INTRODUCTION: Sickle cell disease (SCD) is the commonest cause of stroke in childhood. Ninety percent of patients have radiological or pathological evidence of large vessel disease. However, patients presenting with neurological symptoms and signs after chest crisis have been reported to have generalised, rather than focal, neuroradiological abnormalities (posterior leukencephalopathy and acute demyelination). As few cases have been reported, the pathophysiology and natural history remain obscure. 

METHODS: As part of a 10-year prospectively collected registry of children with SCD, we report our experience with patients presenting acutely and found to have generalised radiological abnormality.

RESULTS: Of 51 patients documented to have had an acute neurological presentation with focal signs, seizures or coma, 4 (8%) had generalised rather than focal neuroradiological abnormality on imaging within 3 days of presentation. 3 had generalised cerebral oedema, of whom 2 had bilateral borderzone infarction involving grey as well as white matter. These patients survived and reintegrated into mainstream school without significant motor disability; none has had a recurrence after follow-up of 4-10 years. The fourth child had posterior leukencephalopathy radiologically; he recovered consciousness but died of his pre-existing renal disease.

CONCLUSION: Our patients extend the neuroradiology associated with acute seizures and coma in SCD to include generalised cerebral oedema and bilateral borderzone infarction as well as posterior leukencephalopathy. The neurological outcome may be favourable if the patient survives the acute phase. The role of acute hypoxia and blood pressure abnormalities requires investigation.