Drepanocytosis, Sickle Cell Disease
Conditions
Keywords
Sickle Cell Disease, Drepanocytosis
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.
Interventions
BEHAVIORALBEARNI 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.
Sponsors
Hospices Civils de Lyon
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| BEARNI questionnaire | 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) |
Countries
France
Outcome results
None listed