Autosomal Dominant Spinocerebellar Ataxias and Social Cognition
Trial statusRecruiting
Trial phaseNot applicable
Trial typeInterventional
Biological sexAll
Age18-100
SponsorUniversity Hospital, Angers
About this trial
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.
Eligibility criteria
This trial accepts healthy volunteersQualifiers
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
Disqualifiers
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
Trial design
Design model
Parallel
Treatments tested in this trial
tests
Other interventionneuropsychological tests + Neuroimaging
Treatment groups
160 Participants
are divided into 2 treatment groupsGroup A: patients autosomal dominant spinocerebellar ataxiaActive comparator 1 intervention
Group B: healthy patientsActive comparator 1 intervention
Trial outcomes
Primary outcomes
1
to assess the social cognition with the Facial Emotion Recognition Test from classical approach
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%
Time frame
Baseline2
to assess the social cognition with the Faux Pas Test from classical approach
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.
Time frame
Baseline3
to assess the social cognition with the Reading the Mind in the Eyes test from classical approach
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.
Time frame
Baseline4
to assess the social cognition with the sequencing test from classical approach
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.
Time frame
BaselineSecondary outcomes
1
Search for and describe any deficits in social cognition by identifying pathological treshold with Facial Emotion Recognition Test
this 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%.
Time frame
Baseline2
Research and describe any deficits in social cognition by identifying pathological treshold with false step test
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 yields different scores: the higher the scores, the better the performance.
Time frame
Baseline3
Research and describe any deficits in social cognition by identifying pathological treshold with 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, which 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.
Time frame
Baseline4
Search for and describe any deficits in social cognition by identifying pathological treshold with sequencing test
Subjects must 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.
Time frame
BaselineSponsors and contacts
Click on the lead sponsor to view all of their trials.
Source ClinicalTrials.gov