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Neurology. 2014 Mar 11;82(10):835-41. doi: 10.1212/WNL.0000000000000188. Epub 2014 Feb 12.

Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition.

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From the Department of Psychiatry (R.S.M.), University of California, San Francisco; Center for Imaging of Neurodegenerative Diseases (R.S.M., P.I., D.T., J.B., M.W.W.), San Francisco Veterans Administration Medical Center, San Francisco; Hematology/Oncology Department (E.P.V., L.D.N.), Children's Hospital & Research Center Oakland; Keck School of Medicine (J.I.G.), University of Southern California; Departments of Anesthesiology and Pediatrics (J.I.G.), Children's Hospital Los Angeles, CA; Rho, Inc. (K.K.), Research Triangle Park, NC; Department of Pediatrics (F.D.A.), University of Miami Miller School of Medicine and Holtz Children's Hospital, Miami, FL; and Veterans Administration Medical Center (M.W.W.), and Departments of Radiology, Medicine, Psychiatry, and Neurology, University of California, San Francisco.



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).


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).


After adjusting for age, sex, education level, and intracranial volume, participants with SCA exhibited thinner frontal lobe cortex (t = -2.99, p = 0.003) and reduced basal ganglia and thalamus volumes compared with HCs (t = -3.95, p < 0.001). Reduced volume of the basal ganglia and thalamus was significantly associated with lower Performance IQ (model estimate = 3.75, p = 0.004) as well as lower Perceptual Organization (model estimate = 1.44, p = 0.007) and Working Memory scores (model estimate = 1.37, p = 0.015). Frontal lobe cortex thickness was not significantly associated with any cognitive measures.


Our findings suggest that basal ganglia and thalamus abnormalities may represent a particularly salient contributor to cognitive dysfunction in adults with SCA.

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