Neurological management of ischemic stroke in sickle cell disease- a case report with an updated review of the literature.

Background: In children and adults with sickle-cell disease (SCD), acute ischemic stroke (AIS) associated with a vaso-occlusive crisis is a leading cause of physical and cognitive disability and death. However, neurological guidelines for acute management of AIS fail to directly address this issue. We here report a case of a man with severe cerebrovascular complications and illustrate the current evidence on the management of SCD-related AIS.

Case Report: A 46-year-old man suffering from SCD and poorly controlled diabetes was admitted to the emergency room complaining of the onset, more than 10 h before, of headache, paresthesia, and right hemianopia. The brain Angio-CT study revealed bilateral occlusion of the internal carotids and the posterior cerebral arteries, with remarkable compensatory hypertrophy of other vessels. The laboratory exams confirmed severe anemia with high hemoglobin-S levels (50%) and severe dehydration. IV hydration was prompted, along with erythrocyte apheresis and antiplatelet therapy. The patient successfully underwent these treatments, and the neurological deficits significantly improved. Nonetheless, due to the time window, he could not be treated with intravenous thrombolysis (IVT).

Discussion: AIS is a time-sensitive condition. In SCD, vaso-occlusive phenomena are the leading cause of AIS, but "classical" vascular risk factors can also play a role. Since current guidelines on the acute management of SCD complications are mainly focused on the hematological- rather than neurological- aspects, it is not clear whether these patients should undergo IVT before or after fluid resuscitation and erythrocyte apheresis. Furthermore, the principles of secondary prophylaxis are still controversial and require further investigation.