Abstract
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.
Highlights
Huntington’s disease is an inherited neurodegenerative disease in which patients suffer from behavioral, motor and cognitive disorders
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 Huntington’s disease (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
Fifty-two percent HD2 and 28% HD1 were depressed according to the Montgomery and Asberg Depression rating scale (MADRS)
Summary
Huntington’s disease is an inherited neurodegenerative disease in which patients suffer from behavioral, motor and cognitive disorders. 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. The only study exploring awareness of memory deficits in HD [9]
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