A case series of hemorrhagic neurological complications of sickle cell disease: Multiple faces of an underestimated problem!

Abstract

Sickle cell disease (SCD) is a group of hemoglobinopathies that vary in severity, the most severe form, homozygous sickle cell anemia, is often associated with neurologic complications. The incidence of various neurologic complications in SCD ranges from 6% to 30% in different series. Although the incidence of acute ischemic stroke and chronic cerebral ischemia is higher in SCD, about 20–30% of the neurological complications may be hemorrhagic in nature. Complications such as spontaneous subarachnoid hemorrhage, hemorrhagic stroke, extradural, and subdural hematomas have been described in the literature. The aim of this article was to report uncommon spontaneous hemorrhagic neurological manifestations of SCD. We have described three uncommon hemorrhagic neurological complications of SCD. One case had a parenchymal (intracerebral) bleed and presented with acute onset of parkinsonism, while two cases had an extradural hematoma (EDH), of which one patient had recurrent EDH at the same site which is hitherto not reported in the literature. Two patients survived, while one with recurrent EDH succumbed. Hemorrhagic neurological complications should be included in the list of differential diagnoses of neurological presentations in patients of SCD. EDH is an uncommon complication of SCD, while recurrent EDH is extremely rare. The condition should be suspected early in patients with SCD, as timely treatment often reduces the mortality.