The Montreal Cognitive Assessment.Test in Adults With Sickle Cell Disease (EvaCAD)

Evaluation of the Montreal Cognitive Assessment Test as a Screening Tool for Cognitive Dysfunction in in Adults With Sickle Cell Disease

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.

Study Overview

• Status: Completed
• Conditions: Sickle Cell Anemia
• Intervention / Treatment: Other: Montreal cognitive assessment

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.

• Study Type: Interventional
• Enrollment (Actual): 65
• Phase: Not Applicable

Contacts and Locations

Study Locations

• France
  • Paris, France, 75020
    • Internal Medicine Department - Tenon

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Screening
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm

Intervention / Treatment

Experimental: Intervention; All patients will undergo both the MoCA test and the standardized neuropsychological evaluation 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)

Other: Montreal cognitive assessment; 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)

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
sensitivity of a Montreal Cognitive Assessment score < 26 to detect cognitive impairment confirmed by a neuropsychological standardized evaluation	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)	Month 3

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
specificity of a Montreal Cognitive Assessment score < 26 to detect cognitive impairment confirmed by a neuropsychological standardized evaluation	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)	Month 3
sensitivity of a Montreal Cognitive Assessment score < 23 to detect cognitive impairment confirmed by a neuropsychological standardized evaluation	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)	Month 3
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	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	Month 3
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	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 )	Month 3
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	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)	Month 3

Collaborators and Investigators

• Sponsor: Assistance Publique - Hôpitaux de Paris
• Investigators: Principal Investigator: Jean-Simon RECH, MD, Assistance Publique - Hôpitaux de Paris

Study record dates

Study Major Dates

• Study Start (Actual): October 20, 2022
• Primary Completion (Actual): April 11, 2024
• Study Completion (Actual): October 14, 2024

Study Registration Dates

• First Submitted: April 20, 2022
• First Submitted That Met QC Criteria: April 20, 2022
• First Posted (Actual): April 26, 2022

Study Record Updates

• Last Update Posted (Actual): February 10, 2026
• Last Update Submitted That Met QC Criteria: February 6, 2026
• Last Verified: February 1, 2026

More Information

Terms related to this study

• Keywords: Neuropsychological Tests; cognitive dysfunction; Diagnostic Techniques and Procedures; Montreal cognitive assessment; sensitivity and specificity; Sickle cell anemia
• Additional Relevant MeSH Terms: Mental Disorders; Genetic Diseases, Inborn; Immune System Diseases; Neurocognitive Disorders; Hematologic Diseases; Cognition Disorders; Anemia, Hemolytic, Congenital; Anemia, Hemolytic; Anemia; Hemoglobinopathies; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Hemic and Lymphatic Diseases; Cognitive Dysfunction; Hypersensitivity; Anemia, Sickle Cell; Behavioral Disciplines and Activities; Psychological Tests; Neuropsychological Tests; Mental Status and Dementia Tests
• Other Study ID Numbers: APHP201174; IDRCB 2021-A01842-39 (Other Identifier: ANSM)

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No