Gelastic fits in a two years old boy with a complex cerebral malformation
J Froelich1, R Sassen, C E Elger
1Department of Child and Adolescent Psychiatry, University of Cologne, Germany.
Insights
This study details a rare case of gelastic seizures in a child with schizencephaly, showing a positive response to carbamazepine treatment and favorable development.
Area of Science:
- Neurology
- Pediatric Neurology
- Neuroimaging
Background:
- Gelastic seizures are rare, often linked to hypothalamic hamartomas.
- Schizencephaly is a complex congenital brain malformation characterized by a cleft in the cerebral hemisphere.
- This case presents a unique combination of schizencephaly, arachnoid cyst, and partial agenesis of the corpus callosum.
Observation:
- A two-year-old boy presented with gelastic fits as the primary symptom.
- Electroencephalography (EEG) with telemetry revealed spike-wave discharges during laughing fits.
- The patient had a complex cerebral malformation including closed-lip schizencephaly.
Findings:
- This is the first reported case of gelastic fits associated with schizencephaly.
- Carbamazepine treatment led to a significant reduction in seizure frequency.
- The patient exhibited favorable psycho-mental and motor development over an 8-month observation period.
Implications:
- Highlights a novel association between gelastic seizures and schizencephaly.
- Suggests carbamazepine as an effective treatment for gelastic seizures in this context.
- Emphasizes the potential for positive neurodevelopmental outcomes despite complex brain malformations.
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 occurring in schizencephaly. In contrast to previously cited cases with laughing fits we observed a good response to anticonvulsive treatment. Moreover our patient presented a surprisingly favourable psycho-mental and motor development during an 8 months observation period.
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