West Syndrome: Questions Aplenty- Few Answers

Kesavan, Shivan; Sankhyan, Naveen

doi:10.1007/s12098-018-02853-9

Cited by 3 publications

(4 citation statements)

References 11 publications

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“…It is characterized by the onset of epileptic spasms, often accompanied with hypsarrhythmia on electroencephalogram (EEG), and developmental stagnation or regression. [1][2][3] Adrenocorticotrophic hormone (ACTH), oral corticosteroids (OCS), and vigabatrin (VGB) are the main treatments for infantile spasms; the optimal treatment for IESS is unknown. 2,4,5 Melatonin is an antioxidant and anti-inflammatory agent synthesized in the pineal gland from tryptophan.…”

Section: Introductionmentioning

confidence: 99%

“…Infantile epileptic spasms syndrome (IESS) is a seizure syndrome that affects infants aged 1–24 months at a rate of 30/100 000. It is characterized by the onset of epileptic spasms, often accompanied with hypsarrhythmia on electroencephalogram (EEG), and developmental stagnation or regression 1–3 . Adrenocorticotrophic hormone (ACTH), oral corticosteroids (OCS), and vigabatrin (VGB) are the main treatments for infantile spasms; the optimal treatment for IESS is unknown 2,4,5 …”

Section: Introductionmentioning

confidence: 99%

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Safety and efficacy of melatonin supplementation as an add‐on treatment for infantile epileptic spasms syndrome: A randomized, placebo‐controlled, double‐blind trial

Sun,

Chen,

Shi

et al. 2023

Journal of Pineal Research

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This was a prospective, randomized, double‐blind, single‐center placebo‐controlled trial to assess the efficacy and safety of melatonin as an add‐on treatment for infantile epileptic spasms syndrome (IESS). Participants aged 3 months to 2 years with a primary diagnosis of IESS were recruited and assigned to two groups in a 1:1 ratio. Both treatment groups received a combination of adrenocorticotrophic hormone (ACTH) and magnesium sulfate (MgSO4) for 2 weeks, and the treatment group also received melatonin (3 mg) between 20:00 and 21:00 daily, 0.5–1 h before bedtime. The study's primary endpoint was the average reduction rate in spasm frequency assessed by seizure diaries. Secondary endpoints included assessment of the response rate, EEG hypsarrhythmia (Kramer score), and psychomotor development (Denver Developmental Screening Test, DDST). Sleep quality was assessed by using the Brief Infant Sleep Questionnaire (BISQ), the Infant Sleep Assessment Scale (ISAS), and actigraphy. Safety parameters were also evaluated. Statistical analyses were conducted on intention‐to‐treat and per‐protocol populations. The trial is registered at Clinicaltrials.gov (ChiCTR2000036208). Out of 119 screened patients, 70 were randomized and 66 completed treatments. In the intention‐to‐treat population, there were no significant differences in the average percentage reduction of spasm frequency (median [interquartile range, IQR: Q3–Q1], 100% [46.7%] vs. 66.7% [55.3%], p = .288), the 3‐day response rate (51.4% vs. 37.1%, p = .229), the 28‐day response rate (42.9% vs. 28.6%, p = .212), EEG Kramer scores (2 [3.5] vs. 2 [3], p = .853), or DDST comprehensive months (5 [2.5] vs. 6 [6], p = .239) between the melatonin (n = 35) and placebo (n = 35) groups. However, caregivers reported improved sleep quality after melatonin treatment, with 85.7% reporting regular sleep compared to 42.9% with placebo (42.9%, p < .001). The melatonin group had lower ISAS scores in 4–11‐month‐old patients compared to the placebo (mean ± SD, 29.3 ± 4.4 vs. 35.2 ± 5.9, p < .001). Moreover, the median (IQR) value of sleep‐onset latency was shortened by 6.0 (24.5) min after melatonin treatment, while that in the placebo group was extended by 3.0 (22.0) min (p = .030). The serum melatonin (6:00 h) level (pg/mL) of the children in the melatonin group after treatment was significantly higher than in the placebo group (median [IQR], 84.8 [142] vs. 17.5 [37.6], p < .001). No adverse effects related to melatonin were observed in the study, and there were no significant differences in adverse effects between the melatonin and placebo groups. Although not statistically significant, the results of this randomized clinical trial proved that melatonin supplementation, as an add‐on treatment, can improve spasm control rate in the treatment of IESS. For IESS children treated with ACTH, the addition of melatonin was found to improve sleep quality, shorten sleep onset latency, and increase blood melatonin levels. Moreover, it was observed to be a safe treatment option.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Safety and efficacy of melatonin supplementation as an add‐on treatment for infantile epileptic spasms syndrome: A randomized, placebo‐controlled, double‐blind trial

Sun,

Chen,

Shi

et al. 2023

Journal of Pineal Research

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“…Infantile spasm (IS), also known as West syndrome, is an age-specific epileptic syndrome in children under the age of 2 years, which occurs in 2-5/10 000 infants and is characterised by a triad of epileptic spasms, hypsarrhythmia on electroencephalogram (EEG) and neurodevelopmental regression. [1][2][3] Treatment strategy for IS has not changed for the last few years and the optimal treatment of IS is still unknown. 4 At present, adrenocorticotrophic hormone (ACTH), oral corticosteroids and vigabatrin are firstline treatments for IS, but there are limited response rate and inevitable side effects.…”

Section: Introductionmentioning

confidence: 99%

Melatonin supplementation for the treatment of infantile spasms: protocol for a randomised placebo-controlled triple-blind trial

et al. 2022

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IntroductionInfantile spasms (IS) is a type of severe epileptic encephalopathy that occurs in infancy and early childhood. IS is characterised clinically by epileptic spasms, often accompanied by sleep disorder and abnormal circadian rhythm. The endogenous circadian rhythm disorder, in turn, can make spasms worse. Melatonin has also been found to have anticonvulsant and neuroprotective properties by adjusting the circadian rhythm. However, there are lack of relevant studies on controlling IS by using melatonin. This study aims to analyse the therapeutic effect of melatonin supplementation for the treatment of IS.Methods and analysisThis is a triple-blinded (trial participant, outcome assessor and the data analyst), prospective, randomised controlled trial to be conducted in the Department of Paediatrics, The First Medical Center of Chinese PLA General Hospital, Beijing, China from November 2020. Patients (n=70) aged 3 months to 2 years with IS will be recruited in this study after receiving written consent from their parents or guardians. Patients will be randomly divided into two equal groups and treated with a combination of adrenocorticotropic hormone, magnesium sulfate and either melatonin or placebo. Clinical data from the patients in the two groups before and after the treatment will be collected and compared. The primary outcome will be assessed 2 weeks later by seizure diaries and reported as the average reduced rate of spasms frequency. Secondary outcomes include the response rate (the rate of spasms-free), electroencephalogram hypsarrhythmia assessment and the psychomotor development assessment (Denver Developmental Screening Test). Sleep quality and safety will also be assessed.Ethics and disseminationThe protocol for this study was approved by the Ethics Committee of Chinese PLA General Hospital (reference number S2020-337-01) and was reported according to the Standard Protocol Items: Recommendations for Interventional Trials statement. Findings of this research will be disseminated through national and international meetings, conferences and peer-reviewed journals.Trial registration numberChiCTR2000036208.

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“…Infantile spasms (IS) is one of the most common age-dependent epileptic encephalopathies in infancy, most of which are difficult to treat and have a poor prognosis ( D’Alonzo et al, 2018 ; Kesavan and Sankhyan, 2019 ). Also known as the WEST syndrome, IS is characterized by a triad of symptoms: epileptic spasm, which is the major seizure type in infancy; impairment of psychomotor development; and the presence of hypsarrhythmia in the interictal electroencephalogram (EEG) ( Wilmshurst et al, 2015 ).…”

Section: Introductionmentioning

confidence: 99%

Automatic BASED scoring on scalp EEG in children with infantile spasms using convolutional neural network

Fan

Chen²,

Wang³

et al. 2022

Front. Mol. Biosci.

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In recent years, the Burden of Amplitudes and Epileptiform Discharges (BASED) score has been used as a reliable, accurate, and feasible electroencephalogram (EEG) grading scale for infantile spasms. However, manual EEG annotation is, in general, very time-consuming, and BASED scoring is no exception. Convolutional neural networks (CNNs) have proven their great potential in many EEG classification problems. However, very few research studies have focused on the use of CNNs for BASED scoring, a challenging but vital task in the diagnosis and treatment of infantile spasms. This study proposes an automatic BASED scoring framework using EEG and a deep CNN. The feasibility of using CNN for automatic BASED scoring was investigated in 36 patients with infantile spasms by annotating their long-term EEG data with four levels of the BASED score (scores 5, 4, 3, and ≤2). In the validation set, the accuracy was 96.9% by applying a multi-layer CNN to classify the EEG data as a 4-label problem. The extensive experiments have demonstrated that our proposed approach offers high accuracy and, hence, is an important step toward an automatic BASED scoring algorithm. To the best of our knowledge, this is the first attempt to use a CNN to construct a BASED-based scoring model.

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West Syndrome: Questions Aplenty- Few Answers

Cited by 3 publications

References 11 publications

Safety and efficacy of melatonin supplementation as an add‐on treatment for infantile epileptic spasms syndrome: A randomized, placebo‐controlled, double‐blind trial

Safety and efficacy of melatonin supplementation as an add‐on treatment for infantile epileptic spasms syndrome: A randomized, placebo‐controlled, double‐blind trial

Melatonin supplementation for the treatment of infantile spasms: protocol for a randomised placebo-controlled triple-blind trial

Automatic BASED scoring on scalp EEG in children with infantile spasms using convolutional neural network

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