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Published on: April 21, 2017
Acute silent cerebral infarction in children with sickle cell anemia
Michael M Dowling1, Charles T Quinn, Zora R Rogers
1Department of Pediatrics, The University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390-9063, USA. michael.dowling@utsouthwestern.edu
Insights
Silent cerebral infarctions (SCI) in children with sickle cell anemia are often missed. Acute SCI can be detected using MRI, even with subtle symptoms, and may result from anemia.
Area of Science:
- Pediatric Neurology
- Hematology
- Radiology
Background:
- Silent cerebral infarctions (SCI) affect up to 35% of children with sickle cell anemia (HbSS).
- Acute SCI are difficult to identify within the critical 10-14 day window using diffusion-weighted MRI.
- Recognizing acute SCI is crucial for understanding disease progression and potential interventions.
Purpose of the Study:
- To investigate the detectability and characteristics of acute silent cerebral infarctions (SCI) in children with sickle cell anemia (HbSS).
- To explore the association between acute anemic events and the occurrence of SCI.
- To determine if acute SCI lead to permanent neurological deficits.
Main Methods:
- Case series reporting on seven children with HbSS and suspected acute SCI.
- Utilized diffusion-weighted magnetic resonance imaging (MRI) to identify areas of restricted diffusion.
- Correlated MRI findings with clinical presentation, including neurologic deficits and hematologic status.
Main Results:
- Seven children with HbSS were identified with acute SCI, characterized by restricted diffusion on MRI.
- Four of these cases were associated with preceding acute anemic events.
- No persistent focal neurological deficits were observed in the affected children despite MRI evidence of acute infarction.
Conclusions:
- Acute silent cerebral infarctions (SCI) in children with sickle cell anemia are detectable via MRI.
- SCI may present with subtle or absent focal neurological deficits.
- Acute anemic events appear to be a potential cause of acute SCI, which can lead to permanent neurological injury.
Abstract:
Silent cerebral infarctions (SCI) occur in up to 35% of children with sickle cell anemia (HbSS) but are rarely recognized during the initial 10-14 days when diffusion weighted magnetic resonance imaging (MRI) can differentiate acute infarctions from remote events. We report acute SCI in seven children with HbSS who had areas of restricted diffusion on MRI without persistent focal neurologic deficits. Four had acute SCI identified following acute anemic events. Our observations suggest that SCI are detectible in the acute phase, present with subtle neurologic symptoms, result in permanent neurologic injury, and may be caused by acute anemic events.
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