Links Between Cognitive Functions and Clinical, Biological and Neuroradiological Outcomes in Adults With Sickle Cell Disease.

NCT ID: NCT04244240

Last Updated: 2025-03-07

Basic Information

• Recruitment Status: COMPLETED
• Total Enrollment: 19 participants
• Study Classification: OBSERVATIONAL
• Study Start Date: 2020-10-13
• Study Completion Date: 2024-04-05

Brief Summary

Sickle cell disease (SCD) is an inherited blood disorder. Symptoms include acute and chronic complications. Due to progress in SCD care, patients with SCD are living longer than before and we focus more attention in chronic complications.

Children with SCD experience worse cognitive functions than healthy children, and fewer is known about cognitive functions in adults. Studies suggest lower cognitive performance in SCD, mostly in executive functions and processing speed, but the biological and anatomical substrates of cognitive decline are not yet well established in SCD. Often times, cognitive impairments and cerebral disorders are not diagnosed and treated in adults with SCD.

The main objective of this study is to propose a deep neuropsychological assessment in adults with SCD and cognitive complaints and to highlight links between cognitive functions and clinical, biological and neuroradiological markers. The hypothesis of this study is that cognitive functions are associated with severity of the SCD, with bood abnormalities, with MRI markers and Transcranial Doppler (TCD) markers of cerebrovascular disease. The secondary objective of this study is to validate a brief cognitive assessment tool (BEARNI tool) in adults with SCD.

This study is an observational cross-sectional study that will enroll adults with SCD and cognitive complaint.

Conditions

Sickle Cell Disease; Drepanocytosis

Study Design

• Observational Model Type: COHORT
• Study Time Perspective: PROSPECTIVE

Study Groups

Sickle cell disease patient

Adults with sickle cell disease (homozygous SS or heterozygous SC, Sβ0 or Sβ+) with cognitive complaint.

BEARNI Tool

Intervention Type: BEHAVIORAL

BEARNI is brief screening tool initially validated for Alcohol-related neuropsychological impairments (Ritz et al., 2015). BEARNI tool detect impairment in visuospatial abilities, executive functions, verbal episodic memory, and verbal working memory.

The score of the test could be expressed as a global score, and also as subscores corresponding to each cognitive subtest. Normative data are available.

Interventions

BEARNI Tool

BEARNI is brief screening tool initially validated for Alcohol-related neuropsychological impairments (Ritz et al., 2015). BEARNI tool detect impairment in visuospatial abilities, executive functions, verbal episodic memory, and verbal working memory.

The score of the test could be expressed as a global score, and also as subscores corresponding to each cognitive subtest. Normative data are available.

Intervention Type: BEHAVIORAL

Eligibility Criteria

Inclusion Criteria

• Age ≥ 18 years old
• Sickle cell anemia (homozygous SS or heterozygous SC, Sβ0, S/C, Sβ+)
• In steady state (without vaso-occlusive crisis or acute chest syndrome at the time of measurements)
• Presence of spontaneous cognitive complaint or requested by the physician.
• Good command of the French language (native language or not)
• No objection to participate in the study
• Affiliated patient or beneficiary of social security scheme

Exclusion Criteria

• Patient not compliant in the management of his disease
• Patient participating in another interventional research protocol that may interfere with this protocol (according to investigator's judgment)
• Language barrier
• Pregnancy or breast feeding
• MRI contraindication
• Patient under guardianship , curatorship or justice
• Inability to express non-opposition

• Minimum Eligible Age: 18 Years
• Eligible Sex: ALL
• Accepts Healthy Volunteers: No

Sponsors

Hospices Civils de Lyon

OTHER

Sponsor Role: lead

Responsible Party

Responsibility Role: SPONSOR

Locations

Hôpital Edouard Herriot

Bron, , France

Countries

France

Other Identifiers

2019-A02709-48

Identifier Type: OTHER

Identifier Source: secondary_id

69HCL19_0753

Identifier Type: -

Identifier Source: org_study_id