Skip to main content
Journal cover image

Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia.

Publication ,  Journal Article
Vichinsky, EP; Neumayr, LD; Gold, JI; Weiner, MW; Rule, RR; Truran, D; Kasten, J; Eggleston, B; Kesler, K; McMahon, L; Orringer, EP; Kutlar, A ...
Published in: JAMA
May 12, 2010

CONTEXT: Sickle cell anemia (SCA) is a chronic illness causing progressive deterioration in quality of life. Brain dysfunction may be the most important and least studied problem affecting individuals with this disease. OBJECTIVE: To measure neurocognitive dysfunction in neurologically asymptomatic adults with SCA vs healthy control individuals. DESIGN, SETTING, AND PARTICIPANTS: Cross-sectional study comparing neuropsychological function and neuroimaging findings in neurologically asymptomatic adults with SCA and controls from 12 SCA centers, conducted between December 2004 and May 2008. Participants were patients with SCA (hemoglobin [Hb] SS and hemoglobin level < or = 10 mg/dL) aged 19 to 55 years and of African descent (n = 149) or community controls (Hb AA and normal hemoglobin level) (n = 47). Participants were stratified on age, sex, and education. MAIN OUTCOME MEASURES: The primary outcome measure was nonverbal function assessed by the Wechsler Adult Intelligence Scale, third edition (WAIS-III) Performance IQ Index. Secondary exploratory outcomes included performance on neurocognitive tests of executive function, memory, attention, and language and magnetic resonance imaging measurement of total intracranial and hippocampal volume, cortical gray and white matter, and lacunae. RESULTS: The mean WAIS-III Performance IQ score of patients with SCA was significantly lower than that of controls (adjusted mean, 86.69 for patients with SCA vs 95.19 for controls [mean difference, -5.50; 95% confidence interval {CI}, -9.55 to -1.44]; P = .008), with 33% performing more than 1 SD (<85) below the population mean. Among secondary measures, differences were observed in adjusted mean values for global cognitive function (full-scale IQ) (90.47 for patients with SCA vs 95.66 for controls [mean difference, -5.19; 95% CI, -9.24 to -1.13]; P = .01), working memory (90.75 vs 95.25 [mean difference, -4.50; 95% CI, -8.55 to -0.45]; P = .03), processing speed (86.50 vs 97.95 [mean difference, -11.46; 95% CI, -15.51 to -7.40]; P < .001), and measures of executive function. Anemia was associated with poorer neurocognitive function in older patients. No differences in total gray matter or hippocampal volume were observed. Lacunae were more frequent in patients with SCA but not independently related to neurocognitive function. CONCLUSION: Compared with healthy controls, adults with SCA had poorer cognitive performance, which was associated with anemia and age.

Duke Scholars

Altmetric Attention Stats
Dimensions Citation Stats

Published In

JAMA

DOI

EISSN

1538-3598

Publication Date

May 12, 2010

Volume

303

Issue

18

Start / End Page

1823 / 1831

Location

United States

Related Subject Headings

  • Neuropsychological Tests
  • Middle Aged
  • Male
  • Magnetic Resonance Imaging
  • Intelligence Tests
  • Hypoxia, Brain
  • Humans
  • General & Internal Medicine
  • Female
  • Cross-Sectional Studies
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Vichinsky, E. P., Neumayr, L. D., Gold, J. I., Weiner, M. W., Rule, R. R., Truran, D., … Neuropsychological Dysfunction and Neuroimaging Adult Sickle Cell Anemia Study Group, . (2010). Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA, 303(18), 1823–1831. https://doi.org/10.1001/jama.2010.562
Vichinsky, Elliott P., Lynne D. Neumayr, Jeffrey I. Gold, Michael W. Weiner, Randall R. Rule, Diana Truran, Jeffrey Kasten, et al. “Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia.JAMA 303, no. 18 (May 12, 2010): 1823–31. https://doi.org/10.1001/jama.2010.562.
Vichinsky EP, Neumayr LD, Gold JI, Weiner MW, Rule RR, Truran D, et al. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010 May 12;303(18):1823–31.
Vichinsky, Elliott P., et al. “Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia.JAMA, vol. 303, no. 18, May 2010, pp. 1823–31. Pubmed, doi:10.1001/jama.2010.562.
Vichinsky EP, Neumayr LD, Gold JI, Weiner MW, Rule RR, Truran D, Kasten J, Eggleston B, Kesler K, McMahon L, Orringer EP, Harrington T, Kalinyak K, De Castro LM, Kutlar A, Rutherford CJ, Johnson C, Bessman JD, Jordan LB, Armstrong FD, Neuropsychological Dysfunction and Neuroimaging Adult Sickle Cell Anemia Study Group. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010 May 12;303(18):1823–1831.
Journal cover image

Published In

JAMA

DOI

EISSN

1538-3598

Publication Date

May 12, 2010

Volume

303

Issue

18

Start / End Page

1823 / 1831

Location

United States

Related Subject Headings

  • Neuropsychological Tests
  • Middle Aged
  • Male
  • Magnetic Resonance Imaging
  • Intelligence Tests
  • Hypoxia, Brain
  • Humans
  • General & Internal Medicine
  • Female
  • Cross-Sectional Studies