Zusammenfassung
Epileptische Enzephalopathien (EE) gehören zu der Gruppe von Epilepsien, die mit ausgeprägten kognitiven Defiziten und Verhaltensstörungen infolge epileptischer Aktivität einhergehen. Trotz großer Bedeutung von EE für die Entwicklung des betroffenen Kindes und dessen psychosozialer Anpassung sind bislang Mechanismen kognitiver Defizite bei EE unzureichend untersucht worden. Der vorliegende Überblick fasst Studien zusammen, die zum Ziel hatten, spezifische neuronale Netzwerke bei EE zu beschreiben. Diese zeigen, dass, obwohl epileptische Aktivität bei EE in unterschiedlichen Gehirnregionen generiert werden kann, spezifische Propagationswege und Netzwerke existieren, die sehr charakteristisch für die jeweilige Art der Enzephalopathie sind. Bei einigen EE scheint die epileptische Aktivität die Integrität des Ruhe(„Default-mode“)-Netzwerks zu beeinträchtigen und möglicherweise über diesen Mechanismus mit kognitiven Funktionen zu interferieren. Darüber hinaus liegen den EE weitere Mechanismen zugrunde, z. B. eine gestörte Konnektivität zwischen Gehirnarealen, die an der Steuerung kognitiver Funktionen beteiligt sind, oder Beeinträchtigungen der Verbindungen bzw. Aktivität in thalamokortikalen Regelkreisen.
Abstract
Epileptic encephalopathies (EE) belong to the group of epilepsies which are associated with expressed cognitive and behavioral disturbances subsequent to epileptic activity. Despite the great importance of EE in the development and psychosocial adaptation of affected children, the mechanisms of cognitive deficits in EE have so far been insufficiently investigated. This review article summarizes the various neuroimaging studies which have tried to describe specific neuronal networks in EE. The results show that although epileptic activity in EE can be generated in different brain regions, specific propagation pathways and networks exist which are very characteristic for each different form of encephalopathy. In some forms of EE the epileptic activity seems to impair the integrity of the default mode network and possibly to interfere with cognitive function through this mechanism. Furthermore, there are further mechanisms underlying EE, e.g. abnormal connectivity patterns between brain regions which participate in the control of cognitive functions and impairment of the connections and activities in the thalamocortical network.
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Interessenkonflikt. M. Siniatchkin, K. Gröning, F. Moeller, N. Japaridze und U. Stephani geben an, dass kein Interessenkonflikt besteht. Der Beitrag enthält keine Studien an Menschen oder Tieren.
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Siniatchkin, M., Gröning, K., Moeller, F. et al. Bildgebung bei epileptischen Enzephalopathien. Z. Epileptol. 27, 100–104 (2014). https://doi.org/10.1007/s10309-013-0354-y
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DOI: https://doi.org/10.1007/s10309-013-0354-y
Schlüsselwörter
- West-Syndrom
- Lennox-Gastaut-Syndrom
- Dravet- Syndrom
- „Continuous spike-and-wave syndrome“
- Funktionelle Magnetresonanztomographie