High-amplitude fast activity in EEG: An early diagnostic marker in children with beta-propeller protein-associated neurodegeneration (BPAN)
High-amplitude fast activity in EEG: An early diagnostic marker in children with beta-propeller protein-associated neurodegeneration (BPAN)
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脑电图高振幅快速活动：β-螺旋桨蛋白相关神经变性 (BPAN) 儿童的早期诊断标志物
DOI：
10.1016/j.clinph.2020.06.006
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发表时间：
2020
影响因子：
4.7
通讯作者：
Natsume Jun
中科院分区：
文献类型：
--
作者：
Kidokoro Hiroyuki;Yamamoto Hiroyuki;Kubota Tetsuo;Motobayashi Mitsuo;Miyamoto Yusaku;Nakata Tomohiko;Takano Kyoko;Shiba Naoko;Okai Yu;Tanaka Masaharu;Sakaguchi Yoko;Maki Yuki;Kawaguchi Masahiro;Suzuki Takeshi;Muramatsu Kazuhiro;Natsume Jun
β-螺旋桨蛋白相关神经变性（BPAN）的早期诊断之前，明显的脑磁共振成像（MRI）结果的铁沉积发生仍然具有挑战性。本研究旨在探讨儿童BPAN是否有特征性的高振幅（>50 μV）快速活动（HAFA）脑电图（EEG）。方法我们进行了回顾性分析，在儿童期进行的EEG在5例BPAN。我们还检查了59例不同病因的患者的143例EEG，包括癫痫（n = 33），急性脑病（n = 6），神经发育障碍（n = 5），非癫痫事件（n = 4）和其他（n = 11）。受过训练的脑电图师审查了所有的脑电图。当观察到过度的快速活动，振幅，频率和位置进行了assessed.ResultsAll 5例BPAN患者进行初始脑电图在12-21个月大，和弥漫性连续HAFA（范围20-50 Hz）观察到清醒和睡眠脑电图。5例患者中4例在清醒时记录无明显后优势心律。虽然143例脑电图中有28%有持续过快活动，主要是在睡眠记录中，（1.4%）的BPAN患儿在睡眠时出现HAFA，清醒时EEG呈明显的后优势节律。本研究为BPAN的早期诊断提供了重要线索。
ObjectiveThe early diagnosis of beta-propeller protein-associated neurodegeneration (BPAN) before distinct brain magnetic resonance imaging (MRI) findings of iron deposition occur remains challenging. This study examined whether children with BPAN have characteristic high-amplitude (>50 μV) fast activity (HAFA) on electroencephalography (EEG).MethodsWe conducted a retrospective analysis of EEG performed during childhood in five patients with BPAN. We also examined 143 EEGs from 59 patients with different etiologies, including epilepsy (n = 33), acute encephalopathy (n = 6), neurodevelopmental disorders (n = 5), non-epileptic events (n = 4), and others (n = 11). Trained electroencephalographers reviewed all of the EEGs. When excessive fast activity was observed, the amplitude, frequency, and locality were assessed.ResultsAll five patients with BPAN underwent initial EEGs at 12–21 months old, and diffuse continuous HAFA (range 20–50 Hz) was observed on both awake and sleep EEGs. In the awake records, there was no clear posterior dominant rhythm in 4 of the 5 patients. Although 28% of the 143 EEGs had continuous excessive fast activity, mainly in the sleep records, only two (1.4%) exhibited HAFA when asleep, and their awake EEGs had clear posterior dominant rhythm.ConclusionsThe EEGs of children with BPAN showed diffuse HAFA continuously when both awake and asleep, which is uncommon in children with other etiologies.SignificanceThis study provides an important clue for the early diagnosis of BPAN.
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影响因子：
7.1
作者：
Paudel R;Li A;Wiethoff S;Bandopadhyay R;Bhatia K;de Silva R;Houlden H;Holton JL
通讯作者：
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影响因子：
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