2型神经元蜡样脂褐质沉积症患者光阵发性反应的独特特征：脑电图能否作为生物标志物？
Unique Characteristics of the Photoparoxysmal Response in Patients With Neuronal Ceroid Lipofuscinosis Type 2: Can EEG Be a Biomarker?
作者信息
Albert Dara V, Yin Han, De Los Reyes Emily C, Vidaurre Jorge
机构信息
Nationwide Children's Hospital/Ohio State University, Division of Pediatric Neurology, Columbus, OH, USA
Biostatistics Core of Nationwide Children's Hospital, Columbus, OH, USA.
出版信息
J Child Neurol. 2016 Nov;31(13):1475-1482. doi: 10.1177/0883073816658659. Epub 2016 Jul 21.
OBJECTIVE
The objective was to identify unique features of the photoparoxysmal response seen in patients with neuronal ceroid lipofuscinosis type 2 as compared to patients with a photoparoxysmal response associated with other epilepsy syndromes.
METHODS
Electroencephalograms from patients with neuronal ceroid lipofuscinosis type 2 seen at the authors' institution in the past 10 years as well as electroencephalograms (EEGs) reported to have a photoparoxysmal response during a single year were reviewed.
RESULTS
A photoparoxysmal response was seen in 60% of the patients with neuronal ceroid lipofuscinosis type 2. This was most commonly seen with low frequency intermittent photic stimulation (76%) which often occurred in a time-locked fashion (63%) and was seen on the patient's initial EEG (78%). A unique pattern the authors called "sentinel" discharge was identified in 30% of EEGs in patients with neuronal ceroid lipofuscinosis.
CONCLUSIONS
Photoparoxysmal responses in patients with neuronal ceroid lipofuscinosis type 2 have features which are distinguishing from photoparoxysmal responses seen in other epilepsies.
目的
目的是确定2型神经元蜡样脂褐质沉积症患者中所见光阵发性反应的独特特征，并与其他癫痫综合征相关的光阵发性反应患者进行比较。
方法
回顾了作者所在机构在过去10年中诊治的2型神经元蜡样脂褐质沉积症患者的脑电图，以及在某一年报告有光阵发性反应的脑电图。
结果
60%的2型神经元蜡样脂褐质沉积症患者出现光阵发性反应。这最常见于低频间歇性光刺激（76%），其常呈时间锁定方式出现（63%），且在患者首次脑电图检查时即可见到（78%）。在30%的神经元蜡样脂褐质沉积症患者的脑电图中发现了一种作者称为“前哨”放电的独特模式。
结论
2型神经元蜡样脂褐质沉积症患者的光阵发性反应具有与其他癫痫中所见光阵发性反应不同的特征。
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