Abstracts

Rationale: The prevalence of seizures in Sickle Cell Disease (SCD) is 10 times that of the general population. SCD is also the most common cause of childhood stroke. Seizures in SCD are thought to be secondary to the intracranial vasculopathy and ischemia that result in strokes. However, few studies have explored the apparent correlation between stroke and seizure occurrence in SCD patients. Our study aims to study the spectrum of seizures associated with stroke in sickle cell disease in children.Methods: An initial word search was performed using the electronic medical record at Children’s Hospital of Alabama from January 1st, 2010 to May 30th, 2015. Search terms included “stroke”, “infarct”, “porencephaly”, and “encephalomalacia.” The identified patient charts were then retrospectively reviewed for clinical history, diagnoses, and neuro-radiologic studies.Results: At our tertiary care hospital, 190 children were diagnosed with stroke, 50 of whom had SCD (26 %). Forty (80%) patients had cortical stroke and ten (20%) had subcortical stroke. The mean age at stroke occurrence was 8.36±4.86 years (range: perinatal – 18 yrs). Of the 50 children with stroke and SCD, 6 (12%) had seizures. One additional patient had initial seizures around 5 months of age and subsequently had a stroke and seizure recurrence nearly 13 years later. This patient was excluded from our analysis. The mean age at seizure occurrence was 8.1±5.6 years (range: 7 mo-15 yrs). Four patients (66%) were male. All 6 patients with seizures had cortical stroke. Three patients had isolated seizures at stroke diagnosis. The other 3 had recurrent seizures with seizure onset 4-7 years after the cortical stroke. All six patients had focal seizures. Five patients were treated with antiepileptic medications.Conclusions: 1. Our retrospective study shows: Both seizures and epilepsy are infrequent in those with SCD and stroke. Despite evidence for large and small vessel disease, as well as cerebral insufficiency in our cohort, seizures were infrequently seen. This raises two possibilities: a. Vasculopathy & ischemia may not be the main underlying mechanism for seizures in SCD. b. Underlying genetic or environmental factors may play a “neuroprotective” role. 2. Recurrent seizures may occur 4-7 years after cortical stroke, in the absence of acute seizures at stroke occurrence. 3. The pathophysiology of SCD associated seizures in patients with strokes warrants further study. This may shed further light on epileptogenesis.