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

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.

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Key information

Conditions

Sickle Cell Disease Anemia Anemia, Hemolytic Anemia, Hemolytic, Congenital Anemia, Sickle Cell Congenital, Hereditary, and Neonatal Diseases and Abnormalities Drepanocytosis Genetic Diseases, Inborn Hematologic Diseases Hemic and Lymphatic Diseases Hemoglobinopathies Sickle Cell Trait

Age range

18 year and older

Sex eligibility

All sexes

Study type

Observational

Primary location

Hôpital Edouard Herriot

Bron, 69437, France

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

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

Treatment and study plan

BEARNI Tool

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.

Primary outcomes

BEARNI questionnaire

Time frame: Day 0

Cognitive performance will be evaluated by a large neuropsychological assessment. The cognitive score will be interpreted as raw scores, z-score adjusted for level of education, age, and sex, according to the tests and binarized into two categories (normal versus pathological performances)

Sponsors and collaborators

Lead sponsor

Hospices Civils de Lyon

Other

Registry information

Official study title

Cognitive Functions in Adults With Sickle Cell Disease and Cognitive Complaints: Neuropsychological Assessment and Links to Demographic, Clinical, Biological, Neuroradiological Outcomes and Validation of a Cognitive Assessment Tool.

Acronym: Drépa-COG

Important dates

Study start: 2020
Primary completion: 2024
Study completion: 2024
First posted: Jan 28, 2020
Registry last updated: Mar 7, 2025

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

Published trials that share one or more normalized conditions with this study.

Completed