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High-amplitude fast activity in EEG: An early diagnostic marker in children with beta-propeller protein-associated neurodegeneration (BPAN)
Clinical Neurophysiology ( IF 3.7 ) Pub Date : 2020-09-01 , DOI: 10.1016/j.clinph.2020.06.006
Hiroyuki Kidokoro 1 , Hiroyuki Yamamoto 1 , Tetsuo Kubota 2 , Mitsuo Motobayashi 3 , Yusaku Miyamoto 4 , Tomohiko Nakata 1 , Kyoko Takano 5 , Naoko Shiba 3 , Yu Okai 1 , Masaharu Tanaka 1 , Yoko Sakaguchi 1 , Yuki Maki 1 , Masahiro Kawaguchi 1 , Takeshi Suzuki 1 , Kazuhiro Muramatsu 6 , Jun Natsume 1
Affiliation  

OBJECTIVE The 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). METHODS We 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. RESULTS All 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. CONCLUSIONS The EEGs of children with BPAN showed diffuse HAFA continuously when both awake and asleep, which is uncommon in children with other etiologies. SIGNIFICANCE This study provides an important clue for the early diagnosis of BPAN.

中文翻译:

EEG 中的高振幅快速活动:β-螺旋桨蛋白相关神经变性 (BPAN) 儿童的早期诊断标志物

目的 在铁沉积的不同脑磁共振成像 (MRI) 发现发生之前,β-螺旋桨蛋白相关神经变性 (BPAN) 的早期诊断仍然具有挑战性。本研究检查了 BPAN 儿童在脑电图 (EEG) 上是否具有特征性的高振幅 (>50 μV) 快速活动 (HAFA)。方法 我们对 5 名 BPAN 患者在儿童时期进行的脑电图进行了回顾性分析。我们还检查了来自 59 名不同病因患者的 143 次 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 = 4) = 11)。受过训练的脑电图技师检查了所有的脑电图。当观察到过快的活动时,评估幅度、频率和位置。结果 所有 5 名 BPAN 患者在 12-21 个月大时都接受了初始脑电图检查,并且在清醒和睡眠脑电图上均观察到了弥漫性连续 HAFA(范围 20-50 Hz)。在清醒记录中,5 名患者中有 4 名没有明显的后优势节律。尽管 143 个脑电图中 28% 有持续的过快活动,主要是在睡眠记录中,但只有两个(1.4%)在睡眠时表现出 HAFA,并且其清醒的脑电图具有明显的后主导节律。结论 BPAN患儿的脑电图在清醒和睡眠时均持续呈弥漫性HAFA,这在其他病因患儿中并不常见。意义 本研究为BPAN的早期诊断提供了重要线索。在清醒记录中,5 名患者中有 4 名没有明显的后优势节律。尽管 143 个脑电图中 28% 有持续的过快活动,主要是在睡眠记录中,但只有两个(1.4%)在睡眠时表现出 HAFA,并且其清醒的脑电图具有明显的后主导节律。结论 BPAN患儿的脑电图在清醒和睡眠时均持续呈弥漫性HAFA,这在其他病因患儿中并不常见。意义 本研究为BPAN的早期诊断提供了重要线索。在清醒记录中,5 名患者中有 4 名没有明显的后优势节律。尽管 143 个脑电图中 28% 有持续的过快活动,主要是在睡眠记录中,但只有两个(1.4%)在睡眠时表现出 HAFA,并且其清醒的脑电图具有明显的后主导节律。结论 BPAN患儿的脑电图在清醒和睡眠时均持续呈弥漫性HAFA,这在其他病因患儿中并不常见。意义 本研究为BPAN的早期诊断提供了重要线索。结论 BPAN患儿的脑电图在清醒和睡眠时均持续呈弥漫性HAFA,这在其他病因患儿中并不常见。意义 本研究为BPAN的早期诊断提供了重要线索。结论 BPAN患儿的脑电图在清醒和睡眠时均持续呈弥漫性HAFA,这在其他病因患儿中并不常见。意义 本研究为BPAN的早期诊断提供了重要线索。
更新日期:2020-09-01
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