SUMMARYObjective: To determine the clinical characteristics, surgical challenges, and outcome in children younger than 3 years of age undergoing epilepsy surgery in Canada. Methods: Retrospective data on patients younger than age 3 years who underwent epilepsy surgery at multiple centers across Canada from January 1987 to September 2005 were collected and analyzed. Results: There were 116 patients from eight centers. Seizure onset was in the first year of life in 82% %, and mean age at first surgery was 15.8 months (1-35 months). Second surgeries were done in 27 patients, and a third surgery in 6. Etiologies were malformations of cortical development (57), tumor (22), Sturge-Weber syndrome (19), infarct (8), and other (10). Surgeries comprised 40 hemispheric operations, 33 cortical resections, 35 lesionectomies, 7 temporal lobectomies, and one callosotomy. There was one surgical mortality. The most common surgical complications (151 operations in 116 patients) were infection (17) and aseptic meningitis in 13. Of 107 patients with seizure outcome assessed more than one year postoperatively, 72 (67.3%) were seizure free (Engel I), 15(14%) had >90% improvement (Engel II), 12 had >50% improvement (Engel III), and 8 did not benefit from surgery (Engel IV). Development improved in 55.3% after surgery. Conclusion: Epilepsy surgery in children younger than 3 years of age is relatively safe and is effective in controlling seizures. Very young age is not a contraindication to surgery in children with refractory epilepsy, and early surgery may impact development positively.
Some potentially preventable complications have been addressed with a resulting rate of complications lower than other published reports. We use two standard adjuncts: the presence of a senior surgeon and strict antimicrobial regimens. We believe that our results could be transferrable to other units by following similar guidelines.
Rare disease Background:SMART (Stroke-like Migraine Attacks after Radiation Therapy) syndrome is an uncommon delayed complication of cerebral radiotherapy. Less than 50 cases have been reported in the literature since it was first described in 1995. On average, presentation is about 20 years after radiotherapy, and patients commonly present with headaches, complex seizures, and stroke-like symptoms. The exact pathophysiology of the disease remains poorly understood, but one theory suggests radiation-induced vascular dysfunction.
Case Report:We present one such case of a 28-year-old man who presented to our Emergency Department with a gradually progressive severe headache and right-sided weakness developing over a few hours. MRI played a central role in the diagnosis of SMART syndrome, with serial studies demonstrating and supporting the theory of vascular dysfunction. The condition is usually self-limiting, and most patients achieve complete recovery of symptoms, as did ours. Its optimal management remains unclear.
Conclusions:Better understanding of the imaging findings in SMART syndrome may help differentiate it from tumor recurrence, cerebral infections, or vasculitis. Because the diagnosis of this condition portends a significantly better prognosis and substantially alters patient expectation and management, it is important that clinicians are aware of the usual delayed presentation, symptomology, and imaging findings.
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