Sickle Cell Anemia
Conditions
Keywords
Sickle cell anemia, cognitive dysfunction, sensitivity and specificity, Diagnostic Techniques and Procedures, Neuropsychological Tests, Montreal cognitive assessment
Brief summary
This study will assess the performances of the Montreal Cognitive Assessment (MoCA) to screen for cognitive impairment in adults with sickle cell anemia. The results of the MoCA and its subscores will be compared to a standardized neuropsychological evaluation using validated tests.
Detailed description
Sickle cell anemia is a monogenic disease affecting the beta-chain of hemoglobin. Cognitive impairment is more prevalent in patients with sickle cell disease than in matched unaffected individuals. Validated tests to screen for cognitive impairment are lacking in this population. Our goal is therefore to assess the performance of the Montreal Cognitive Assessment (MoCA) as a screening tool for cognitive impairment in adults with sickle cell anemia. The results of the MoCA and its subscales will be compared to a standardized neuropsychological evaluation using validated tests.
Interventions
Montreal cognitive assessment and standardized neuropsychological evaluation (Test of Attentional Performance of Zimmermann and Fimm ; Wechsler Adult Intelligence Scale 4th version digit span ; Wechsler memory scale III, spatial span ; Wisconsin Card Scoring Test, GREFEX version ; Wechsler Adult Intelligence Scale 4th version block design ; Six elements test GREFEX version; free and cued selective reminding test, 16 items, B version ; Behavioral Rating Inventory of Executive Function ; Modified Fatigue Impact Scale)
Sponsors
Assistance Publique - Hôpitaux de Paris
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
SCREENING
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Sickle cell disease, SS or SBêta0 subtypes only * Attending a consultation or a daycare hospitalization in the Tenon hospital sickle cell center * Aged 18 years old or more * Providing written informed consent * With Health Insurance
Exclusion criteria
* Obvious cognitive impairment of neurologic (e.g. previous stroke) or psychiatric origin (e.g. psychotic disorder with negative symptoms) * Insufficient French language fluency to understand the tests, inability to read and write or with no schooling beyond primary school * Anticipated unavailability in the next 6 months * Patients under supervision \& guardianship
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| sensitivity of a Montreal Cognitive Assessment score < 26 to detect cognitive impairment confirmed by a neuropsychological standardized evaluation | Month 3 | sensitivity of a Montreal Cognitive Assessment score \< 26 to detect cognitive impairment defined as an abnormal result in at least one of the following tests : Test of Attentional Performance of Zimmermann and and Fimm (\< 5th percentile in at least one of the subscales) ; Wechsler Adult Intelligence Scale 4th version digit span (standard score ≤ 5) ; Wechsler memory scale III, spatial span (standard score ≤ 5) ; Wisconsin Card Scoring Test, GREFEX version (number of categories ≤ 5th percentile or if the number of perseverations is ≥ 95th percentile) Wechsler Adult Intelligence Scale 4th version block design (standard score ≤ 5) ; Six elements test GREFEX version (rank score ≤ 5th percentile ); free and cued selective reminding test, 16 items, B version (Z-score ≤ -1,65 in at least one of the subscales) |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| specificity of a Montreal Cognitive Assessment score < 26 to detect cognitive impairment confirmed by a neuropsychological standardized evaluation | Month 3 | specificity of a Montreal Cognitive Assessment score \< 26 to detect cognitive impairment defined as an abnormal result in at least one of the following tests : Test of Attentional Performance of Zimmermann and Fimm (\< 5th percentile in at least one of the subscales) ; Wechsler Adult Intelligence Scale 4th version digit span (standard score ≤ 5) ; Wechsler memory scale III, spatial span (standard score ≤ 5) ; Wisconsin Card Scoring Test, GREFEX version (number of categories ≤ 5th percentile or if the number of perseverations is ≥ 95th percentile); Wechsler Adult Intelligence Scale 4th version block design (standard score ≤ 5) ; Six elements test GREFEX version (rank score ≤ 5th percentile); free and cued selective reminding test, 16 items, B version (Z-score ≤ -1,65 in at least one of the subscales) |
| sensitivity of a Montreal Cognitive Assessment score < 23 to detect cognitive impairment confirmed by a neuropsychological standardized evaluation | Month 3 | sensitivity of a Montreal Cognitive Assessment score \< 23 to detect cognitive impairment defined as an abnormal result in at least one of the following tests : Test of Attentional Performance of Zimmermann and Fimm (\< 5th percentile in at least one of the subscales) ; Wechsler Adult Intelligence Scale 4th version digit span (standard score ≤ 5) ; Wechsler memory scale III, spatial span (standard score ≤ 5) ; Wisconsin Card Scoring Test, GREFEX version (number of categories ≤ 5th percentile or if the number of perseverations is ≥ 95th percentile) * Wechsler Adult Intelligence Scale 4th version block design (standard score ≤ 5) ; Six elements test GREFEX version (rank score ≤ 5th percentile) ; free and cued selective reminding test, 16 items, B version (Z-score ≤ -1,65 in at least one of the subscales) |
| sensitivity and specificity of a score ≤ 5/6 in the attention subscale of the Montreal Cognitive Assessment to detect attentional impairment confirmed by a neuropsychological standardized evaluation | Month 3 | sensitivity and specificity of a score ≤ 5/6 in the attention subscale of the Montreal Cognitive Assessment to detect attentional impairment as defined by a Test of Attentional Performance of Zimmermann and Fimm \< 5th percentile in at least one of the subscales of the test |
| sensitivity and specificity of a score ≤ 4/5 in the visuospatial/executive subscale of the Montreal Cognitive Assessment to detect executive impairment confirmed by a neuropsychological standardized evaluation | Month 3 | sensitivity and specificity of a score ≤ 4/5 in the visuospatial/executive subscale of the Montreal Cognitive Assessment to detect executive impairment as defined by an abnormal result in one of the following test : Wisconsin Card Scoring Test, GREFEX version (number of categories ≤ 5th percentile or if the number of perseverations is ≥ 95th percentile); Wechsler Adult Intelligence Scale 4th version block design (standard score ≤ 5) ; Six elements test GREFEX version (rank score ≤ 5th percentile ) |
| sensitivity and specificity of a score ≤ 4/5 in the delayed recall subscale of the Montreal Cognitive Assessment to detect a long term memory impairment confirmed by a neuropsychological standardized evaluation | Month 3 | sensitivity and specificity of a score ≤ 4/5 in the attention subscale of the Montreal Cognitive Assessment to detect attentional impairment as defined by a free and cued selective reminding test, 16 items, B version Z-score ≤ -1,65 (in at least one of the subscales) |
Countries
France
Contacts
PRINCIPAL_INVESTIGATORJean-Simon RECH, MD
Assistance Publique - Hôpitaux de Paris
Outcome results
None listed