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Overt and incomplete (silent) cerebral infarction in sickle cell anemia: diagnosis and management
Wing-Yen Wong1, Darleen R Powars
1Department of Pediatrics, Division of Hematology/Oncology, Children's Hospital Los Angeles, Keck School of Medicine at the University of Southern California, Los Angeles, CA, USA.
Insights
Sickle cell anemia (HbSS) can cause strokes in children and adults. Early diagnosis using clinical and neuroimaging tools is crucial for managing neurocognitive effects and preventing future strokes.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Sickle cell anemia (HbSS) is linked to intracranial artery stenosis, leading to severe strokes in children.
- Incomplete cerebral infarctions are more common than previously thought and impact neurodevelopment.
- A concerning
Purpose of the Study:
- To outline an algorithmic approach for diagnosing stroke in sickle cell disease patients.
- To highlight clinical and neuroimaging modalities for stroke detection.
- To address the rising incidence of stroke in young adults with HbSS.
Main Methods:
- Review of clinical and neuroimaging studies.
- Sequential ordering of diagnostic modalities based on yield and availability.
- Focus on identifying subtle stroke findings.
Main Results:
- Incomplete infarctions have significant neurocognitive consequences.
- Stroke risk persists into adulthood, with a notable increase in the third decade.
- An algorithmic approach aids in timely diagnosis.
Conclusions:
- Early and accurate stroke diagnosis in HbSS is vital.
- Neuroimaging and clinical assessments are key to managing stroke risk.
- Increased vigilance is needed for stroke detection in young adults with HbSS.
Abstract:
Regional complete infarctions in children with sickle cell anemia (HbSS) are often associated with stenosis of the large intracranial arteries and result in lifetime disability. Incomplete infarction occurs more frequently than previously recognized and has far-reaching effects on neurocognitive development and the risk for overt secondary strokes into adulthood. Clinical and neuroimaging modalities have been highlighted in an algorithmic approach, with the studies giving the highest yield in results and most likely to be available listed in sequential order. The recognition of an emerging "second peak" incidence in the third decade of life is worrisome and warrants more intense scrutiny and diagnosis of subtle findings of stroke in this young adult population.
