This is a well-defined syndrome, with mean age of onset of 6 years, in which seizures occur with prominent visual symptomatology, including hemianopia and amaurosis, abstract and complex structured visual hallucinations, eye deviation and prominent postictal headaches with nausea and vomiting. There may be secondary generalization. The EEG shows prominent occipital epileptiform spike-wave activity which appears after eye closure and is suppressed by eye opening. The condition has an excellent prognosis with full remission in most cases. The condition needs to be differentiated from migraine and also from symptomatic occipital epilepsies, which include those due to mitochon-drial disease (Alpers disease, MELAS), coeliac disease or cortical dysplasia. Seizures show a complete response to carbamaze-pine in over 90% of cases, and the prognosis is excellent. However, some patients require long-term treatment.
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