Abstracts

Rationale: Of the 10% of epilepsy patients with photosensitivity, a small subset self-induce seizures by seeking out photic triggers. Sunflower Syndrome describes reflex seizures – typically characterized by eyelid myoclonia – triggered by waving abducted fingers in front of the eyes while staring at the sun (Ames & Saffer, 1983). There is debate regarding whether Sunflower Syndrome is a distinct clinical entity (Striano et al., 2009). We aimed to describe the clinical course, electrographic features, and treatment response in patients with Sunflower Syndrome at our institution. Methods: We searched the electronic medical record and electroencephalogram (EEG) database of Lucile Packard Children’s Hospital using the following terms: Sunflower syndrome, fixation off epilepsy/seizures, eyelid myoclonia with absences, Jeavon's Syndrome. Epileptologists were also surveyed. Sixty-eight charts were individually reviewed. Inclusion criteria included seizures triggered by self-induced photic stimulation. Results: Baseline Variables: Seven patients (5 female, 2 male) met criteria for Sunflower Syndrome. Mean age of seizure onset was 5.5 +/- 2.2 years. Average length of follow-up was 5.6 +/- 4.6 years. Three patients had normal school performance, 3 had significant attention or processing issues, and 1 had intellectual disability (ID). Four patients underwent magnetic resonance imaging (MRI), 3 of which were normal and 1 of which showed low-lying cerebellar tonsils. Only the patient with ID underwent genetic testing, which was unremarkable. Semiology: All patients had eyelid myoclonia. Five had altered consciousness and 2 had preserved consciousness during eyelid myoclonia; 2 also had extremitiy myoclonus. Two patients had rare generalized convulsions with hand-waving. EEG: EEG was available for only 6 patients and universally demonstrated generalized spike and polyspike wave discharges accompanying the eyelid myoclonia, typically of a frontal predominance. Three patients had focal or multifocal spike waves that were likely fragments of generalized discharges. No patient had focal or generalized slowing. All 4 patients undergoing photic stimulation had a photoparoxysmal response. Treatment: Four patients had resolution of seizures with valproate monotherapy, though 2 had persistence of the hand-waving behavior without epileptiform discharges. One patient had seizure resolution on combination topiramate and levetiracetam therapy. The final patient had marked improvement of generalized seizures though persistent eyelid myoclonia on combination clobazam and valproate. One subject was lost to follow-up. Seizures were frequently refractory to medications: 4 patients failed courses of levetiracetam, 3 failed lamotrigine, 2 failed ethosuximide, 1 failed zonisamide, 1 failed clobazam, and 1 failed clonazepam. Conclusions: Patients with Sunflower Syndrome have generalized discharges on EEG. While patients failed multiple medicaitons, 70% achieved significant seizure control with valproate therapy, which should be considered early in treatment. Funding: F. Baumer is supported by a KL2 Mentored Career Development Award of the Stanford Clinical and Translational Science Award to Spectrum (NIH KL2 TR 001083) and (UL1 TR 001085).