Baumer Fiona M, Porter Brenda E
Stanford University School of Medicine, Department of Neurology, Division of Child Neurology, United States.
Epilepsy Res. 2018 May;142:58-63. doi: 10.1016/j.eplepsyres.2018.03.002. Epub 2018 Mar 3.
Sunflower Syndrome describes reflex seizures - typically eyelid myoclonia with or without absence seizures - triggered when patients wave their hands in front of the sun. While valproate has been recognized as the best treatment for photosensitive epilepsy, many clinicians now initially treat with newer medications; the efficacy of these medications in Sunflower Syndrome has not been investigated. We reviewed all cases of Sunflower Syndrome seen at our institution over 15 years to describe the clinical course, electroencephalogram (EEG), and treatment response in these patients.
Search of the electronic medical record and EEG database, as well as survey of epilepsy providers at our institution, yielded 13 cases of Sunflower Syndrome between 2002 and 2017. We reviewed the records and EEG tracings.
Patients were mostly young females, with an average age of onset of 5.5 years. Seven had intellectual, attentional or academic problems. Self-induced seizures were predominantly eyelid myoclonia ± absences and 6 subjects also had spontaneous seizures. EEG demonstrated a normal background with 3-4 Hz spike waves ± polyspike waves as well as a photoparoxysmal response. Based on both clinical and EEG response, valproate was the most effective treatment for reducing or eliminating seizures and improving the EEG; 9 patients tried valproate and 66% had significant improvement or resolution of seizures. None of the nine patients on levetiracetam or seven patients on lamotrigine monotherapy achieved seizure control, though three patients had improvement with polypharmacy.
Valproate monotherapy continues to be the most effective treatment for Sunflower Syndrome and should be considered early. For patients who cannot tolerate valproate, higher doses of lamotrigine or polypharmacy should be considered. Levetiracetam monotherapy, even at high doses, is unlikely to be effective.
向日葵综合征描述的是一种反射性癫痫发作——通常为伴有或不伴有失神发作的眼睑肌阵挛——当患者在太阳前挥动双手时引发。虽然丙戊酸盐已被公认为是治疗光敏性癫痫的最佳药物，但现在许多临床医生最初会使用更新的药物进行治疗；这些药物在向日葵综合征中的疗效尚未得到研究。我们回顾了15年来在我们机构中见到的所有向日葵综合征病例，以描述这些患者的临床病程、脑电图（EEG）及治疗反应。
检索电子病历和EEG数据库，并对我们机构的癫痫治疗人员进行调查，在2002年至2017年间共发现13例向日葵综合征病例。我们回顾了这些病例记录和EEG描记图。
患者大多为年轻女性，平均发病年龄为5.5岁。7名患者存在智力、注意力或学习方面的问题。自我诱发的发作主要为眼睑肌阵挛±失神发作，6名患者也有自发性发作。EEG显示背景正常，有3 - 4Hz的棘波±多棘波以及光阵发性反应。基于临床和EEG反应，丙戊酸盐是减少或消除发作及改善EEG最有效的治疗方法；9名患者试用了丙戊酸盐，66%的患者发作有显著改善或得到控制。接受左乙拉西坦治疗的9名患者及接受拉莫三嗪单药治疗的7名患者均未实现发作控制，不过有3名患者联合用药后病情有所改善。
丙戊酸单药治疗仍然是向日葵综合征最有效的治疗方法，应尽早考虑使用。对于不能耐受丙戊酸盐的患者，应考虑使用更高剂量的拉莫三嗪或联合用药。即使是高剂量的左乙拉西坦单药治疗也不太可能有效。
