Specchio Nicola, Balestri Martina, Trivisano Marina, Japaridze Natia, Striano Pasquale, Carotenuto Antonio, Cappelletti Simona, Specchio Luigi M, Fusco Lucia, Vigevano Federico
Division of Neurology, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.
J Child Neurol. 2012 Apr;27(4):439-44. doi: 10.1177/0883073811419262. Epub 2011 Oct 21.
The aim of the study was to evaluate interictal electroencephalogram features in 22 patients with Dravet syndrome from the onset of the disease through the next 5 years. Electroencephalogram was abnormal in 5 patients (22.7%) at onset, and in 17 (77.3%) at the end of the study. Epileptiform abnormalities (focal, multifocal, or generalized) were seen in 6 patients at the onset and in 14 (27% vs 64%) at the end of the study. Photoparoxysmal response was present in 41% of patients at the end of follow-up. No statistical differences were found between mutated and nonmutated groups regarding evolution of background activity, interictal abnormalities, and presence of photoparoxysmal response. Electroencephalogram findings seemed to be age dependent, variable among different patients, and not influenced by the presence of sodium channel, voltage-gated, type I, alpha subunit (SCN1A) mutation. The lack of specific epileptiform abnormalities contributes to the difficulty of patients' management in Dravet syndrome.
该研究的目的是评估22例德雷维特综合征患者从疾病发作开始至接下来5年期间的发作间期脑电图特征。5例患者(22.7%)在疾病发作时脑电图异常,17例(77.3%)在研究结束时脑电图异常。6例患者在疾病发作时出现癫痫样异常(局灶性、多灶性或全身性),14例(27%对64%)在研究结束时出现癫痫样异常。随访结束时,41%的患者存在光阵发性反应。在背景活动演变、发作间期异常和光阵发性反应的存在方面,突变组和非突变组之间未发现统计学差异。脑电图结果似乎与年龄有关,不同患者之间存在差异,且不受I型电压门控钠通道α亚基(SCN1A)突变的影响。缺乏特异性癫痫样异常导致德雷维特综合征患者的管理困难。