CLINICAL REVIEW Neurological Manifestations in Sickle-Cell Disease With a Review of the Literature and Emphasis on the Prevalence of Hemiplegia

A high prevalence of hemiplegia was found in 89 patients with sickle-cell disease seen over a 5-year period. Twenty-three patients (26%) had neurological manifestations; hemiplegia occurred in 15 (17%). During the same period, hemiplegia occurred in only 5 % of patients with sickle-hemoglobin C disease and in 1.7 % of patients with sickle-cell trait; the latter is the same as that in the negro population with normal hemoglobin (1.8%). A review of the English-language literature shows that the high frequency of hemiplegia in the natural history of sickle-cell disease has not been sufficiently emphasized, despite its previous documentation in isolated case reports. Unless better methods for controlling the clinical expressions of this hemoglobinopathy can be found, stroke will remain one of the commonest serious complications in patients with sickle-cell disease. DESPITE comprehensive reviews of sickle-cell disease (1, 2) and numerous case reports (3-65) with cen-tral nervous system abnormalities, there have been few reports emphasizing the severity and frequency of neurological complications in this disorder. Our paper describes the high prevalence of serious neuro-logical findings, especially hemiplagia, found in a study of a group of 89 patients with sickle-cell dis-ease and reviews the relevant literature to demon-strate the frequency of cerebrovascular accident in the natural history of the disease. Methods The medical records of patients with recognized sickle-cell hemoglobinopathies seen at the North Car-olina Memorial Hospital (NCMH), Chapel Hill, be