Cerebrovascular complications of sickle cell anemia.

IN THE PAST several years a resurgence of interest in sickle cell anemia has led to new information about its effects on the central nervous system. The literature has several extensive reviews of the various types of neurological complications observed in patients with this disorder. However, it remains unclear whether many of the complications mentioned are directly attributable to sickle cell disease or are incidentally associated. The majority of investigators, however, agree that the cerebral vascular complications are a direct result of pathophysiological abnormalities produced by the abnormal hemoglobin. The presence of S-hemoglobin appears to be a definite predisposing factor to the development of cerebral vascular disease, particularly in children. This is illustrated in a study by Solomon et al., whose investigations of infantile hemiplegia revealed that 7% were attributable to or associated with sickle cell disease.