Cognitive screening examinations for silent cerebral infarcts in sickle cell disease

DeBaun, Michael R.; Schatz, Jeffrey; Siegel, Marilyn J.; Koby, Myles; Craft, Suzanne; Resar, Linda M.S.; Chu, Jhih‐Wei; Launius, G.; Dadash-Zadeh, Mahboubeh; Lee, R. B.; Noetzel, Michael J.

doi:10.1212/wnl.50.6.1678

Cited by 135 publications

(89 citation statements)

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“…Most researchers investigating executive functions of children with SCD focused on mental flexibility and sustained attention, overlooking other relevant frontal lobe functions such as response inhibition and planning [9][10][11][12]34]. The results of the present study failed to find support for deficits in response inhibition and verbal working memory.…”

Section: Discussioncontrasting

confidence: 95%

“…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].…”

Section: Discussionmentioning

confidence: 99%

“…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%

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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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Section: Discussioncontrasting

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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

Hijmans

Fijnvandraat

Grootenhuis

et al. 2010

Pediatric Blood & Cancer

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“…Cerebral infarction can cause impaired cognitive abilities with devastating physical impairments (Fowler et al, 1988;Swift et al, 1989;Brown et al, 1993;Armstrong et al, 1996;DeBaun et al, 1998;Brown et al, 2000;Steen et al, 2003b). Most recently, it has been recognised that 30% of adults with sickle cell disease develop pulmonary hypertension, which is strongly associated with a high rate of sudden death (Gladwin et al, 2004).…”

Section: Discussionmentioning

confidence: 99%

Matched‐related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research

Panepinto¹,

Walters²,

Carreras³

et al. 2007

Br J Haematol

162

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SummaryWe report outcomes after myeloablative haematopoietic cell transplantation (HCT) from human leucocyte antigen (HLA)‐matched sibling donors in 67 patients with sickle cell disease transplanted between 1989 and 2002. The most common indications for transplantation were stroke and recurrent vaso‐occlusive crisis in 38% and 37% of patients respectively. The median age at transplantation was 10 years and 67% of patients had received >10 red blood cell transfusions before HCT. Twenty‐seven percent of patients had a poor performance score at transplantation. Ninety‐four percent received busulfan and cyclophosphamide‐containing conditioning regimens and bone marrow was the predominant source of donor cells. Most patients achieved haematopoietic recovery and no deaths occurred during the early post‐transplant period. Rates of acute and chronic graft‐versus‐host disease were 10% and 22% respectively. Sixty‐four of 67 patients are alive with 5‐year probabilities of disease‐free and overall survival of 85% and 97% respectively. Nine patients had graft failure with recovery of sickle erythropoiesis, eight of who had recurrent sickle‐related events. This report confirms and extends earlier reports that HCT from HLA‐matched related donors offers a very high survival rate, with few transplant‐related complications and the elimination of sickle‐related complications in the majority of patients who undergo this therapy.

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“…5 SCIs in children with SCA are associated with increased risk of future overt strokes and new or progressive SCIs. 6,7 In addition, children with SCA and SCI have been found to have poorer cognitive function than children with SCA with normal MRI of the brain [8][9][10] or sibling controls. 10,11 Clinical and laboratory risk factors for SCI have been evaluated only sparingly.…”

Section: Introductionmentioning

confidence: 99%

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

DeBaun

Sarnaik

Rodeghier³

et al. 2012

Blood

190

144

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The most common form of neurologic injury in sickle cell anemia (SCA) is silent cerebral infarction (SCI). In the Silent Cerebral Infarct Multi-Center Clinical Trial, we sought to identify risk factors associated with SCI. In this cross-sectional study, we evaluated the clinical history and baseline laboratory values and performed magnetic resonance imaging of the brain in participants with SCA (HbSS or HbS␤°thalassemia) between the ages of 5 and 15 years with no history of overt stroke or seizures. Neuroradiology and neurology committees adjudicated the presence of SCI. SCIs were diagnosed in 30.8% (251 of 814) participants who completed all evaluations and had valid data on all prespecified demographic and clinical covariates. The mean age of the participants was 9.1 years, with 413 males (50.7%). In a multivariable logistic regression analysis, lower baseline hemoglobin concentration (P < .001), higher baseline systolic blood pressure (P ‫؍‬ .018), and male sex (P ‫؍‬ .030) were statistically significantly associated with an increased risk of an SCI. Hemoglobin concentration and systolic blood pressure are risk factors for SCI in children with SCA and may be therapeutic targets for decreasing the risk of SCI. This study is registered at www.clinicaltrials.gov as #NCT00072761. IntroductionSilent cerebral infarcts (SCIs) have been recognized by neuroimaging in neurologically normal older adult populations since 1981 1 and were documented in sickle cell anemia (SCA) soon afterward. 2 As with overt stroke, SCIs represent a clinical finding that is common in older adults without SCD, but they appear during early childhood in persons with SCA. SCIs are defined as an MRI signal abnormality visible on 2 views on the T2-weighted images (axial and coronal) that must measure at least 3 mm in one dimension; further, the person deemed to have an SCI must have an absence of focal neurologic deficit compatible with the anatomic location of the brain lesion. 3 SCI is the most common form of neurologic injury among children with SCA, occurring in at least 27% before 6 years of life 4 and 37% by 14 years of life. 5 SCIs in children with SCA are associated with increased risk of future overt strokes and new or progressive SCIs. 6,7 In addition, children with SCA and SCI have been found to have poorer cognitive function The publication costs of this article were defrayed in part by page charge payment. Therefore, and solely to indicate this fact, this article is hereby marked ''advertisement'' in accordance with 18 USC section 1734. 3684BLOOD, 19 APRIL 2012 ⅐ VOLUME 119, NUMBER 16For personal use only. on March 28, 2019. by guest www.bloodjournal.org From than children with SCA with normal MRI of the brain 8-10 or sibling controls. 10,11 Clinical and laboratory risk factors for SCI have been evaluated only sparingly. In the most rigorous study to date, the investigators from the Cooperative Study for Sickle Cell Disease (CSSCD) described risk factors associated with SCI in 42 participants, comparing them with 188 controls with ...

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Cognitive screening examinations for silent cerebral infarcts in sickle cell disease

Abstract: Brief cognitive screening measures, if properly constructed, may be an effective means of identifying children with silent cerebral infarct. Future prospective studies should be pursued to assess the utility of cognitive screening for silent cerebral infarcts in SCD.

Cited by 135 publications

References 0 publications

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

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

Matched‐related donor transplantation for sickle cell disease: report from the Center for International Blood and Transplant Research

Associated risk factors for silent cerebral infarcts in sickle cell anemia: low baseline hemoglobin, sex, and relative high systolic blood pressure

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