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Central nervous system events in children with sickle cell disease presenting acutely with headache
Patrick C Hines1, Therese P McKnight, Wendy Seto
1Division of Hematology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Insights
Acute headaches are common in children with sickle cell disease (SCD-SS), with a higher risk of central nervous system (CNS) events. Specific symptoms and elevated platelets may indicate serious events, warranting imaging.
Area of Science:
- Pediatric Neurology
- Hematology
- Sickle Cell Disease Research
Background:
- Acute headache is a frequent reason for emergency care in children.
- Sickle cell disease (SCD-SS) is associated with various neurological complications.
- Identifying risk factors for central nervous system (CNS) events in pediatric SCD-SS is crucial.
Purpose of the Study:
- To determine the incidence of acute care visits for headache in children with SCD-SS.
- To identify risk factors associated with acute CNS events in this population.
- To evaluate the diagnostic yield of neuroimaging for CNS events in pediatric SCD-SS.
Main Methods:
- Retrospective cohort study analyzing acute care visits for headache in children with SCD-SS.
- Calculation of prevalence for headache visits, neuroimaging, and acute CNS events.
- Assessment of clinical and laboratory variables to identify risk factors.
Main Results:
- Headache accounted for 3.8% of acute care visits in children with SCD-SS.
- Acute CNS events occurred in 6.9% of headache visits.
- Neuroimaging revealed acute CNS events in 16.3% of studies, with older age, prior neurological events, symptoms, focal findings, and elevated platelets being significant risk factors.
Conclusions:
- Acute headaches in pediatric SCD-SS are more commonly linked to acute CNS events than in the general pediatric population.
- Children with a history of stroke, TIA, seizures, neurological symptoms, focal exam findings, or elevated platelets require confirmatory imaging.
- Further prospective studies are needed to determine appropriate workup strategies for other children presenting with headache.
Objective:
To determine the frequency of acute care visits and risk factors for central nervous system (CNS) events in children with homozygous sickle cell disease (SCD-SS) with an acute headache.
Study Design:
This is a retrospective cohort study of acute care visits for headache in children with SCD-SS. The prevalence of headache visits, neuroimaging evaluation, and acute CNS events were calculated and clinical and laboratory variables assessed.
Results:
Headache was the chief complaint in 102 of 2685 acute care visits (3.8%) by children with SCD-SS. Acute CNS events were detected in 6.9% of these visits. Neuroimaging was performed in 42.2% of visits, and acute CNS events were identified in 16.3% of studies. Factors associated with acute CNS events included older age, history of stroke, transient ischemic attack, or seizure, neurologic symptoms, focal neurologic exam findings, and elevated platelets.
Conclusions:
Acute headache is common in pediatric SCD-SS and more frequently associated with acute CNS events than in the general pediatric population. A history of stroke, transient ischemic attack, seizures, neurologic symptoms, focal neurologic exam, or elevated platelet counts at presentation warrant confirmatory imaging studies. Whether a more limited workup is adequate for other children should be confirmed in a larger, prospective study.
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