Stroke in a cohort of patients with homozygous sickle cell disease

Strokes occurred in 17 of 310 children with homozygous sickle cell disease who were followed from birth, representing an incidence of 7.8 percent by the age of 14 years. Two children had subarachnoid hemorrhage, one having resolution of symptoms after aneurysm surgery and another dying of a presumed second hemorrhage 14 days later. The remaining 15 strokes were presumed to be cerebral infarction, although autopsy, angiographic, or computed tomographic evidence was available in only 8 children. There were 6 deaths, 2 in the acute events and 4 after recurrence, which occurred in 6 (46 percent) of 13 patients who survived the initial episode. There were 10 recurrent episodes at a median interval of 9 months after the initial event. Steady-state hematologic data revealed significantly higher leukocyte counts than in control subjects without strokes at age 1 year and in the last study preceding the stroke. The initial stroke coincided with an acutely lowered hemoglobin value in 5 patients (3 aplastic crises, 1 acute splenic sequestration, 1 probable pulmonary sequestration) and with painful crises in another 7 patients. We conclude that a high leukocyte count and an acute decrease of hemoglobin are risk factors for stroke in patients with homozygous sickle cell disease.(AU)

Cerebro-vascular complications of sickle cell disease in Jamaica - abstract

Homozygous sickle cell disease is an uncommon but important cause of hemiparesis and subarachnoid haemorrhage. A 24-year review showed that 42 cases of hemiparesis and 9 cases of subarachnoid haemorrhage had been seen at the University Hospital of the West Indies. All living patients (32) were examined in 1984 for residual defects. Twenty-two males and 20 females, with a median age of 7 years (range 8 months to 36 years) had had a stroke. Most (69 percent) were under 10 years of age at the time of the first stroke. No haematological or clinical predictor for stroke was identified, although transient ischaemic episodes prior to the onset of a complete stroke were seen in one 13-year-old. Recurrence clustered within the first 30 months with 10 of the 17 cases occurring in the first year. Contralateral occurrences were commonest (14) and resulted in death in 13. Subarachnoid haemorrhage was seen in 5 males and 4 females, aged 6 to 57 years (median 19 years), and 3 patients died. At re-evaluation of 32 patients, 8 had complete recovery, 20 residual hemiplegia and 4 quadriplegia. It is concluded that cerebro-vascular accident, though uncommon in sickle cell disease, affects a young age group and is associated with devastating consequences (AU)