Language Processing Deficits in Sickle Cell Disease in Young School-Age Children

Abstract

Verbal IQ deficits are frequently reported for school-age children with sickle cell disease (SCD), yet the profile of language abilities in SCD is unclear. We examined semantic, syntactic, and phonological processing in five-to-seven-year-olds at high neurologic risk based on SCD subtype (N = 33), at low neurologic risk with SCD (N = 21), and without SCD (N = 54). Low-risk SCD did not show language processing deficits. High-risk SCD showed deficits in all three language domains. Language processing deficits in SCD at the start of middle childhood are related to neurologic risks and include language skills beyond vocabulary.

Academic Performance and Intelligence Scores of Primary School-aged Children with Sickle Cell Anemia

“…The similarity in other subjects agrees with the observations of other workers [8]. The observed difference in English language in this study may be due to impairment in language skills outside vocabulary, especially in children with cerebral infarcts [20]. However, this possibility was not explored in this study, as it was not designed to explore this.…”

“…These children performed significantly less than SCA patients without infarcts on assessed tests [21]. Silent cerebral infarcts have been found to have direct impact on the intellectual abilities of affected children [20,21]. However, whether SCA children with poor academic performance in this study had silent cerebral infarcts was not determined.…”

Neurocognitive deficits in children with sickle cell disease: a comprehensive profile

“…These deficits have previously been observed in children with SCD [14,16,34], although findings in this area have been remarkably inconsistent [3,12,38]. In the present study, a visual working memory measurewas chosenthat is relatively independentofmotor-functioning (N-Back task) and an instrument to assess visuo-motor functioning was included as well (Beery VMI), as was recently recommended [16].…”

“…In addition to detrimental effects on general cognitive functioning, SCD has been associated with deficits in specific areas of neurocognitive functioning including executive functioning [1,[9][10][11][12][13][14] and visuo-motor functions [15,16]. Deficits in these two areas are expected, given that silent infarcts commonly occur in frontal lobe white matter, within the border zone between the middle and anterior cerebral artery distribution [17,18].…”

“…Although deficits on this neurocognitive domain have been recognized in children with SCD, findings remain inconsistent [1]. Some researchers have reported average motor skills in children with SCD [10,20] while others found clear deficits compared to healthy controls [15,16]. Many studies investigated either visual-spatial or motor skills, but not both [1].…”