Pädiatrische Epileptologie

Zusammenfassung

Epilepsien zählen zu den häufigsten chronischen pädiatrischen Erkrankungen. Die Kenntnis pädiatrischer Epilepsiesyndrome und der adäquaten Notfalltherapie epileptischer Anfälle ist bedeutsam. Das West-Syndrom ist eine frühkindliche epileptische Enzephalopathie mit Entwicklungsregression, infantilen Spasmen und Hypsarrhythmie im EEG. Therapeutika der 1. Wahl sind ACTH, Kortikosteroide und Vigabatrin. Prognostisch entscheidend ist die Ätiologie. Die Absence- Epilepsie und benigne Partialepilepsie mit zentrotemporalen Spikes (Rolando-Epilepsie) sind gut behandelbare Syndrome mit günstiger Prognose. Für die Absence-Epilepsie ist Ethosuximid Mittel der 1. Wahl. Bei der Rolando-Epilepsie treten meist nur sporadische, schlafgebundene Anfälle auf. Medikamentös hat sich Sultiam hinsichtlich Anfallskontrolle und günstigem Nebenwirkungsprofil bewährt. Ab einer Anfallsdauer von 3 Minuten ist eine adäquate Notfalltherapie indiziert. Benzodiazepine sind Mittel der Wahl. Persistiert der Anfall, sollte die weitere Therapie unter intensivmedizinischen Bedingungen erfolgen

Summary

Epilepsies belong to the most chronic diseases in childhood. West-Syndrome, Childhood absence epilepsy (CAE) and benign epilepsy childhood with centro-temporal spikes (BECTS) differ in etiology, clinical course, treatment options and prognosis. West-Syndrome ist hallmarked by infantile spasms beginning in the first months of life. Medications of choice are ACTH, Corticosteroids and Vigabatrin. Overall prognosis is poor, developmental disorders and lifelong (severe) epilepsies are common. CAE and BECTS are idiopathic epileptic syndromes with favourable clinical outcome. Ethosuximide is the treatment of choice for typical absence seizures with frequent, but short lasting episodes with impaired consciousness. BECTS is characterized by rare, short-lasting motor seizures of the oropharynx and hemifacial motor seizures, often during sleep. Sulthiame was shown to be efficient and safe.

A generalized tonic clonic seizure lasting longer than 3 minutes requires proper rescue medication. Benzodiazepines should be initially applied. If seizure termination is not achieved, admittance to an intensive care unit is indicated.

Schlüsselwörter

Absence - Rolando - West-Syndrom - Epilepsie - Status epilepticus - Notfalltherapie

Keywords

Childhood absence epilepsy - west syndrome - benign epilepsy in childhood with centro-temporal spikes - status epilepticus - treatment

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