Recurrent large volume silent strokes in sickle cell disease

Area of Science:

• Neurology
• Hematology
• Vascular Medicine

Background:

• Cerebrovascular events are a frequent complication of sickle cell disease (SCD).
• The pathophysiology of these events in SCD is multifactorial.
• Large intracranial artery stenosis is an underrecognized contributor to stroke in SCD.

Observation:

• A 30-year-old woman with SCD experienced a silent large-volume stroke.
• Two months later, she had a second large-volume stroke with transient arm weakness.
• Magnetic resonance angiography revealed segmental stenosis in two major intracranial arteries supplying the stroke areas.

Findings:

• Partial regression of the identified vascular stenosis was observed on follow-up magnetic resonance imaging.
• The findings suggest a dynamic process of stenosis in large intracranial vessels in SCD.
• Reticulocyte and inflammatory element adhesion to vascular endothelium may promote injury and stenosis.

Implications:

• Stenosis of large intracranial vessels is a significant mechanism for large-volume brain infarcts in sickle cell disease.
• This case underscores the need for monitoring intracranial vascular health in SCD patients.
• Further research into the role of endothelial adhesion in SCD-related cerebrovascular events is warranted.