Background Seizures may be a feature of SMART (stroke-like migraine attacks after radiation therapy) syndrome but their role in the pathophysiology and clinical presentation is unclear.
Method The clinical and investigation findings in a series of adult patients with SMART syndrome presenting primarily with seizures were reviewed.
Results Four patients with SMART syndrome presenting with seizures were identified (mean age 51 years). Mean time from radiation therapy to SMART syndrome was 22.5 years (range 15-32 years). Indications for radiation were primary brain tumour (three patients) and haematological malignancy (one patient). Two patients had a history of seizures prior to SMART syndrome. Three patients had headaches at presentation. All patients presented with focal impaired awareness seizures with motor features. One patient had refractory non-convulsive status epilepticus requiring intravenous anaesthesia. Three patients had persistent negative motor deficits, associated with ongoing electrographic seizures with no clinical correlate, only identified on repeated EEGs or continuous EEG (cEEG). All patients failed initial anti-seizure medications (ASM), requiring a mean of five ASMs for seizure control. All patients had enhancing cortical MRI changes consistent with SMART syndrome that corresponded to the clinical deficit and ictal changes on EEG. At follow-up all patients improved but had persistent neurological deficits.
Conclusion SMART syndrome presents with seizures and less frequently status epilepticus and may be the basis for the associated clinical features and radiologic abnormalities. Judicious use of EEG and where necessary CEEG to identify non-convulsive seizures should be considered in patients with SMART syndrome presenting with prolonged neurological deficits.
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