Self‐awareness of executive dysfunction in <scp>H</scp>untington's disease: Comparison with <scp>P</scp>arkinson's disease and cervical dystonia

Sitek, Emilia J.; Sołtan, Witold; Wieczorek, Dariusz; Schinwelski, Michał; Robowski, Piotr; Harciarek, Michał; Guzińska, Katarzyna; Sławek, Jarosław

doi:10.1111/pcn.12006

Cited by 21 publications

(27 citation statements)

References 7 publications

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“…Two studies using the Dysexecutive Questionnaire demon-strated that HD patients could accurately rate proxies' but not their own behaviour [9,10], providing evidence of reduced self-awareness of behavioural change that cannot be attributable to generalized deficient judgement. A third study [13], which used the same questionnaire to compare insight in HD and PD, revealed only mild reduction in awareness for executive symptoms in HD, based on differences in patient and proxy ratings. It is possible though that relatively small group numbers resulted in lack of power to elicit significant differences.…”

Section: Insight Into Behavioural and Executive Impairmentmentioning

confidence: 98%

“…An additional 'preclinical' stage refers to people with the HD mutation who do not yet manifest overt clinical signs of disease. Awareness has been studied in both preclinical [2][3][4] and early-stage (stages 1-3) HD [2,3,[5][6][7][8][9][10][11][12][13][14].…”

Section: Identification and Classification Of Studiesmentioning

confidence: 99%

“…Most studies of awareness in HD have involved comparison of patients' self-ratings with those of caregivers/companions [2,[8][9][10][11][12][13][14] (see: Table 1). Other studies have compared patients' ratings with ratings or measurements made by clinicians [3][4][5][6][7].…”

Section: Identification and Classification Of Studiesmentioning

confidence: 99%

“…Hoth et al [10] showed that HD patients' lack of awareness extended across the symptom domains of behavioural control, emotional control and activities of daily living. Sitek et al [11][12][13] studied the same patient sample across different domains and found deficient insight for motor, executive and memory functions. These findings, taken at face value, might point to anosognosia as a generalized phenomenon.…”

Section: Is Anosognosia In Hd Domain Specific Of Generalized?mentioning

confidence: 99%

“…Some studies failed to demonstrate an association between insight and disease severity (measured by Unified Huntington's Disease Rating Scale (UHDRS) motor score and Independence Scale), which may be due to small sample sizes [11,13]. In other studies the relationship between the degree of awareness and disease severity was not addressed [6,9].…”

Section: Is Anosognosia Influenced By Disease Severity?mentioning

confidence: 99%

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Unawareness of Deficits in Huntington's Disease

Sitek

Thompson

Craufurd³

et al. 2014

Journal of Huntington's Disease

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People with Huntington's disease (HD) may show reduced awareness of physical and mental changes in themselves. This article reviews the evidence for loss of awareness (anosognosia) in an attempt to elucidate its characteristics and possible underlying mechanisms. It is shown that defective awareness occurs across domains. People with HD may under-report the presence or severity of involuntary movements, under-estimate cognitive impairment and deny behavioural change. Nevertheless, awareness is not all or none. Moreover, it may be affected differentially for different symptom domains and emerge at different stages of disease, raising the possibility of distinct contributory mechanisms. Findings of an inverse relationship between insight and severity of disease suggest that cognitive impairment, in particular executive dysfunction, may be an important contributory factor. Evidence has accrued to support this argument. However, cognitive impairment cannot fully account for patients' lack of awareness of involuntary movements. Findings that patients accurately report consequences but not the experience of involuntary movements, and better acknowledge their presence when watching videotapes of themselves suggests that physiological factors play an important role. The putative role of denial as a coping mechanism is discussed. Recognition by clinicians of deficient self-awareness is crucial because of its implications for diagnosis and optimal clinical management of HD.

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Section: Insight Into Behavioural and Executive Impairmentmentioning

confidence: 98%

Section: Identification and Classification Of Studiesmentioning

confidence: 99%

Section: Identification and Classification Of Studiesmentioning

confidence: 99%

Section: Is Anosognosia In Hd Domain Specific Of Generalized?mentioning

confidence: 99%

Section: Is Anosognosia Influenced By Disease Severity?mentioning

confidence: 99%

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Unawareness of Deficits in Huntington's Disease

Sitek

Thompson

Craufurd³

et al. 2014

Journal of Huntington's Disease

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Altered awareness of cognitive and neuropsychiatric symptoms in Parkinson's disease and Dementia with Lewy Bodies: A systematic review

Pennington

Duncan

Ritchie

2020

Int J Geriat Psychiatry

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Objectives: Altered awareness of cognitive and neuropsychiatric symptoms is a common feature of neurodegeneration, which can significantly impact on quality of life, medication concordance and personal safety. Elucidating how awareness is affected by common alpha-synucleinopathies therefore has significant clinical relevance. We performed a systematic review of the literature on awareness of cognitive and neuropsychiatric symptoms in Parkinson's disease and Dementia with Lewy Bodies. Methods: Searches of PubMed and Web of Science were carried out, using keywords and MeSH subheadings, limited to papers in English dealing with humans. The terms "Parkinson's" or "Lewy body" were used to denote the disease of interest, combined with either "agnosia", "anosognosia", "insight", "metacognition", or "neuropsychology" to denote the neuropsychological area of interest. Results: 21 publications investigating awareness of cognitive symptoms, and 18 publications on awareness of neuropsychiatric symptoms were identified. The large majority focused on Parkinson's disease rather than Dementia with Lewy Bodies. Cognitively intact people with Parkinson's disease may over-report cognitive symptoms, whilst those with cognitive impairment under-report symptoms. Awareness of neuropsychiatric symptoms is likely to decline over time, particularly in those with progressive cognitive impairment. Conclusions: Altered awareness of cognitive and neuropsychiatric symptoms is common in Parkinson's disease. Symptom awareness varies significantly between individuals, and appears to be influenced by mood and global cognitive functioning, with executive functioning specifically implicated. There are gaps in our understanding of how dopaminergic medications influence symptom awareness, and a need for longitudinal studies of how awareness changes over time in Parkinson's disease and Dementia with Lewy Bodies.

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Placebo Response in Fragile X‐associated Tremor/Ataxia Syndrome

Hill

Goetz

Stebbins

et al. 2020

Movement Disord Clin Pract

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Background Fragile X‐associated tremor/ataxia syndrome (FXTAS) is a neurodegenerative disorder characterized by intention tremor, cerebellar ataxia, and executive dysfunction in carriers of a CGG repeat expansion premutation (55–200 repeats) in the fragile X mental retardation 1 (FMR1) gene. Given reports of poor insight in FXTAS, we postulated that patients with FXTAS would be less likely to exhibit placebo response. Objective To analyze placebo response from the first randomized controlled trial in FXTAS that evaluated cognitive and motor outcomes after 1 year of treatment with memantine. Methods Data from the placebo arm of the first randomized controlled trial in FXTAS were analyzed. There were 2 coprimary outcomes. Based on studies in Parkinson's disease, placebo responders were defined as individuals with an improvement of at least 50% in the coprimary outcomes. Improvements of 20% and 30% served as secondary cutoff values based on the suggested magnitude of placebo response in other movement disorders. Results A total of 36 participants in the placebo group completed baseline and follow‐up evaluations. The average age was 66 ± 7 years, and 60% were men. Average CGG repeat size was 86 ± 18. A total of 19 participants had stage 3 disease. Only 1 patient showed 50% improvement in both coprimary outcomes. At 30% and 20% improvement, there were 2 and 3 patients showing placebo response in the coprimary outcomes, respectively. Conclusions Patients with FXTAS exhibited low rates of placebo response in a randomized controlled trial. Further studies on the relationship between baseline insight and placebo responsivity are applicable to FXTAS and other disorders exhibiting cognitive impairment.

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Self‐awareness of executive dysfunction in Huntington's disease: Comparison with Parkinson's disease and cervical dystonia

Cited by 21 publications

References 7 publications

Unawareness of Deficits in Huntington's Disease

Unawareness of Deficits in Huntington's Disease

Altered awareness of cognitive and neuropsychiatric symptoms in Parkinson's disease and Dementia with Lewy Bodies: A systematic review

Placebo Response in Fragile X‐associated Tremor/Ataxia Syndrome

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