NEUROBEHAVIORAL FEATURES OF SICKLE-CELL DISEASE

This investigation was designed to evaluate cognitive and behavioral s tatus in an adult population of sickle cell anemia patients with and w ithout structural CNS lesions demonstrated by neuroimaging techniques. This work was intended as a pilot investigation; therefore, not all p atients underwent full testing or neuroimaging procedures. Three of th e patients had no neurodiagnostic procedures, while eight had either a n 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 ra nging in age from 21 to 36. The set of neurobehavioral tasks presented measured attention and orientation, motor function, gross verbal skil l, perceptual and constructional ability, memory, conceptual function, and intellectual functioning. All subjects scored in the impaired ran ge of at least 1 of the 10 tasks. The findings show that visual-spatia l and visual-motor tasks seem to be overwhelmingly impaired in this ad ult 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 d emonstrated by MRI, CT, or EEG. Considering the idea that neuropsychol ogical 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 standa rdized and comprehensive neuropsychological assessment is a valuable t ool for the clinician working with this population.