Neuropsychological Functioning of Youths With Sickle Cell Disease: Comparison With Non-Chronically Ill Peers

Noll, Robert B.; Stith, Laura E.; Gartstein, Maria A.; Ris, M. Douglas; Grueneich, Royal; Vannatta, Kathryn; Kalinyak, Karen

doi:10.1093/jpepsy/26.2.69

Cited by 78 publications

(86 citation statements)

References 23 publications

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“…Although the primary risk factor for psychomotor slowing is stroke, 10 there has been mounting evidence that cognitive impairment also occurs in patients without a history of overt or silent stroke. [11][12][13] Risk factors for cognitive impairment in patients with SCD without stroke are, however, not completely known, 14 particularly in relationship to the SCD genotype. To date, there is only 1 major study characterizing cognitive impairment in adult patients with SCD in the absence of stroke.…”

Section: Introductionmentioning

confidence: 99%

Disease severity and slower psychomotor speed in adults with sickle cell disease

Jorgensen

Metti

Butters³

et al. 2017

Blood Advances

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Section: Introductionmentioning

confidence: 99%

Disease severity and slower psychomotor speed in adults with sickle cell disease

Jorgensen

Metti

Butters³

et al. 2017

Blood Advances

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“…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].…”

Section: Introductionmentioning

confidence: 99%

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

Hijmans

Fijnvandraat

Grootenhuis

et al. 2010

Pediatric Blood & Cancer

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BackgroundSickle cell disease (SCD) can lead to profound cerebral damage, associated with neurocognitive deficits. The aim of the current study was to evaluate a broad range of neurocognitive functions in children with SCD compared to a SES‐matched control group, in order to gain more insight into the specific deficits of these patients.MethodsForty‐one children with homozygous SCD (HbSS or HbS‐β0‐thalassemia) and 38 controls were assessed on a comprehensive set of well‐defined and validated measures of neurocognitive functioning. Besides general intelligence, we evaluated executive functioning extensively (including response inhibition, sustained attention, planning, visuo‐spatial working memory, and verbal working memory) as well as visuo‐motor functioning.ResultsSCD was clearly associated with lower IQ scores. More than one in three children with SCD had a Full‐scale IQ below 75. Furthermore, children with SCD showed deficits in visuo‐motor functioning. Some evidence was found for executive dysfunction: Children with SCD displayed poor visuo‐spatial working memory, as well as subtle deficits in sustained attention and planning. No significant differences were found between children with SCD and controls in terms of response inhibition and verbal working memory.ConclusionsChildren with SCD are at increased risk of lower intelligence, visuo‐motor impairments, and executive dysfunction. These neurocognitive deficits may underlie high rates of scholastic impairments in these children. The present findings further illuminate the importance of regular neurocognitive evaluations and future neurocognitive rehabilitation programs for children with SCD. Pediatr Blood Cancer 2011;56:783–788. © 2010 Wiley‐Liss, Inc.

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“…Neurocognitive deficits among children with sickle cell anemia (SCA) have been reported in a variety of domains, [1][2][3][4][5][6] but deficiencies in intellectual function (IQ) have been the most consistently documented. 3,[6][7][8][9][10] Cognitive dysfunction in SCA appears to worsen with age, 7,11 and individuals with SCA are now living longer.…”

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confidence: 99%

Neuroimaging abnormalities in adults with sickle cell anemia

Mackin

Insel²,

Truran³

et al. 2014

Neurology

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Objective: This study was conducted to determine the relationship of frontal lobe cortical thickness and basal ganglia volumes to measures of cognition in adults with sickle cell anemia (SCA).Methods: Participants included 120 adults with SCA with no history of neurologic dysfunction and 33 healthy controls (HCs). Participants were enrolled at 12 medical center sites, and raters were blinded to diagnostic group. We hypothesized that individuals with SCA would exhibit reductions in frontal lobe cortex thickness and reduced basal ganglia and thalamus volumes compared with HCs and that these structural brain abnormalities would be associated with measures of cognitive functioning (Wechsler Adult Intelligence Scale, 3rd edition). Results:

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Neuropsychological Functioning of Youths With Sickle Cell Disease: Comparison With Non-Chronically Ill Peers

Cited by 78 publications

References 23 publications

Disease severity and slower psychomotor speed in adults with sickle cell disease

Disease severity and slower psychomotor speed in adults with sickle cell disease

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

Neuroimaging abnormalities in adults with sickle cell anemia

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