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Co-existence of self-limited focal epilepsies of childhood (SelFEC) and epileptiform discharges in the context of SelFEC in children with structural focal epilepsies and in children with brain lesions

Implications for diagnosis and management

Koexistenz von selbstlimitierenden fokalen Epilepsien des Kindesalters (SelFEK) und epileptiformen Entladungen im Kontext von SelFEK bei Kindern mit strukturellen fokalen Epilepsien und Kindern mit Hirnläsionen

Bedeutung für Diagnose und Behandlung

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Abstract

The co-occurrence of self-limited focal epilepsies of childhood (SelFEC) and epileptiform discharges in the context of SelFEC (EDSelFEC) in children with structural focal epilepsies and with brain lesions has received little attention in the literature to date, mainly in the form of case reports. The few reports on the occurrence of EDSelFEC and SelFEC in children who have undergone presurgical evaluation due to focal seizures that cannot be controlled by medication also suggests that these are relatively rare constellations. Recent publications, on the other hand, are showing that in children who have suffered pre-/perinatal complications, the incidence and prevalence of EDSelFEC and SelFEC are relatively high, especially when the white matter is damaged. The pathophysiology that leads to the generation of EDSelFEC and SelFEC, when the white matter is damaged, is not fully understood. A surprisingly high number of abnormalities is detected when magnetic resonance imaging is performed systematically on children with self-limited epilepsy with centrotemporal spikes (SelECTS). A misunderstanding of EDSelFEC and SelFEC as expressions of epileptogenic focal structural (cortical) lesions can, in individual cases, lead to a significant deterioration of epilepsy due to the administration of potentially aggravating medications, such as sodium channel blockers, and in children who have suffered pre-/perinatal complications the parents may not be informed about the relatively positive long-term prognosis of their child’s epilepsy. This article reviews recent findings on the prevalence of EDSelFEC and SelFEC in children with structural focal epilepsies and with brain lesions and their potential impact on diagnosis and treatment.

Zusammenfassung

Das gleichzeitige Auftreten von selbstlimitierenden fokalen Epilepsien des Kindesalters (SelFEK) und epileptiformen Entladungen im Kontext von SelFEK (EDSelFEK) bei Kindern mit Hirnläsionen und Kindern mit strukturellen fokalen Epilepsien hat in der medizinischen Literatur bisher nur wenig Beachtung gefunden, hauptsächlich in Form von Fallberichten. Berichte über das Auftreten von SelFEK und EDSelFEK bei Kindern, bei denen wegen fokaler epileptischer Anfälle, die nicht durch Medikamente zu kontrollieren sind, eine prächirurgische Diagnostik durchgeführt wurde, vermitteln ebenfalls den Eindruck, dass es sich eher um seltene Konstellationen handelt. Neuere Publikationen zeigen andererseits auf, dass bei Kindern, bei welchen Hirnläsionen aufgrund prä-/perinataler Komplikationen aufgetreten sind, die Inzidenz und Prävalenz von SelFEK und EDSelFEK relativ hoch ist, insbesondere dann, wenn eine Schädigung der weißen Substanz vorliegt. Die Pathophysiologie, welche zu einer vermehrten Generierung von EDSelFEK und SelFEK bei einer Schädigung der weißen Substanz führt, ist noch unklar. Eine überraschend hohe Zahl von neuroradiologischen Auffälligkeiten zeigt sich in systematischen Magnetresonanztomographie-Untersuchungen von Kindern mit selbstlimitierenden fokalen Epilepsien mit zentro-temporalen spikes (englischsprachige Abkürzung SELECTS). Das Verkennen von EDSelFEK und SelFEK als Ausdruck von strukturellen (kortikalen) Läsionen kann in Einzelfällen durch die Verabreichung von potenziell aggravierenden Medikamenten, z. B. Natrium-Kanal-Blocker, zu einer erheblichen Verschlechterung der Epilepsie führen, und bei Kindern, bei denen prä-/perinatale Komplikationen aufgetreten sind, werden die Eltern möglicherweise nicht über die eher günstige Langzeitprognose der Epilepsie ihres Kindes informiert. In der vorliegenden Arbeit werden aktuelle Ergebnisse zur Prävalenz von EDSelFEK und SelFEK bei Kindern mit strukturellen fokalen Epilepsien und Hirnläsionen sowie deren mögliche Auswirkungen auf die Diagnose und Behandlung erörtert.

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Authors and Affiliations

  1. Center for Pediatric Neurology, Neurorehabilitation, and Epileptology, schoen Clinic Vogtareuth, Vogtareuth, Krankenhausstr.20, 83569, Germany

    H. Holthausen, T. Pieper, A. Zsoter, G. Kluger, S. Berweck & T. Hartlieb

  2. Center for Pediatric Orthopedics, schoen Clinic Vogtareuth, Vogtareuth, Germany

    A. Brockhaus

  3. iSPZ Hauner, Comprehensive Epilepsy Program for Children Division of Pediatric Neurology, Developmental Medicine and Social Pediatrics, Department of Pediatrics, University Hospital Munich, Munich, Germany

    C. von Stülpnagel

  4. Research Institute “Rehabilitation, Transition and Palliation”, Paracelsus Medical University Salzburg, Salzburg, Austria

    C. von Stülpnagel, G. Kluger & T. Hartlieb

  5. Dr. von Hauner’sches Kinderspital, Ludwig Maximilian University Clinic, Munich, Germany

    S. Berweck

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  1. H. Holthausen
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Correspondence to H. Holthausen.

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H. Holthausen, A. Brockhaus, C. von Stülpnagel, T. Pieper, A. Zsoter, G. Kluger, S. Berweck and T. Hartlieb declare that they have no competing interests.

For this article no studies with human participants or animals were performed by any of the authors. All studies mentioned were in accordance with the ethical standards indicated in each case.

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Holthausen, H., Brockhaus, A., von Stülpnagel, C. et al. Co-existence of self-limited focal epilepsies of childhood (SelFEC) and epileptiform discharges in the context of SelFEC in children with structural focal epilepsies and in children with brain lesions. Clin Epileptol 37, 25–35 (2024). https://doi.org/10.1007/s10309-023-00653-6

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