Poor insight into memory impairment in patients with Huntington disease

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

doi:10.5114/ninp.2012.30262

Cited by 13 publications

(33 citation statements)

References 43 publications

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“…Furthermore, since HD is a neurodegenerative condition, individual’s in the later stages often exhibit anosognosia, or a lack of insight into one’s own symptoms and deficits (53–55). Anosognosia can compromise the reliability of a PRO, highlighting the importance of capturing information from another source (i.e., ClinRO and/or ObsRO).…”

Section: Discussionmentioning

confidence: 99%

Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review

Carlozzi

Miciura

Migliore

et al. 2014

Journal of Huntington's Disease

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Background:The identification of the gene mutation causing Huntington disease has raised hopes for new treatments to ease symptoms and slow functional decline. As such, there has been a push towards designing efficient pharmacological trials (i.e., drug trials), especially with regard to selecting outcomes measures that are both brief and sensitive to changes across the course of the disease, from subtle prodromal changes, to more severe end-stage changes. Objectives: Recently, to aid in efficient development of new HD research studies, the National Institute of Neurological Disorders and Stroke (NINDS) published recommendations for measurement selection in HD. While these recommendations are helpful, many of the recommended measures have little published data in HD. As such, we conducted a systematic review of the literature to identify the most common outcomes measures used in HD clinical trials. Methods: Major medical databases, including PubMed, Embase, CINAHL, and the Cochrane Central Register of Controlled Trials, were used to identify peer-reviewed journal articles in English from 2001 through April 2013; 151 pharmacological trials were identified. Results: The majority of HD clinical trials employed clinician-reported outcomes measures (93%); patient reported outcome measures (11%) and observer reported outcome measures (3%) were used with much less frequency. Conclusions: We provide a review of the most commonly used measures across these trials, compare these measures to the clinical recommendations made by the NINDS working groups, and provide recommendations for selecting measures for future clinical trials that meet the Food and Drug Administration standards.

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Section: Discussionmentioning

confidence: 99%

Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review

Carlozzi

Miciura

Migliore

et al. 2014

Journal of Huntington's Disease

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“…This measure is designed to capture PROs for individuals with HD. However, there is much discussion in the HD community about an individual's ability to provide reliable self-report data during the later phases of the disease, when both cognitive problems and anosognosia are common 33–35. To this end, the general consensus is that self-report measures, by themselves, only capture one component of the clinical picture.…”

Section: Discussionmentioning

confidence: 99%

HD-PRO-TRIAD™ Validation: a Patient-Reported Instrument for the Symptom Triad of Huntington Disease

Carlozzi

Victorson

Sung

et al. 2014

Tremor and Other Hyperkinetic Movements

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“…In particular, their memory is impaired, showing poor retrieval capacity [1]. HD patients have been described as unaware of their motor symptoms, their behavioral disorders, their cognitive deficits [2], [3], [4], [5], [6], [7], [8], but also recently their memory deficits [9]. Although unawareness of memory deficits is a classical clinical feature in patients with Alzheimer's disease (AD) [10], [11], it was recently shown that patients with Parkinson's disease (PD) can report their own memory difficulties through auto-questionnaires [12].…”

Section: Introductionmentioning

confidence: 99%

“…Although unawareness of memory deficits is a classical clinical feature in patients with Alzheimer's disease (AD) [10], [11], it was recently shown that patients with Parkinson's disease (PD) can report their own memory difficulties through auto-questionnaires [12]. Given that both PD and HD are basal ganglia neurodegenerative disorders yielding executive dysfunction and memory retrieval deficits [13], preserved vs. impaired awareness for memory deficits in PD [12] vs. in HD [9] respectively would suggest that unawareness for memory deficit might not depend on striatal dysfunction per se but on another neural basis. If this hypothesis were true, one would expect good awareness of memory deficits at early stage Huntington's disease where degeneration predominates in the striatum [14], [15] and unawareness of these deficits at more advanced stages.…”

Section: Introductionmentioning

confidence: 99%

“…If this hypothesis were true, one would expect good awareness of memory deficits at early stage Huntington's disease where degeneration predominates in the striatum [14], [15] and unawareness of these deficits at more advanced stages. However the only study exploring awareness of memory deficits in HD [9] included patients with different levels of independence (from 60 to 100% on the independence scale [16]) (Sitek et al, personal communication) suggesting mild to moderately severe stages of the disease [17].…”

Section: Introductionmentioning

confidence: 99%

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Awareness of Memory Deficits in Early Stage Huntington's Disease

et al. 2013

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Patients with Huntington's disease (HD) are often described as unaware of their motor symptoms, their behavioral disorders or their cognitive deficits, including memory. Nevertheless, because patients with Parkinson's disease (PD) remain aware of their memory deficits despite striatal dysfunction, we hypothesize that early stage HD patients in whom degeneration predominates in the striatum can accurately judge their own memory disorders whereas more advanced patients cannot. In order to test our hypothesis, we compared subjective questionnaires of memory deficits (in HD patients and in their proxies) and objective measures of memory dysfunction in patients. Forty-six patients with manifest HD attending the out-patient department of the French National Reference Center for HD and thirty-three proxies were enrolled. We found that HD patients at an early stage of the disease (Stage 1) were more accurate than their proxies at evaluating their own memory deficits, independently from their depression level. The proxies were more influenced by patients' functional decline rather than by patients' memory deficits. Patients with moderate disease (Stage 2) misestimated their memory deficits compared to their proxies, whose judgment was nonetheless influenced by the severity of both functional decline and depression. Contrasting subjective memory ratings from the patients and their objective memory performance, we demonstrate that although HD patients are often reported to be unaware of their neurological, cognitive and behavioral symptoms, it is not the case for memory deficits at an early stage. Loss of awareness of memory deficits in HD is associated with the severity of the disease in terms of CAG repeats, functional decline, motor dysfunction and cognitive impairment, including memory deficits and executive dysfunction.

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Poor insight into memory impairment in patients with Huntington disease

Abstract: Huntington disease patients underestimate memory dysfunction. These results add to the previous reports on poor insight in HD in other domains and suggest that anosognosia in HD, albeit usually rather mild, may be a generalized phenomenon.

Cited by 13 publications

References 43 publications

Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review

Understanding the Outcomes Measures used in Huntington Disease Pharmacological Trials: A Systematic Review

HD-PRO-TRIAD™ Validation: a Patient-Reported Instrument for the Symptom Triad of Huntington Disease

Awareness of Memory Deficits in Early Stage Huntington's Disease

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