Ataxies SpinoCérébelleuses Autosomiques Dominantes et Cognition Sociale - Etude SoCoSca
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 160
- 试验地点
- 1
- 主要终点
- to assess the social cognition with the Movie for the Assessment of Social Cognition Test from ecological approach
研究概览
简要总结
Spinocerebellar ataxias are a group of rare neurodegenerative diseases, clinically and genetically highly heterogeneous, with an estimated mean prevalence of 2.7 per 100,000 population. The term "spinocerebellar ataxia" or "SCA" is often used for ataxias of genetic origin of autosomal dominant transmission, which are the subject of this study. Recent studies of social cognition in patients with genetic cerebellar pathologies, and autosomal dominant spinocerebellar ataxia in particular, are still few and far between (around 15 studies), and seem to highlight impairment of basic emotion recognition and theory of mind skills. That said, data have very often been collected on very small samples of patients (sometimes in case study format). They also remain contradictory, including in the examination of the cerebellar anatomoclinical correlates of the deficits. Thus, the question arises as to whether patients with spinocerebellar ataxia also show impairments in emotion recognition and cognitive and affective theory of mind in more ecologically valid dynamic and interactive assessment situations.
研究设计
- 研究类型
- Interventional
- 分配方式
- Non Randomized
- 干预模型
- Parallel
- 主要目的
- Other
- 盲法
- None
入排标准
- 年龄范围
- 18 Years 至 100 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 是
入选标准
- •For all participants:
- •Men or women aged 18 and over
- •At least 7 years' schooling (CEP level)
- •Ability to read, write and speak French
- •Signed informed consent to participate in the study
- •For patients :
- •- With molecularly confirmed autosomal dominant spinocerebellar ataxia (SCA1, 2, 3, 6, 7, 27B)
- •For controls:
- •- With no neurological pathology (questioning and neurological examination)
排除标准
- •For patients and controls:
- •Simultaneous participation in another protocol that may interfere with the measurement of the criteria of interest
- •Physical or cultural factors likely to interfere with test performance
- •History likely to interfere with cognition (stroke, cranioencephalic trauma, other neurodegenerative disease, epilepsy, learning disability, alcohol dependence syndrome, psychiatric disorders...)
- •Persons with contraindications to MRI scans
- •Pregnant, nursing or parturient women
- •Persons deprived of their liberty by judicial or administrative decision
- •Persons under compulsory psychiatric care
- •Persons subject to a legal protection measure
- •Persons unable to express their consent
- •Persons not affiliated to or not benefiting from a social security scheme (beneficiary or beneficiary entitled)
研究组 & 干预措施
patients autosomal dominant spinocerebellar ataxia
干预措施: tests (Other)
healthy patients
干预措施: tests (Other)
结局指标
主要结局
to assess the social cognition with the Movie for the Assessment of Social Cognition Test from ecological approach
时间窗: Baseline
evaluates theory of mind in dynamic modality. It was adapted and validated in French (MASC-VF) as part of a partnership between Patricia Garel's team (Hopital Universitaire de Sainte-Justine, Montreal, Quebec) and Isabelle Amado's team (Centre Hospitalier Sainte-Anne, Service Hospitalo-Universitaire, Paris, France). This test assesses both the affective and cognitive sides of theory of mind, and also includes six control questions to rule out a comprehension problem. The test takes around 40 minutes to administer. The test will assess the cognitive and affective theory-of-mind abilities of ALS patients in comparison with those of matched control subjects. The MASC calculates 5 scores: 1 comprehension score, 1 correct response score ranging from 0 to 45 points and 3 error scores (wrong answers are divided into 3 categories: under-interpretation error, over-interpretation error and no interpretation). The higher the score of correct answers, the better the performance.
to assess the social cognition with the Faux Pas Test from classical approach
时间窗: Baseline
Faux Pas Test: in its shortened 10-story version, it assesses the ability to recognize social awkwardness (or faux-pas). Fictitious stories are presented to the person, in paper format: 5 stories contain faux-pas and 5 others do not. The person can reread them as many times as necessary, and must detect whether "someone said something they shouldn't have, or something awkward". If this is the case, she is asked 5 additional questions to assess her understanding of the clumsiness. Each story is followed by 2 comprehension questions, to rule out a comprehension problem. This task takes 20 minutes to administer. The test produces different scores: the higher the score, the better the performance.
to assess the social cognition with the sequencing test from classical approach
时间窗: Baseline
sequencing test: sequential arrangement of 12 scenarios, all consisting of 4 images in the format of short comic strips. They represent mechanical (non-social) events and social scripts including true (4 scenarios) and false beliefs (4 scenarios). Subjects are asked to put each image sequence, presented in random order, back into chronological order. There are 4 non-social scenarios, 4 true-belief scenarios and 4 false-belief scenarios for a 12-point score. The test begins with 2 practice trials. The test lasts 20 minutes.
to assess the social cognition with the social problem-solving test from interactionist approach
时间窗: Baseline
This test has been developped at Angers . A total of 10 stories involving social problems will be proposed. For 5 of them, the participant will have to produce verbal solutions, and for the other 5, act them out in a social role-playing situation, with a partner. The test lasts 40 minutes.
to assess the social cognition with the Facial Emotion Recognition Test from classical approach
时间窗: Baseline
Facial Emotion Recognition Test evaluates the ability to recognize 6 fundamental and universal emotions: happiness, anger, sadness, fear, disgust and contempt. Each emotion is expressed by a different face at 9 levels of expressive intensity, ranging from 20% to 100%
to assess the social cognition with the Reading the Mind in the Eyes test from classical approach
时间窗: Baseline
Reading the Mind in the Eyes test: Thirty-six photographs of gazes (male and female) are presented to the participant, who is asked to select from 4 expression proposals the one that seems most appropriate to the subject's affective mental state (what he or she may be feeling). A lexicon that can be consulted at will is provided to the subject to compensate for any uncertainties concerning the proposed items. The maximum score is 36 (1 point per correctly chosen item). The test takes 15 to 20 minutes to complete.
to assess the social cognition with the French Emotion Evaluation Test from ecological approach
时间窗: Baseline
French Emotion Evaluation Test: audiovisual, ecological and valid emotion recognition test. It tests subjects' ability to recognize basic emotions expressed by other people. It comprises 35 short video sequences (15 to 30 seconds) plus an example item. In each of these sequences, actors interpret everyday situations associated with one of the 6 basic emotions: joy, sadness, anger, surprise, disgust and fear. The test produces a total response score of between 0 and 35.
to assess the social cognition with the sequencing test from classical approach
时间窗: Baseline
sequencing test: sequential arrangement of 12 scenarios, all consisting of 4 images in the format of short comic strips. They represent mechanical (non-social) events and social scripts including true (4 scenarios) and false beliefs (4 scenarios). Subjects are asked to put each image sequence, presented in random order, back into chronological order. There are 4 non-social scenarios, 4 true-belief scenarios and 4 false-belief scenarios for a 12-point score. The test begins with 2 practice trials. The test lasts 20 minutes.
to assess the social cognition with the French Emotion Evaluation Test from ecological approach
时间窗: Baseline
French Emotion Evaluation Test: audiovisual, ecological and valid emotion recognition test. It tests subjects' ability to recognize basic emotions expressed by other people. It comprises 35 short video sequences (15 to 30 seconds) plus an example item. In each of these sequences, actors interpret everyday situations associated with one of the 6 basic emotions: joy, sadness, anger, surprise, disgust and fear. The test produces a total response score of between 0 and 35.
to assess the social cognition with the Facial Emotion Recognition Test from classical approach
时间窗: Baseline
Facial Emotion Recognition Test evaluates the ability to recognize 6 fundamental and universal emotions: happiness, anger, sadness, fear, disgust and contempt. Each emotion is expressed by a different face at 9 levels of expressive intensity, ranging from 20% to 100%
to assess the social cognition with the Faux Pas Test from classical approach
时间窗: Baseline
Faux Pas Test: in its shortened 10-story version, it assesses the ability to recognize social awkwardness (or faux-pas). Fictitious stories are presented to the person, in paper format: 5 stories contain faux-pas and 5 others do not. The person can reread them as many times as necessary, and must detect whether "someone said something they shouldn't have, or something awkward". If this is the case, she is asked 5 additional questions to assess her understanding of the clumsiness. Each story is followed by 2 comprehension questions, to rule out a comprehension problem. This task takes 20 minutes to administer. The test produces different scores: the higher the score, the better the performance.
to assess the social cognition with the Reading the Mind in the Eyes test from classical approach
时间窗: Baseline
Reading the Mind in the Eyes test: Thirty-six photographs of gazes (male and female) are presented to the participant, who is asked to select from 4 expression proposals the one that seems most appropriate to the subject's affective mental state (what he or she may be feeling). A lexicon that can be consulted at will is provided to the subject to compensate for any uncertainties concerning the proposed items. The maximum score is 36 (1 point per correctly chosen item). The test takes 15 to 20 minutes to complete.
to assess the social cognition with the Movie for the Assessment of Social Cognition Test from ecological approach
时间窗: Baseline
evaluates theory of mind in dynamic modality. It was adapted and validated in French (MASC-VF) as part of a partnership between Patricia Garel's team (Hopital Universitaire de Sainte-Justine, Montreal, Quebec) and Isabelle Amado's team (Centre Hospitalier Sainte-Anne, Service Hospitalo-Universitaire, Paris, France). This test assesses both the affective and cognitive sides of theory of mind, and also includes six control questions to rule out a comprehension problem. The test takes around 40 minutes to administer. The test will assess the cognitive and affective theory-of-mind abilities of ALS patients in comparison with those of matched control subjects. The MASC calculates 5 scores: 1 comprehension score, 1 correct response score ranging from 0 to 45 points and 3 error scores (wrong answers are divided into 3 categories: under-interpretation error, over-interpretation error and no interpretation). The higher the score of correct answers, the better the performance.
to assess the social cognition with the social problem-solving test from interactionist approach
时间窗: Baseline
This test has been developped at Angers . A total of 10 stories involving social problems will be proposed. For 5 of them, the participant will have to produce verbal solutions, and for the other 5, act them out in a social role-playing situation, with a partner. The test lasts 40 minutes.
次要结局
- Search for and describe any deficits in social cognition by identifying pathological treshold with Facial Emotion Recognition Test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with Reading the Mind in the Eyes test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with social problem-solving test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with false step test(Baseline)
- Search for and describe any deficits in social cognition by identifying pathological treshold with sequencing test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with brain neuroimaging(3 months)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral and emotional abilities assessed by the Frontal System Behaviour Scale(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with French Emotion Evaluation Test(Baseline)
- Study correlations between social cognition efficiency and - Global cognitive functioning assessed by the CCAS-S scale(Baseline)
- Study correlations between social cognition efficiency and duration of disease progression(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Sequencing test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with Movie for the Assessment of Social Cognition(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and emotional abilities assessed by the Neuropsychiatric Inventory Questionnaire(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Hospital Anxiety and Depression scale(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Ambiguous Intentions Hostility Questionnaire-Short(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Social Cognitive Impairment Rating Scale(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Bermond-Vorst Alexithymia Questionnaire(Baseline)
- Study the correlations between the efficiency of social cognition and the severity of ataxia as assessed by neurofilament dosage.(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by scale of levels of emotional awareness(Baseline)
- Study correlations between social cognition efficiency and imaging data(3 months)
- Study correlations between social cognition efficiency and ataxia severity by the Scale for the Assessment and Rating of Ataxia(Baseline)
- Study the correlations between the efficiency of social cognition and the severity of ataxia as assessed by the International Co-operative Ataxia Rating Scale.(Baseline)
- To study the correlations between the efficiency of social cognition and the global motor skills of the upper limb using the Box and Block test (assessing the gross dexterity of the upper limb tested).(Baseline)
- To study the correlations between the efficiency of social cognition and the fine motor skills by the Nine Hole Peg Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Facial Emotion Recognition Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Reading the Mind in the Eyes test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using social problem-solving test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using False Step Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using French Emotion Evaluation Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Movie for the Assessment of Social Cognition(Baseline)
- Search for and describe any deficits in social cognition by identifying pathological treshold with Facial Emotion Recognition Test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with false step test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with Reading the Mind in the Eyes test(Baseline)
- Search for and describe any deficits in social cognition by identifying pathological treshold with sequencing test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with French Emotion Evaluation Test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with Movie for the Assessment of Social Cognition(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with social problem-solving test(Baseline)
- Research and describe any deficits in social cognition by identifying pathological treshold with brain neuroimaging(3 months)
- Study correlations between social cognition efficiency and - Global cognitive functioning assessed by the CCAS-S scale(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and emotional abilities assessed by the Neuropsychiatric Inventory Questionnaire(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral and emotional abilities assessed by the Frontal System Behaviour Scale(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Hospital Anxiety and Depression scale(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Ambiguous Intentions Hostility Questionnaire-Short(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Social Cognitive Impairment Rating Scale(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by Bermond-Vorst Alexithymia Questionnaire(Baseline)
- Study correlations between social cognition efficiency and Sociobehavioral and Sociobehavioral by scale of levels of emotional awareness(Baseline)
- Study correlations between social cognition efficiency and imaging data(3 months)
- Study correlations between social cognition efficiency and duration of disease progression(Baseline)
- Study correlations between social cognition efficiency and ataxia severity by the Scale for the Assessment and Rating of Ataxia(Baseline)
- Study the correlations between the efficiency of social cognition and the severity of ataxia as assessed by the International Co-operative Ataxia Rating Scale.(Baseline)
- Study the correlations between the efficiency of social cognition and the severity of ataxia as assessed by neurofilament dosage.(Baseline)
- To study the correlations between the efficiency of social cognition and the global motor skills of the upper limb using the Box and Block test (assessing the gross dexterity of the upper limb tested).(Baseline)
- To study the correlations between the efficiency of social cognition and the fine motor skills by the Nine Hole Peg Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Facial Emotion Recognition Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using False Step Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Reading the Mind in the Eyes test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Sequencing test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using French Emotion Evaluation Test(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using Movie for the Assessment of Social Cognition(Baseline)
- Study the supposed differences between SCA subtypes, by comparing pure cerebellar forms with cerebro-cerebellar forms and by comparing the different genetic subtypes with each other, using social problem-solving test(Baseline)