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.

About the study

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.

Study point of contact

Romain FORT, MD, PhD
04 72 11 91 85 ext +33
04 72 43 21 27 ext +33


1 France site


> 18 Years

Study type








participation requirements

– 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

– 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

participation restrictions

– 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


  • Bron, France, Hôpital Edouard Herriot, 69437 [Recruiting]
Last updated 2020-11-17