Neurobehavioral Features of Sickle Cell Disease

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Summary:This investigation was designed to evaluate cognitive and behavioral status in an adult population of sickle cell anemia patients with and without structural CNS lesions demonstrated by neuroimaging techniques. This work was intended as a pilot investigation; therefore, not all patients underwent full testing or neuroimaging procedures. Three of the patients had no neurodiagnostic procedures, while eight had either an MRI study, CT scan, or electroencephalogram (EEG). All participants underwent standardized neuropsychological assessment of cognitive and behavioral functioning. The subjects included 6 females and 5 males ranging in age from 21 to 36. The set of neurobehavioral tasks presented measured attention and orientation, motor function, gross verbal skill, perceptual and constructional ability, memory, conceptual function, and intellectual functioning. All subjects scored in the impaired range of at least 1 of the 10 tasks. The findings show that visual-spatial and visual-motor tasks seem to be overwhelmingly impaired in this adult population, which is consistent with findings in studies of young sickle cell patients. Our results suggest that impaired performance on the neuropsychological measures is independent of the abnormalities demonstrated by MRI, CT, or EEG. Considering the idea that neuropsychological deficits may be overlooked in adult sickle cell patients, or be discounted as representing a (psychiatric) functional disorder rather than the result of an organic process, our findings imply that standardized and comprehensive neuropsychological assessment is a valuable tool for the clinician working with this population.

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