Huntington's disease (HD) is a hereditary neurodegenerative disorder characterized by progressively worsening motor, cognitive, psychiatric, and behavioral deficits. Cognitive deficits occur early on, affecting in particular executive functions (inhibition, flexibility), decision-making, memory, attention (selective, sustained), perceptual and visuospatial skills, and information processing speed. More specifically, memory deficits quickly affect different memory systems (short-term memory, long-term memory, etc.), including autobiographical memory. Autobiographical memory is usually defined as a system that stores all the information (semantic component) and specific memories (episodic component) specific to an individual, accumulated from an early age. Autobiographical memory is now considered essential to the construction of a sense of identity and continuity. It is also considered indispensable for projecting into the future, otherwise known as "episodic future thinking," a fundamental human capacity that is both anticipatory and adaptive. Autobiographical memory deficits remain largely unexplored in HD, with only three studies identified in the international literature on the subject, one of which is actually based on the same neuropsychological data as another, adding a neuroanatomical analysis focused on autobiographical memory. These studies show that the autobiographical recollections of patients with HD are mainly descriptive recollections of personal events lacking in detail, and that the abnormalities appear to be linked to the progressive degeneration of a vast cortico-subcortical brain network comprising the medial temporal cortex, the frontal cortex, and the posterior striatal and parietal regions. Deficits in episodic future thinking have never been explored in HD. A better understanding of the mechanisms underlying this type of cognitive impairment (recalling personal memories and mentally simulating future personal events) remains a major challenge today in improving the care of patients with HD. Several recent studies have shown, in different pathological contexts (Alzheimer's disease, etc.), that the parallel use of neuropsychological tests (tasks and questionnaires) and an eye-tracking system allows for a much more accurate and in-depth examination of cognitive functions (for a review, see). In addition, eye movements, such as fixations and saccades, have been associated with the retrieval of autobiographical events . These movements better reflected the person's subjective experience, particularly with regard to the visual elements of mental imagery of recovered events. This suggests that the analysis of eye behavior could enrich the assessment of autobiographical memory, beyond the data provided by traditional tests. The examination of eye movements is therefore, alongside neuropsychological testing, a promising non-invasive method for better understanding the characteristics of autobiographical memory in HD. This project therefore aims to explore the autobiographical memory of HD patients by analyzing their eye activity during tasks involving the recall of personal events using standard neuropsychological tools. By identifying oculomotor markers associated with autobiographical memory disorders, this research could: (1) provide a better understanding of the neurocognitive profile of HD, (2) pave the way for more accurate diagnostic tools, and (3) form an important basis for the development of future interventions aimed at supporting memory function in this population.
Study Type
INTERVENTIONAL
Allocation
NON_RANDOMIZED
Purpose
OTHER
Masking
NONE
Enrollment
80
different neuropsycholocical tests
Eye movement assessment/recording is performed under two conditions: a "control" condition and an "autobiographical recall" condition.
CHU Angers
Angers, France
Assess the recall of personal memories and mental simulation of future personal events in patients with genetically confirmed HD, through analysis of the specificity
It will be assessed using the TEMPau specificity scale. This scale uses a Likert scale ranging from 0 to 4 points (0: no response or general information; 1: vague event without spatial-temporal context; 2: generic or specific event without spatial-temporal context; 3: specific event in a non-detailed spatial-temporal context; 4: specific event in a detailed spatial-temporal context). This scale allows two main scores to be collected for each period of life explored: 1) an overall autobiographical memory score counting all events, regardless of their nature (max.: 3 x 1 = 3) and 2) a strictly episodic score, counting only memories that meet all the criteria for episodicity, rated 4 (max.: 3 x 4 = 12). The scale also provides: 1) an overall autobiographical memory score for all four life periods explored (max.: 3 x 4 = 12) and an overall strictly episodic score, counting only memories that meet all the criteria for episodicity across all four periods, rated 4 (max.: 12 x 4 = 48).
Time frame: inclusion
Assess the recall of personal memories and mental simulation of future personal events in patients with genetically confirmed HD through analysis of the phenomenology
self-questionnaire comprising eight items, measured on a Likert scale ranging from 1 to 4 points (1: no, that is completely untrue; 2: no, that is rather untrue; 3: yes, that is rather true; 4: yes, that is completely true). The 8 items are as follows: 1: I felt like I was reliving the original event; 2: I felt like I was travelling back in time to the moment when the event happened; 3: I don't just know that this event happened, I remember actually experiencing it; 4: I believe that the event in my memory actually happened as I remember it; 5: I can see and hear elements of the event in my mind; 6: I can now feel the emotions I felt when the event happened; 7: I can remember where the event took place; I can remember the time when the event occurred. This scale allows a phenomenological score to be obtained for each period of life explored (max.: 3 x 8 x 4 = 96) and an overall phenomenological score for all four periods of life explored (max.: 3 x 8 x 4 x 4 = 380).
Time frame: inclusion
Assess the recall of personal memories and mental simulation of future personal events in patients with genetically confirmed HD through analysis of the self-defined nature
Each memory is evaluated according to six empirically based dimensions: 1. the specificity of the event (rated from 0 to 4 according to TEMPau criteria, with 0 = extended or semantic memory, 4 = unique event, situated in time and space), 2. identity relevance ('This memory reflects an important part of who I am') 3. the presence of a lasting personal conflict or theme ('This memory is linked to an important theme or conflict in my life') 4. emotional intensity ('This memory expresses a very strong or striking emotion') 5. reflective integration ('This memory has helped me learn a personal lesson') 6. frequency of mental access ('This is a memory I often think back on or talk about regularly') The last five dimensions are assessed by self-questionnaire, on a Likert scale ranging from 1 to 4 (1 = not at all, 4 = completely). This combination gives an overall SDM score ranging from 5 (weakly self-defined memory) to 24.
Time frame: inclusion
Assess the recall of personal memories and mental simulation of future personal events in patients with genetically confirmed HD through analysis of the emotional valence
It will be assessed using the 'Then' and 'Now' scale, which consists of asking the subject to evaluate (self-questionnaire), for each memory, on a Likert scale ranging from -3 to 3, how they felt at the time the event occurred and how they feel when they recall it.
Time frame: inclusion
Assess the recall of personal memories and mental simulation of future personal events in patients with genetically confirmed HD through analysis of the degree of integration of memories
It will be assessed qualitatively using the definitions provided by Blagov and Singer (2004): * Integrative memories: There is a shift away from narrative events and descriptions to make an additional statement about the importance or significance of the memory to the individual. A meaningful statement must go beyond simply saying that the memory is 'important', 'the most painful' or 'a memory I will never forget'. There must be an indication of why the memory is important and moving. * Non-integrative memories: These are discussed in the manual and do not meet the requirements of integrative memories.
Time frame: inclusion
To study the variation in pupil dilation characteristics during the recall of autobiographical memories.
Measured in millimeters (between 1.5 to 8 mm) with Pupil Labs Core monocular device
Time frame: inclusion
To study the variation of eye movements during the recall of autobiographical memories (number).
number of fixation and saccades measured with Pupil Labs Core monocular device
Time frame: inclusion
To study the variation of eye movements during the recall of autobiographical memories (duration).
duration of fixation and saccades measured with Pupil Labs Core monocular device
Time frame: inclusion
Study the links between participants' temporal perspective (past, present, future)and their autobiographical performance
assessed using the ZTPI-short (Zimbardo Time Perspective Inventory - short version)
Time frame: inclusion
analyse the current representation of the self (psychological, social, physical)
identity fluency task: through spontaneous verbal productions
Time frame: inclusion
Evaluate the functional dimension of autobiographical memory (why we use it)
It will be assessed using the TALE-15 (Thinking About Life Experiences) questionnaire
Time frame: inclusion
Investigate clinical and cognitive data from routine assessments with UHDRS
using Unified Huntington's Disease Rating Scale; This scale is divided into several subscales grouping together different tests assessing: motor difficulties (dysarthria, dystonia, choreic movements, bradykinesia, gait disorders, etc.), cognitive difficulties \[flexibility (literal verbal fluency), processing speed (Symbol Digit Modalities Test), inhibition (Stroop task)\], level of independence, and functional difficulties. The TFC (Total Functional Capacity) is a specific subscale of the UHDRS that assesses patients' functional independence. It takes into account five areas: occupational capacity, financial management, domestic management, independence in activities of daily living, and degree of care required. The overall score ranges from 0 (complete dependence) to 13 (complete independence). A high score indicates preserved independence, while a low score reflects a significant loss of independence. TFC is a benchmark indicator of disease severity and progression.
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Time frame: inclusion
Investigate clinical and cognitive data from routine assessments with MMSE
overall efficiency measured using Mini Mental Scale examination
Time frame: inclusion
Investigate clinical and cognitive data from routine assessments with categorical fluency
flexibility measured using categorical fluency
Time frame: inclusion
Investigate clinical and cognitive data from routine assessments with Trail making Test
flexibility measured using Trail making Test
Time frame: inclusion
Investigate clinical and cognitive data from routine assessments with PBA-s
Behaviour troubles measured with Problem Behaviours Assesment scale
Time frame: inclusion