ObjectiveTo assess the effects of the Coronavirus disease 2019 (COVID-19) epidemic on patients with infantile epileptic spasms syndrome and their parents through an online questionnaire survey.MethodsThis study was a cross-sectional study conducted from May to June 2022. The questionnaire focused on seizure attacks, medical visits, and the mental health assessment of parents. ResultsA total of 67 patients with infantile epileptic spasms syndrome were included in this study. During the epidemic, 25.37% of the patients experienced increased spasms, and 73.13% of the patients had limited commercial availability of anti-seizure medications, mainly vigabatrin and clobazam. All patients had difficulty seeking medical services, and about 73.13% of them used telemedicine service. In addition, 31.34% of parents felt anxious, and 73.14% had different degrees of depressive symptoms. ConclusionThe difficulty in seeking medical services, limited commercial availability of anti-seizure medications, and the anxiety and depressive states of parents during the COVID-19 pandemic aggravate the difficulty in management of patients with infantile epileptic spasms syndrome. Improvement of the health care system and medication management strategy, as well as reasonable use of telemedicine service, may help solve this problem.
Objective To explore the evaluation value of burden of amplitudes and epileptiform discharges score (BASED) in the efficacy of adrenocorticotropic hormone (ACTH) combined with magnesium sulfate therapy for infantile epileptic spasms syndrome (IESS). Methods Retrospective collection the clinical and EEG data of 124 patients admitted to the Dongguan Maternal and Child Health Care Hospital from 2015 to 2023, who were diagnosed with IESS and treated with ACTH combined with magnesium sulfate. According to whether there were epileptic seizures 14 days after ACTH treatment, the patients were divided into two groups: non seizure group (n=74 cases) and seizure group (n=50 cases). The BASED system was used to evaluate the relationship between changes in EEG before and after ACTH treatment and clinical efficacy in both groups. The analysis of electroencephalogram included: ① abnormally high amplitude background waves, ② >3 spike foci , ③ grouped multifocal spikes, ④ paroxysmal voltage attenuation. ResultThe control rate of ACTH combined with magnesium sulfate in the treatment of IESS was 59.7% (74/124), and there was no statistically significant difference in the control rate among children with different etiologies (P=0.09). The BASED score suggests that the overall response rate of electroencephalogram (EEG) in infants with epileptic spasm syndrome after treatment was 57.2%. The EEG remission rate in the seizure control group was 81% (60/74), while in the uncontrolled group was 22% (11/50). The EEG remission rate in the seizure control group was significantly higher than that in the uncontrolled group after treatment (P<0.001), and the EEG score was closely related to clinical efficacy (Spearman correlation coefficient rp=0.601, P<0.001). ConclusionThe BASED score is related to clinical efficacy, and it can provide a quantitative basis for evaluating the efficacy of ACTH combined with magnesium sulfate in the treatment of IESS.
Infantile epileptic spasm syndrome (IESS) is an age-dependent epileptic encephalopathy that onset within 3 ~ 12 months. Commonly used Anti-seizure medications (ASMs) have poor efficacy, low long-term control rate and high recurrence rate, and often leave behind sequelae such as developmental delay/intellectual disability, and autism spectrum disorders. At present, the first-line recommended treatments for IESS are Adrenocorticotropic hormone (ACTH), adrenocortical hormone and vigabatrin. If ineffective, other ASMs, ketogenic diets, and surgical treatments can be tried. This article will provide a review of the current status and related clinical applications of IESS treatment.
ObjectiveTo compare and analyze the electroencephalographic (EEG) characteristics of infants with infantile epileptic spasms syndrome (IESS) and healthy infants during sleep using power spectral density (PSD) analysis. MethodsInfants aged 5 to 9 months with IESS were included, along with an equal number of age-matched healthy controls. EEG signals during sleep were recorded using the Nihon Kohden EEG-1200C system. The energy distribution in the theta (θ), alpha (α), sigma (σ), and beta (β) frequency bands, as well as the morphology and values of PSD within the 4 ~ 30 Hz range, were analyzed. Additionally, spectral entropy (SpEn) was calculated to evaluate signal complexity. Results A total of 10 IESS patients and 10 healthy infants were included. There were no significant differences in gender or age between the two groups (P=0.64, P=0.88). In both groups, PSD values showed a linear decreasing trend with increasing frequency. However, the IESS group showed notable differences in PSD morphology, amplitude, and energy distribution compared to controls. These included the absence of a σ-band peak, greater PSD dispersion across electrodes, significant alterations in energy distribution across θ, α, σ, and β bands, and significantly higher PSD values in the 4 ~ 30 Hz range (P<0.000 1). SpEn analysis revealed significantly elevated spectral entropy across the sigma band in the IESS group, indicating a lack of dominant frequencies, increased complexity, reduced rhythmicity, and enhanced disorder. In contrast, healthy controls exhibited elevated SpEn in the alpha band, reflecting the physiological reduction or disappearance of dominant alpha rhythms during sleep. Conclusion Infants with IESS demonstrate distinct EEG characteristics in both PSD and SpEn analyses compared to healthy infants. These quantitative spectral features reflect the underlying abnormalities of EEG in IESS and provide objective insights that complement conventional visual assessment, offering a novel perspective for early diagnosis and therapeutic monitoring.