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

Abstract

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.