ABSTRACT
Acute neurological complications are common in sickle cell disease (SCD) and include, in addition to central nervous system infections, ischaemic stroke, typically presenting as hemiplegia with or without seizures, haemorrhagic stroke presenting as sudden onset of severe headache and reduced level of consciousness, and posterior reversible encephalopathy syndrome presenting as seizures. Emergency management of seizures according to local protocol and partial exchange transfusion is the mainstays of acute management, while hydroxyurea reduces the risk of recurrence if chronic transfusion is not feasible. Abnormal (>200 cm/sec) time-averaged mean of the maximum velocity in the middle cerebral artery on imaging or non-imaging transcranial Doppler predicts a high risk of ischaemic stroke which may be reduced by hydroxyurea at a dose of at least 10 mg/kg. Cognitive difficulties include reduced processing speed and executive dysfunction.
