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 more commonly associated with neurologic complications. These are attributed to the vaso-occlusion and micro-obstruction in the circulation of the central nervous system. The incidence of various neurologic complications in SCD ranges from 6% to 30% in various series. The commonly reported in literature include silent cerebral infarction (SCI), ischemic stroke, transient ischemic attacks (TIAs), headaches, seizures and neurocognitive impairment. However, hemorrhagic complications like subarachnoid hemorrhage (SAH), hemorrhagic stroke, extradural and subdural hematomas, especially in absence of trauma are rarely thought of. We report three uncommon spontaneous hemorrhagic manifestations of sickle cell anemia – one case of parenchymal (intracerebral) bleed who presented with acute onset of parkinsonism and two cases of extradural hematoma (EDH) of which one patient had recurrent EDH at the same site which is hitherto not reported in the literature.

Keywords

• anemia
• hematoma
• neurological
• sickling
• stroke