Children with Sickle Cell Disease (SCD) face more developmental delays and cognitive challenges compared to unaffected peers . Neurocognitive deficits in children with SCD are well documented, and include impaired overall intellectual ability, executive functioning, processing speed, attention, memory, language, and academic achievement .
The neurological burden of SCD is severe; the developing brain is especially vulnerable to hypoxemia and ischemia, which children with SCD often experience. In the first decade of life, approximately one-third of children with SCD will have silent cerebral infarction (SCI) , and by 20 years old, 39% of individuals with Sickle Cell Anemia (SCA) will have an overt stroke . An overt stroke causes observable symptoms such as asymmetrical arm weakness or speech impediment, and is defined as neurological deficit lasting for over 24 hours . SCI describes abnormal magnetic resonance imaging (MRI) of the brain, or dead cerebral tissue found by neuroimaging without a history or physical findings of an overt stroke . Overt strokes commonly occur in lesions of the cerebral cortex and deep white matter. SCI occur in various regions of the brain, including the thalamus, temporal lobes, and the deep white matter of the frontal or parietal lobes . The cerebral cortex is responsible for higher-order brain functions such as sensation, perception, working memory, processing speed, verbal comprehension, and visual motor speed coordination .
Silent Cerebral Infarcts
Cerebral injury limits the full potential of a developing child or adult. SCI is the most common neurological disease in children with sickle cell . SCI gained widespread awareness in 1996, when the Cooperative Study of Sickle Cell Disease (CSSCD) standardized MRI radiological parameters to define SCI . The results of an observational study conducted by the CSSCD supported that the presence of SCI, in children with SCD, is a risk factor for additional neurological injury. Notably, a 14-fold increased risk of clinical stroke, and higher risk of progressive silent infarction . Children with SCI exhibit lower cognitive test scores than children with a normal MRI of the brain , . SCI have been associated with neurological deficits in executive functions such as selective attention, working memory, processing speed, visuomotor coordination, vocabulary, visual memory, abstract reasoning, and verbal comprehension , –. Consequentially, SCD greatly impacts academic achievement, with one study finding that 35% of children with SCA and SCI had twice the likelihood of academic difficulties than those without SCI .
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