Değerliyurt Aydan, Teber Serap, Bektaş Omer, Senkon Gözde
Department of Pediatric Neurology, Ankara Pediatrics, Hematology-Oncology Training and Research Hospital, Ankara, Turkey.
Department of Pediatric Neurology, Ankara Pediatrics, Hematology-Oncology Training and Research Hospital, Ankara, Turkey.
Epilepsy Behav. 2014 Jul;36:24-32. doi: 10.1016/j.yebeh.2014.04.018. Epub 2014 May 20.
The aim of the study was to evaluate the demographic, clinical, and EEG characteristics of patients with Panayiotopoulos syndrome (PS) and the course of their illness. Thirty-eight patients followed up with a diagnosis of PS between January 2011 and December 2013 were evaluated. We found high rates of personal history of febrile convulsions, breath-holding spells, and family history of febrile convulsions, afebrile convulsion/epilepsy, migraine, and breath-holding spells. Seizures started before the age of eight in 87% of the patients, and the mean age at seizure onset was 4.6 years. Seizures were sleep-related in 81.5%, and autonomic status was seen in a third of the patients. The number of seizures was between 2 and 10 in 66% of the patients. The most common symptoms were ictus emeticus, eye/head deviation, and altered consciousness. Rolandic features were seen in 26% of the patients, and visual symptoms in 5%. Multifocal epileptiform discharges on EEG were identified in 84% of the patients. Two or more antiepileptic drugs were required in only 13% of the patients. Evolution to electrical status epilepticus in sleep and Gastaut-type epilepsy were seen in patients with more than ten seizures. The high rates of febrile convulsions, afebrile convulsions/epilepsy, migraine, and breath-holding spells in the patients and families suggest the importance of genetic factors and, perhaps, a common pathogenesis. However, the high rates of febrile convulsions and breath-holding spells in patients can be related to a misdiagnosis because of the similar symptoms. Despite its disturbing symptoms, PS is a benign epileptic syndrome requiring multiple antiepileptic drug use only in a small proportion of patients.
本研究旨在评估潘纳约托普洛斯综合征(PS)患者的人口统计学、临床和脑电图特征及其病程。对2011年1月至2013年12月期间确诊为PS并接受随访的38例患者进行了评估。我们发现,患者热性惊厥、屏气发作的个人史以及热性惊厥、无热惊厥/癫痫、偏头痛和屏气发作的家族史发生率很高。87%的患者癫痫发作始于8岁之前,癫痫发作的平均年龄为4.6岁。81.5%的癫痫发作与睡眠相关,三分之一的患者出现自主神经症状。66%的患者癫痫发作次数在2至10次之间。最常见的症状是呕吐性发作、眼/头偏斜和意识改变。26%的患者有罗兰多区特征,5%的患者有视觉症状。84%的患者脑电图显示多灶性癫痫样放电。仅13%的患者需要两种或更多种抗癫痫药物。癫痫发作超过十次的患者出现了睡眠中癫痫性电持续状态和加斯东型癫痫的演变。患者及其家族中热性惊厥、无热惊厥/癫痫、偏头痛和屏气发作的高发生率表明遗传因素的重要性,也许还有共同的发病机制。然而,由于症状相似,患者中热性惊厥和屏气发作的高发生率可能与误诊有关。尽管PS有令人不安的症状,但它是一种良性癫痫综合征,仅一小部分患者需要使用多种抗癫痫药物。