Gelastic Fits in a Two Years Old Boy with a Complex Cerebral Malformation

Zusammenfassung

Wir berichten den Fall eines zwei Jahre alten Jungen mit Lachanfällen, bei dem im Verlauf der diagnostischen Abklärung eine komplexe Hirnfehlbildung mit Schizenzephalie, einer Arachnoidalzyste sowie einem partiellen Balkenmangel festgestellt wurde. Unter telemetrischer Diagnostik konnte im EEG ein epileptogener Fokus im rechten zentroparietalen Lappen identifiziert werden. Die Anfallsfrequenz remittierte unter einer konservativen Behandlung mit Carbamazepin fast vollständig. Nach unserer Kenntnis handelt es sich um den ersten Bericht von Lachanfällen in Verbindung mit einer Schizenzephalie. Entgegen dieser üblicherweise medikamentös schwierig zu behandelnden Anfallsform erzielten wir ein äußerst zufriedenstellendes Therapieergebnis. Darüber hinaus entwickelte sich das Kind im Verlauf einer 8 Monate andauernden Beobachtungsphase psychomotorisch völlig normal.

Abstract

We report the case of a two years old boy showing gelastic fits as the leading clinical symptom of a rare complex cerebral malformation with closed-lip schizencephaly, an arachnoid cyst and a partial agenesis of the corpus callosum. After 5 uneventful interictal electroencephalograms the patient underwent 24 h EEG with telemetry while finally presenting two laughing fits with associated spike-wave discharges in the right-centroparietal region. Medical treatment with carbamacepine was initiated and the seizure frequency decreased significantly. This is the first cited case of gelastic fits occuring in schizencephaly. In contrast to previously cited cases with laughing fits we observed a good response to anticonvulsive treatment. Moreover our patient presented a surprisingely favourable psychomental and motor development during an 8 months observation period.

Schlüsselwörter

Schizenzephalie - Arachnoidalzyste - Lachanfälle - medikamentöse Behandlung

Key words

Schizencephaly - arachnoid cyst - partial seizures - psychomental development - medical treatment

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