Temporal Characteristics of High Amplitude Fast Activity on EEG in Children with Beta-propeller Protein-associated Neurodegeneration
Abstract number :
3.518
Submission category :
3. Neurophysiology / 3G. Computational Analysis & Modeling of EEG
Year :
2024
Submission ID :
1600
Source :
www.aesnet.org
Presentation date :
12/9/2024 12:00:00 AM
Published date :
Authors :
Presenting Author: Ryosuke Suzui, MD – Aichi Children's Health and Medical Center
Mitsumatsu Takamasa, MD – Nagoya University Graduate School of Medicine
Ayano Yanagisawa, MD – Nagoya University Graduate School of Medicine
Misa Hashimoto, MD – Nagoya University Graduate School of Medicine
Misae Yamada, MD – Nagoya University Graduate School of Medicine
Hajime Narita, MD – Nagoya University Graduate School of Medicine
Sumire Kumai, MD – Nagoya University Graduate School of Medicine
Anna Shiraki, MD – Nagoya University Graduate School of Medicine
Yuji Ito, PhD – Nagoya University Graduate School of Medicine
Hiroyuki Yamamoto, Assistant Professor – Nagoya University Graduate School of Medicine
Tomohiko Nakata, Lecturer – Nagoya University Graduate School of Medicine
Jun Natsume, Professor – Nagoya University Graduate School of Medicine
Koichi Fujiwara, Associate Professor – Nagoya University Graduate School of Engineering, Nagoya
Kazuhiro Muramatsu, Professor – Jichi Medical University
Hiroyuki Kidokoro, Lecturer – Nagoya University Graduate School of Medicine
Rationale: Beta-propeller protein-associated neurodegeneration (BPAN) is a rare X-linked dominant neurodegeneration with brain iron accumulation disorder, caused by de novo mutations in the WDR45 gene. BPAN is initially characterized by global developmental delay and epilepsy in infancy and childhood. In young adulthood, patients experience rapid cognitive and motor declines with dyskinesia and parkinsonism. Brain MRI shows iron deposits in the globus pallidus and substantia nigra, but these features typically appear in childhood or adolescence. Early diagnosis is challenging because clinical features in infancy and early childhood are nonspecific and the lack of significant findings on imaging studies. We have previously reported that high amplitude fast activity (HAFA) on EEG may serve as a useful diagnostic marker. In this study, we aimed to quantitatively assess the temporal characteristics of HAFA in children with BPAN.
Methods: The scalp EEG channels were placed in accordance with the International 10-20 scalp-electrode allocation. Sampling frequencies of EEG ranged from 200 to 500 Hz. To minimize contamination of motion artifacts, EEG records during sleep were used. We computed the average power across all channels, the power spectrum and the multiscale entropy (MSE) of HAFA, which indicate the scale of amplitude, the distribution of frequency and regularity of waveform, respectively. All of computations were performed on MATLAB 2022b.
Results: A total of twenty EEG recordings from ten children with BPAN (aged 9 to 238 months at the time of recordings) were analyzed. Three patients were diagnosed as epilepsy for focal seizures, atonic seizures and epileptic spasms, relatively. All patients were female and none of the patients were receiving benzodiazepines or barbiturates. The number of recordings per patient ranged from one to four. Figure 1 shows EEG visualization showing longitudinal changes over time in a patient with 4 EEG recordings. Six patients had longitudinal EEG recordings, and a trend of decreasing power values over time was observed (Figure 2). In five of the six patients, the first EEG had the highest power value, and in four patients, the last EEG had the lowest power value. Thirteen (65%) records showed HAFA dominance in the frontal region, two (10%) in the central region, and five (25%) in the occipital region. Some cases exhibited significant changes in electrode distribution depending on the age at the time of recording. However, no clear age-related trends were observed for the power spectrum or MSE.
Conclusions: The power of HAFA tended to decrease with age, suggesting that early diagnosis of BPAN based on HAFA may be more effective when the amplitude is higher at younger ages. However, no consistent temporal trends were found in the frequency distribution or regularity of HAFA.
Funding: No
Neurophysiology