The significance of focal pattern in hypsarrhythmia

Introduction: Infantile Epileptic Spasms Syndrome (IESS) presents a therapeutic challenge and is frequently associated with developmental delay. It is characterized by seizures and hypsarrhythmia on the EEG and has multiple etiologies that influence therapeutic decisions. Severity of hypsarrhythmia...

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Main Authors: Anna Wiedemann, Birgit Stark, Gudrun Gröppel
Format: Article
Language:English
Published: Elsevier 2025-03-01
Series:Brain Disorders
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Online Access:http://www.sciencedirect.com/science/article/pii/S2666459325000083
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author Anna Wiedemann
Birgit Stark
Gudrun Gröppel
author_facet Anna Wiedemann
Birgit Stark
Gudrun Gröppel
author_sort Anna Wiedemann
collection DOAJ
description Introduction: Infantile Epileptic Spasms Syndrome (IESS) presents a therapeutic challenge and is frequently associated with developmental delay. It is characterized by seizures and hypsarrhythmia on the EEG and has multiple etiologies that influence therapeutic decisions. Severity of hypsarrhythmia serves as an indicator for evaluating treatment efficacy. However, the correlation between clinical symptoms, EEG patterns, and cerebral lesions requires further investigation. Methods: Eighteen infants diagnosed with IESS underwent video-EEG monitoring and MRI. Hypsarrhythmia severity was assessed using the Kramer et al. scoring system. Clinical semiology was evaluated for lateralizing features. Statistical analyses were conducted to examine correlations between EEG findings, clinical symptoms, and cerebral lesions. Results: All infants exhibited hypsarrhythmia and 72.3 % presented with cerebral lesions, predominantly bilateral. Clinical symptoms frequently indicated lateralization, whereas EEG findings demonstrated lateralization in only 22.2 % of cases. No significant correlation was identified between hypsarrhythmia patterns and clinical symptoms or lesions. The severity of hypsarrhythmia was not a reliable predictor of the underlying etiology. Conclusion: This study found no association between hypsarrhythmic patterns and focal semiology or lesions. Given the heterogeneity of IESS, individualized diagnostic approaches remain essential. While the hypsarrhythmia score enhances the comparability of EEG patterns, its utility in determining the underlying etiology appears to be limited.
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spelling doaj-art-d607c894683d4549be0473ab9eec013d2025-02-08T05:01:19ZengElsevierBrain Disorders2666-45932025-03-0117100188The significance of focal pattern in hypsarrhythmiaAnna Wiedemann0Birgit Stark1Gudrun Gröppel2Department of Paediatrics and Adolescent Medicine, Johannes Kepler University Linz, Krankenhausstraße 26-30, 4020 Linz, AustriaDepartment of Paediatrics and Adolescent Medicine, Johannes Kepler University Linz, Krankenhausstraße 26-30, 4020 Linz, AustriaCorresponding author.; Department of Paediatrics and Adolescent Medicine, Johannes Kepler University Linz, Krankenhausstraße 26-30, 4020 Linz, AustriaIntroduction: Infantile Epileptic Spasms Syndrome (IESS) presents a therapeutic challenge and is frequently associated with developmental delay. It is characterized by seizures and hypsarrhythmia on the EEG and has multiple etiologies that influence therapeutic decisions. Severity of hypsarrhythmia serves as an indicator for evaluating treatment efficacy. However, the correlation between clinical symptoms, EEG patterns, and cerebral lesions requires further investigation. Methods: Eighteen infants diagnosed with IESS underwent video-EEG monitoring and MRI. Hypsarrhythmia severity was assessed using the Kramer et al. scoring system. Clinical semiology was evaluated for lateralizing features. Statistical analyses were conducted to examine correlations between EEG findings, clinical symptoms, and cerebral lesions. Results: All infants exhibited hypsarrhythmia and 72.3 % presented with cerebral lesions, predominantly bilateral. Clinical symptoms frequently indicated lateralization, whereas EEG findings demonstrated lateralization in only 22.2 % of cases. No significant correlation was identified between hypsarrhythmia patterns and clinical symptoms or lesions. The severity of hypsarrhythmia was not a reliable predictor of the underlying etiology. Conclusion: This study found no association between hypsarrhythmic patterns and focal semiology or lesions. Given the heterogeneity of IESS, individualized diagnostic approaches remain essential. While the hypsarrhythmia score enhances the comparability of EEG patterns, its utility in determining the underlying etiology appears to be limited.http://www.sciencedirect.com/science/article/pii/S2666459325000083HypsarrhythmiaInfantile spasmsInfantile epileptic spasms syndrome
spellingShingle Anna Wiedemann
Birgit Stark
Gudrun Gröppel
The significance of focal pattern in hypsarrhythmia
Brain Disorders
Hypsarrhythmia
Infantile spasms
Infantile epileptic spasms syndrome
title The significance of focal pattern in hypsarrhythmia
title_full The significance of focal pattern in hypsarrhythmia
title_fullStr The significance of focal pattern in hypsarrhythmia
title_full_unstemmed The significance of focal pattern in hypsarrhythmia
title_short The significance of focal pattern in hypsarrhythmia
title_sort significance of focal pattern in hypsarrhythmia
topic Hypsarrhythmia
Infantile spasms
Infantile epileptic spasms syndrome
url http://www.sciencedirect.com/science/article/pii/S2666459325000083
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