluency tests. Nevertheless the frequencies of probably impaired and impaired scores on individual tests were markedly higher for Alternating and Lexical fluency tests than for SDMT. We found distinct group differences in frequency of impairment on measures of EF and PMS in manifest and premanifest HD gene-expansion carriers. Our results indicate to what degree these measures can be expected to be clinically impaired.

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“Forgetting to Remember” in Huntington's Disease: A Study of Laboratory, Semi-Naturalistic, and Self-Perceptions of Prospective Memory

Cited by 14 publications

References 45 publications

A Novel Ecological Approach Reveals Early Executive Function Impairments in Huntington’s Disease

A Novel Ecological Approach Reveals Early Executive Function Impairments in Huntington’s Disease

The role of the amygdala during emotional processing in Huntington's disease: From pre-manifest to late stage disease

Assessing Impairment of Executive Function and Psychomotor Speed in Premanifest and Manifest Huntington’s Disease Gene-expansion Carriers

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