Anosognosia Is Associated With Greater Caregiver Burden and Poorer Executive Function in Huntington Disease

Wibawa, Pierre; Zombor, R.; Dragović, Milan; Hayhow, Brad; Lee, J.; Panegyres, Peter K.; Rock, Danny; Starkstein, Sergio E.

doi:10.1177/0891988719856697

Cited by 18 publications

(23 citation statements)

References 33 publications

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“…Remarkably, after controlling for covariates, the effects remained. One might thereafter hypothesize-as a potential explanation-that being a family control for an affected HD patient goes along with cognitive impairments, possibly due to a more loaded or stressful situation, seen in other investigations with smaller cohorts [10,13,15] As a further explanation, stressful situations for caregivers along with burdens and depressive symptoms, might have led to cognitive disorders, which has been described earlier as pseudodementia [58]. However, we cannot entirely exclude loaded family situations or even disease burden in gene-negative participants coming from an HD family.…”

Section: Discussionmentioning

confidence: 97%

“…It is known that HD has major impacts on family systems by changing roles and there is urgent need to provide the best individual support, depending on the stage of disease and specific situation [ 9 ]. Wibawa et al identified that psychiatric–cognitive symptoms, such as anosognosia or being unawareness of own deficits, are associated with greater caregiver burden in a cohort of n = 38 HD patients [ 15 ]. These psychiatric symptoms have been well described and develop frequently in HD, increasing with disease progression but being present even in prodrome HD [ 6 ].…”

Section: Introductionmentioning

confidence: 99%

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Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

Achenbach

Saft

2021

Brain Sciences

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Background: In addition to the effects on patients suffering from motor-manifest Huntington’s disease (HD), this fatal disease is devasting to people who are at risk, premanifest mutation-carriers, and especially to whole families. There is a huge burden on people in the environment of affected HD patients, and a need for further research to identify at-risk caregivers. The aim of our research was to investigate a large cohort of family members, in comparison with genotype negative and premanifest HD in order to evaluate particular cohorts more closely. Methods: We used the ENROLL-HD global registry study to compare motoric, cognitive, functional, and psychiatric manifestation in family members, premanifest HD, and genotype negative participant as controls. Cross-sectional data were analyzed using ANCOVA-analyses in IBM SPSS Statistics V.28. Results: Of N = 21,116 participants from the global registry study, n = 5174 participants had a premanifest motor-phenotype, n = 2358 were identified as family controls, and n = 2640 with a negative HD genotype. Analysis of variance revealed more motoric, cognitive, and psychiatric impairments in premanifest HD (all p < 0.001). Self-reported psychiatric assessments revealed a significantly higher score for depression in family controls (p < 0.001) when compared to genotype negative (p < 0.001) and premanifest HD patients (p < 0.05). Family controls had significantly less cognitive capacities within the cognitive test battery when compared to genotype negative participants. Conclusions: Within the largest cohort of HD patients and families, several impairments of motoric, functional, cognitive, and psychiatric components can be confirmed in a large cohort of premanifest HD, potentially due to prodromal HD pathology. HD family controls suffered from higher self-reported depression and less cognitive capacities, which were potentially due to loaded or stressful situations. This research aims to sensitize investigators to be aware of caregiver burdens caused by HD and encourage support with socio-medical care and targeted psychological interventions. In particular, further surveys and variables are necessary in order to implement them within the database so as to identify at-risk caregivers.

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Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

Achenbach

Saft

2021

Brain Sciences

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“…Our findings also revealed no differences in empathy between people with premanifest HD and healthy controls, which aligns with some, but not all prior studies. It is noteworthy that these conflicting findings may be due to the self-report nature of instruments used to date, which may be due to impairments in insight and cognition such as memory deficits which are notable features of HD (Wibawa et al, 2020). While the aforementioned findings indicate that group-level differences exist between people with premanifest HD and healthy controls, at least for this cohort, these findings do not describe the presence or severity of individual deficits in social cognition, which is of relevance from a clinical perspective, particularly with respect to personalised medicine.…”

Section: Discussionmentioning

confidence: 99%

Profiling Social Cognition in Premanifest Huntington's Disease

Turner

Bartlett

Grainger

et al. 2021

J Int Neuropsychol Soc

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Objective: Discrepancies exist in reports of social cognition deficits in individuals with premanifest Huntington’s disease (HD); however, the reason for this variability has not been investigated. The aims of this study were to (1) evaluate group- and individual-level social cognitive performance and (2) examine intra-individual variability (dispersion) across social cognitive domains in individuals with premanifest HD. Method: Theory of mind (ToM), social perception, empathy, and social connectedness were evaluated in 35 individuals with premanifest HD and 29 healthy controls. Cut-off values beneath the median and 1.5 × the interquartile range below the 25th percentile (P25 – 1.5 × IQR) of healthy controls for each variable were established for a profiling method. Dispersion between social cognitive domains was also calculated. Results: Compared to healthy controls, individuals with premanifest HD performed worse on all social cognitive domains except empathy. Application of the profiling method revealed a large proportion of people with premanifest HD fell below healthy control median values across ToM (>80%), social perception (>57%), empathy (>54%), and social behaviour (>40%), with a percentage of these individuals displaying more pronounced impairments in empathy (20%) and ToM (22%). Social cognition dispersion did not differ between groups. No significant correlations were found between social cognitive domains and mood, sleep, and neurocognitive outcomes. Conclusions: Significant group-level social cognition deficits were observed in the premanifest HD cohort. However, our profiling method showed that only a small percentage of these individuals experienced marked difficulties in social cognition, indicating the importance of individual-level assessments, particularly regarding future personalised treatments.

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“…These findings contrast with previous studies of the AS specifically, as well as with conclusions based on a review of the anosognosia construct in HD more broadly. First, previous research observed a higher CAG burden in a high AS group of individuals with HD (Wibawa et al, 2020). Our findings ran contrary; however, our sample was twice as large and left the AS Discrepancy score as a continuous variable in correlational analyses due to the lack of an empirically supported cutoff for the AS.…”

Section: Discussionmentioning

confidence: 94%

Anosognosia and Memory Encoding in Huntington Disease

Considine

Hughes

Gibson

et al. 2022

Cognitive and Behavioral Neurology

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Background: Anosognosia can manifest as an unawareness of neurobehavioral symptoms in individuals with Huntington disease (HD). Measurement of anosognosia is challenging, but the Anosognosia Scale (AS) represents a brief option with promising findings in small samples.Objective: To replicate application of the AS in a larger HD sample than previous studies in order to assess psychometrics and demographic correlates and to investigate the genetic, motor, and neuropsychological correlates of the AS in individuals with HD.Method: We retrospectively reviewed the AS ratings of 74 genetically confirmed Huntington gene carriers, nearly all early motor manifest, who had been referred for clinical neuropsychological assessment. Concurrent clinical neurologic examination and neuropsychometric assessment data were compiled, where available (ns = 35-74). The severity of the anosognosia per AS ratings was characterized for the HD sample.Results: The AS ratings did not correlate with demographic variables, genetic markers, or motor dysfunction severity. Correlation analyses revealed that higher AS ratings correlated with worse recognition-discrimination memory performance (r = 0.38, P < 0.05) but not cognitive control on executive functioning performance or on collateral-reported frontal-behavioral symptoms. Higher AS ratings also correlated with fewer patientreported depressive symptoms (r = -0.38, P < 0.01) and diurnal hypersomnia symptoms (r = -0.44, P < 0.01). Conclusion:Anosognosia (per AS) is associated with recognition-discrimination deficits and fewer self-reported neuropsychiatric symptoms in individuals with pre-to-early manifest HD, though not with HD severity per genetic or motor markers, nor to executive dysfunction or collateral-reported frontalbehavioral symptoms.

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Anosognosia Is Associated With Greater Caregiver Burden and Poorer Executive Function in Huntington Disease

Cited by 18 publications

References 33 publications

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

Another Perspective on Huntington’s Disease: Disease Burden in Family Members and Pre-Manifest HD When Compared to Genotype-Negative Participants from ENROLL-HD

Profiling Social Cognition in Premanifest Huntington's Disease

Anosognosia and Memory Encoding in Huntington Disease

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