Introduction: Between 6% and 17% of the patients with drepanocytosis will have an acute cerebrovascular accident (ACVA). Precipitating factors have been described including bacterial meningitis, upper respiratory tract obstruction, dehydration, intense exercise, postoperatively, and hypoxia due to altitude.
Clinical cases: We report two Negro children with drepanocytosis who, at the ages of 8 and 20 months, had pneumococcal meningitis complicated by an ACVA. The 20 month old boy had been previously diagnosed as having drepanocytosis; the 8 month old girl was diagnosed when she was admitted to hospital with meningitis. In both cases the clinical features of the ACVA were focal epileptic seizures followed by hemiplegia. On cranial CT and MR regions of focal ischaemia of the hemisphere were observed. The boy of 20 months made an excellent recovery with no long term sequelae. The 8 month old girl had severe permanent sequelae: hemiparesia, blindness, mental deficiency and epileptic encephalopathy.
Conclusions: We present two Negro children with drepanocytosis who had ischemic ACVAs, a common complication of the disease, during the course of pneumococcal meningitis but with very different clinical courses. Recent increases in immigration will mean that previously rare illnesses will be more commonly seen in our environment, and should be considered in the differential diagnosis.