Validation of the Performance of the French Version of the Cerebellar Cognitive-Affective Syndrome Scale (CCASS) as a Screening Test for Cerebellar Cognitive-affective Syndrome: a National Multicenter Study

The primary objective of the study is to assess the performance of the French version of the Cerebellar Cognitive-Affective Syndrome Scale (CCASS) as a screening test for cerebellar cognitive-affective syndrome.

The primary endpoint will be the sensitivity of version 1A of the French scale. The result will be considered positive if the patient fails at least one of the scale's subtests. The diagnosis of a cerebellar cognitive-affective syndrome will be made on the basis of a pathological score in the executive, language, visuospatial or psychoaffective domains of the neuropsychological evaluation (gold standard).

Secondary objectives:

1. To determine the other accuracy measures for version 1A of the French CCAS scale;
2. To determine the performance indices (i.e., sensitivity, specificity) of French versions 1B, 1C and 1D of CCAS scale;
3. To determine the performance indices of versions 1A, 1B, 1C, and 1D of CCAS scale when positivity is defined by ≥ 2 or ≥ 3 failed subtests, respectively;
4. To evaluate the consistency of results from versions 1B, 1C and 1D compared with version 1A;
5. To assess the inter-rater reliability of the different versions of the French scale;
6. To assess the learning effect on version 1A of the French scale in the subgroup of patients included at Mulhouse hospital;
7. To measure the time taken by patients to complete version 1A of the French scale.

   Conduct of research:

   The study will take place in the following french centers: GHRMSA - Hôpital Emile Muller (Mulhouse), AP-HP - Hôpital Pitié-Salpêtrière (Paris), CHRU Nancy - Hôpital Central (Nancy), Hôpitaux civils de Colmar (Colmar).

   - V0 Screening: The protocol will be proposed to all eligible patients by the investigator.

   - V1 Inclusion visit and CCASS test: The inclusion visit will be scheduled on the date of the patient's next follow-up medical appointment.

   The CCASS will be administered by a neuropsychologist starting with version 1A. An alternative version (1B, 1C or 1D) will also be administered. An interval of approximately 30 minutes should be observed between the two administrations. In order to assess the inter-rater reliability of the scale, a second neuropsychologist will be present in the room and will independently evaluate (i.e., blind to his/her colleague's rating) patient's responses, without directly interfering in the administration of the scale.

   The choice of alternative version (i.e., 1B, 1C, or 1D) will be randomized.

   - V2 Gold standard: Within 7 days of completing CCASS, a complete neuropsychological assessment will be carried out by a third neuropsychologist, blinded to the results of the scale.

   - V3 Re-testing of version 1A of CCASS: In the subgroup of patients included at Mulhouse hospital, a second administration of version 1A of CCASS will be carried out by one of the two neuropsychologists who carried out the V1 visit.

   V3 will be scheduled 80 to 100 days after V1..

Eligibility Criteria:
Inclusion criteria :

* French speaking
* Acquired lesion or predominantly degenerative cerebellar pathology of any etiology
* Affiliated or beneficiary of a social security scheme
* Written informed consent

Non-inclusion criteria:

* History or progressive pathology of the central nervous system other than cerebellar disease
* Chronic ethylic patient with active consumption, or alcohol withdrawal of less than three months
* Use of other toxic substances
* Blind or severely visually impaired patient
* Deaf or severely hearing-impaired patient unable to understand instructions
* Mute patient
* Patient deprived of liberty or under legal protection

Secondary exclusion criteria :

- Patients with incomplete French scale and/or neuropsychological assessment will be replaced.