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Blitz-Nick-Salaam-Epilepsie

Aktuelle Praxis sowie Perspektiven in Diagnostik und Therapie

Infantile spasms

Current practice and perspectives in diagnostics and therapy

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Zusammenfassung

Die Blitz-Nick-Salaam(BNS)-Epilepsie ist eine altersgebundene, epileptische Enzephalopathie mit meist ungünstiger Prognose für die Entwicklung und die Anfallsfreiheit der Betroffenen. Sie wird auch als West-Syndrom oder „infantile spasms (IS)“ bezeichnet und über die typische Anfallssemiologie und ein typisches Muster im Elektroenzephalogramm (EEG, Hypsarrhythmie) definiert. Durch wachsende Erkenntnisse zu Pathophysiologie und Epileptogenese wird die Entwicklung dieses Krankheitsbilds in der frühen Phase der Hirnentwicklung zunehmend verständlich, sodass sich veränderte diagnostische und therapeutische Zugänge ergeben könnten. Ein wesentliches Ziel ist die Besserung der Prognose durch einen frühen Therapiebeginn, um Kindern mit dem erhöhten Risiko, an einer BNS-Epilepsie zu erkranken, Entwicklungschancen zu erhalten. Im vorliegenden Beitrag werden ein Überblick über die derzeitigen Empfehlungen zu diagnostischem und therapeutischem Vorgehen vermittelt sowie die aktuelle Diskussion um das Verständnis dieser Epilepsie dargestellt.

Abstract

Infantile spasms are an age-related epileptic encephalopathy also known as West syndrome, with a mostly unfavorable prognosis concerning development and persisting seizures. The disease is defined by seizure semiology and the typical electroencephalogram (EEG) pattern (hypsarrhythmia). Recent insights into the evolutionary process of the pathophysiology of this disease in the maturing brain possibly open up an altered approach in diagnostics and treatment. A major aim is preserving the developmental chances in children at risk. This review gives an overview on therapeutic and diagnostic guidelines and also discusses new perspectives in the comprehension of infantile epileptic encephalopathy.

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Einhaltung ethischer Richtlinien

Interessenkonflikt. C. Lampe, M. von Rhein, B. Walther und M. Knuf geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Klinik für Kinder und Jugendliche, Dr.-Horst-Schmidt-Kliniken, Ludwig-Erhard-Str. 100, 65199, Wiesbaden, Deutschland

    C. Lampe, B. Walther &  M. Knuf

  2. Departement Kinder- und Jugendmedizin, Sozialpädiatrisches Zentrum, Kantonsspital Winterthur, Winterthur, Schweiz

    M. von Rhein

  3. Abteilung Entwicklungspädiatrie, Kinderspital Zürich, Zürich, Schweiz

    M. von Rhein

Authors
  1. C. Lampe
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  2. M. von Rhein
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  3. B. Walther
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  4. M. Knuf
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Corresponding author

Correspondence to M. Knuf.

Additional information

Frau Dr. med. Birgit Walther zum 70. Geburtstag.

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Lampe, C., von Rhein, M., Walther, B. et al. Blitz-Nick-Salaam-Epilepsie. Monatsschr Kinderheilkd 163, 804–811 (2015). https://doi.org/10.1007/s00112-015-3402-z

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