Neuropsychological Dysfunction and Neuroimaging Abnormalities in Neurologically Intact Adults With Sickle Cell Anemia

Vichinsky, Elliott; Neumayr, Lynne; Gold, Jeffrey I.; Weiner, Michael W.; Rule, Randall R.; Truran, Diana; Kasten, Jeffrey; Eggleston, Barry; Kesler, Karen; McMahon, Lillian; Orringer, Eugene P.; Harrington, Thomas J.; Kalinyak, Karen; Castro, Laura M. De; Kutlar, Abdullah; Rutherford, Cynthia J.; Johnson, Cage S.; Bessman, J. David; Jordan, Lanetta; Armstrong, F. Daniel

doi:10.1001/jama.2010.562

Cited by 258 publications

(310 citation statements)

References 34 publications

Supporting

Mentioning

272

Contrasting

Unclassified

Order By: Relevance

“…Hypoxia and chronic anemia are additional risk factors for ischemic brain injury and increase the likelihood of neurocognitive impairment with age [61,105]. In a large multicenter study assessing neurocognitive function in neurologically intact adults with SCA, Vichinsky et al found that adults with SCA score significantly lower on the Wechsler Adult Intelligence Scale III than community controls [106]. While there was a decrease in intracranial volume and more lacunar infarcts in adults with SCA, this did not explain the difference in findings.…”

Section: Pulmonary Complicationsmentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

View full text Add to dashboard Cite

Over the past 40 years, public health measures such as universal newborn screening, penicillin prophylaxis, vaccinations, and hydroxyurea therapy have led to an impressive decline in sickle cell disease (SCD)-related childhood mortality and SCD-related morbidity in high-income countries. We remain cautiously optimistic that the next 40 years will be focused on meeting current challenges in SCD by addressing chronic complications of SCD to reduce mortality and improve quality of life in a growing population of adults with SCD in high-income countries, while simultaneously decreasing the disparity of medical care between high and low-income countries.

show abstract

Section: Pulmonary Complicationsmentioning

confidence: 99%

Evolution of sickle cell disease from a life‐threatening disease of children to a chronic disease of adults: The last 40 years

2015

View full text Add to dashboard Cite

show abstract

“…It is possible that the mean FSIQ of 80, which was lower than typically reported for SCD patients, was influenced by inclusion of the stroke patients, who often have scores in the 70s or lower. On the other hand, an alleviating effect might have resulted from chronic transfusion in six patients, since the severity of anemia has an important negative influence on neuropsychometric performance [9]. Thirdly, as the authors acknowledge, the lack of MRI data prevented assessment of the role of silent cerebral infarcts on neuropsychological performance.…”

mentioning

confidence: 94%

Sickle‐cell disease and compromised cognition

Wang

2011

Pediatric Blood & Cancer

View full text Add to dashboard Cite

(Commentary on Hijmans et al., page 783) T he article by Hijmans et al. [1] in the current issue of Pediatric Blood and Cancer, notes that the ''most devastating complication of sickle-cell disease (SCD) is cerebral vascular infarction.'' Although stroke in SCD has been recognized for 80 years, the past three decades have seen an increasing number of reports describing the neuropsychological performance of children with SCD [2][3][4]. Over the same period, an improved, but still incomplete, understanding of the pathophysiology of SCD in the brain has been gained [5]. The association of stenosis or occlusion of large intracranial arteries and stroke was first demonstrated by cerebral angiography. Subsequently, transcranial Doppler ultrasonography (TCD) screening has allowed identification of those with abnormally elevated cerebral artery flow velocities who are at very high risk for stroke and the dramatic reduction in the incidence of stroke through transfusion management [6]. Both overt strokes and cerebral silent infarcts on MRI of the brain are associated with cognitive deficits and diminished school performance [7]. Abnormalities in cerebral blood flow may be a common pathophysiologic factor for CNS injury [8], but our understanding of the incidence, pathophysiology, prognosis, and appropriate treatment of diminished neurocognitive performance remains limited.In the Hijmans study, 41 subjects, ages 6-18 years, with HbSS or HbSb 0 -thalassemia were evaluated with a well-defined battery of tests to measure general intelligence (full-scale IQ), executive function, and visuo-motor function. Subjects were seen at a center in the Netherlands and were mostly low socio-economic status (SES) immigrants from western African and Surinam. Importantly, the investigators evaluated an ethnicly and SES-matched control group of healthy siblings of the tested cohort or of other patients with SCD who were receiving care at the same hospital. Among the subjects with SCD were 3 who had had an overt stroke and 6 who were receiving chronic transfusion. Major findings were that SCD was associated with markedly lower IQ scores (one-third had FSIQ <75), diminished visuo-motor function, and executive dysfunction, including deficits in sustained attention, planning, and visuo-spatial working memory.The study had several important strengths. First, SCD subjects were compared to a control group, unlike many investigations which have lacked appropriate controls. Secondly, the subjects represented a different and perhaps more globally heterogeneous group than the African-American population which typically has been examined. Thirdly, the neuropsychometric instruments utilized in the study were well defined and specifically targeted at executive and visuo-motor function, domains known to be particularly compromised in SCD [2,3].The study also had significant limitations. First, it was unclear how the three patients who had experienced overt strokes influenced the overall results, since individual patient data was not included. It is poss...

show abstract

“…Brain damage can be present in SCD patients without any clinical evidence of stroke [8]. However, silent cerebral infarction, defined as an ischemic change in brain tissue in the absence of any clinical history of stroke, is observed by magnetic resonance imaging (MRI) in approximately 17% of the patients with SCA [9,10]. Furthermore, children with haemoglobin SS (HbSS) and normal MRI findings have been noted to have cognitive deficits and a decline in academic performances [11].…”

Section: Introductionmentioning

confidence: 99%

“…The factors that are highlighted as possible causes of depression and anxiety in patients living with sickle cell disease include the chronicity of their health challenges; unpredictable nature of sickle cell crises; frequent recurrent painful episodes; repeated medical problems such as anemia, leg ulcers, kidney failure, central nervous HATASO rabm.scholasticahq.com system complications, poor growth, physical structural abnormalities, and reduced life expectancy; and social prejudice and stereotyping [4]. The Pain in Sickle Cell Epidemology Study (PiSCES) project observed that 27.6% of the patients with SCD had depression while 6.5% had anxiety disorder, and these subjects had poorer functioning on all aspects of health-related quality of life [10]. Other studies provided a prevalence rate of depression ranging from 18% to 44% [20][21][22].…”

Section: Introductionmentioning

confidence: 99%