Auditory event-related potentials and reaction times during simple sensorymotor tasks in subjects with sickle cell disease and related hemoglobinopathies.

40 subjects of both sexes assigned to five groups of 8: healthy students, homozygous sicklemics, hemoglobin SC sicklemics, sickle cell Bo thalassemics, and sickle cell B+ thalassemics were subjected to 1/simple auditory stimuli; 2/paired auditory stimuli. The subjects were asked to respond quickly, by pressing a push button on detection of the simple stimulus or the unconditional stimulus of paired stimuli. A subsequent increase in reaction times and a reduction in event-related potential component amplitudes were obvious in major (SS and SC) sicklemics compared to controls and sickle cell B thalassemics. Between simple and paired auditory conditions, reaction times were more increased in sicklemics than in controls. These findings suggest that impairment of attentiveness, performance and cognitive capability in sicklemics is a function of sickle cell anemia severity.