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Purpose of Review <p>Infantile Epileptic Spasms Syndrome (IESS) and Developmental and/or Epileptic Encephalopathy with Spike-Wave Activation in Sleep (DEE-SWAS, EE-SWAS) are two of the infantile and early childhood epileptic encephalopathies that require ongoing research to improve and standardize consensus guidelines for diagnosis and treatment.</p> Recent Findings <p>In patients with IESS, recent studies regarding the “2021 BASED” score have proposed a reliable and consistent manner in determining hypsarrhythmia, while others have investigated the question of non-inferiority between sequential versus combination therapy for treatment of IESS. As for D/EE-SWAS, there is no updated consensus on the required threshold for the diagnosis of D/EE-SWAS, but recent studies have helped clinical neurophysiologists become more efficient in reviewing these studies, either with shortened review periods or with automated spike detectors. In terms of treatment, there is not enough evidence to name a first line treatment for D/EE-SWAS (either steroids or clobazam), despite a recent randomized control trial.</p> Summary <p>For IESS, many physicians and centers continue to utilize sequential therapy while some centers are using combination therapy (vigabatrin plus hormonal therapy), both with decent results. There is a movement in the United States to try to use the “BASED” score for determination of hypsarrhythmia.</p> <p>For D/EE-SWAS, despite a recent randomized control trial, it is still appropriate to use clobazam or corticosteroids for first line treatment. There are also more efficient ways to calculate the spike wave index that are still clinically meaningful and significant, such as with semi-automated spike detection software, or focusing on just the first 100&#xa0;seconds of slow wave sleep.</p>

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Update on Diagnosis and Treatment of Infantile Epileptic Spasms Syndrome and Developmental and Epileptic Encephalopathy with Spike Wave Activation in Sleep

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2026年1月23日

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  • Daniel Lapid,
  • Natasha Varughese

摘要

Purpose of Review

Infantile Epileptic Spasms Syndrome (IESS) and Developmental and/or Epileptic Encephalopathy with Spike-Wave Activation in Sleep (DEE-SWAS, EE-SWAS) are two of the infantile and early childhood epileptic encephalopathies that require ongoing research to improve and standardize consensus guidelines for diagnosis and treatment.

Recent Findings

In patients with IESS, recent studies regarding the “2021 BASED” score have proposed a reliable and consistent manner in determining hypsarrhythmia, while others have investigated the question of non-inferiority between sequential versus combination therapy for treatment of IESS. As for D/EE-SWAS, there is no updated consensus on the required threshold for the diagnosis of D/EE-SWAS, but recent studies have helped clinical neurophysiologists become more efficient in reviewing these studies, either with shortened review periods or with automated spike detectors. In terms of treatment, there is not enough evidence to name a first line treatment for D/EE-SWAS (either steroids or clobazam), despite a recent randomized control trial.

Summary

For IESS, many physicians and centers continue to utilize sequential therapy while some centers are using combination therapy (vigabatrin plus hormonal therapy), both with decent results. There is a movement in the United States to try to use the “BASED” score for determination of hypsarrhythmia.

For D/EE-SWAS, despite a recent randomized control trial, it is still appropriate to use clobazam or corticosteroids for first line treatment. There are also more efficient ways to calculate the spike wave index that are still clinically meaningful and significant, such as with semi-automated spike detection software, or focusing on just the first 100 seconds of slow wave sleep.