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[Gelastic seizures treated by partial resection of a hypothalamic hamartoma]
T Watanabe1, T Enomoto, K Uemura
1Department of Neurosurgery, University of Tsukuba, Japan.
Insights
Surgical removal of hypothalamic hamartoma can effectively treat gelastic epilepsy. Conservative resection, even if partial, may resolve intractable seizures and improve EEG findings.
Area of Science:
- Neurology
- Neurosurgery
- Pediatric Epilepsy
Background:
- Gelastic epilepsy, characterized by sudden, brief episodes of laughter or smiling, can be a challenging neurological condition.
- Hypothalamic hamartomas are rare tumors often associated with refractory epilepsy, including gelastic seizures.
- Intractable epilepsy in children necessitates exploration of alternative therapeutic strategies beyond medical management.
Observation:
- A 13-year-old girl presented with a history of atonic seizures from age 2 and gelastic seizures from age 10.
- MRI revealed a hypothalamic mass extending into the basal cistern and third ventricle.
- Interictal EEG demonstrated paroxysmal spike-and-wave discharges, indicative of epileptic activity.
Findings:
- Surgical resection via an interhemispheric trans-lamina terminalis approach was performed for the hypothalamic mass.
- Histological examination confirmed the lesion as a hamartoma.
- Postoperative MRI showed residual tumor, yet the patient remained seizure-free. One-year follow-up EEG normalized, despite continued anticonvulsant medication.
Implications:
- Surgical intervention should be considered for intractable gelastic epilepsy associated with hypothalamic hamartoma.
- A conservative surgical approach, prioritizing avoidance of complications, can yield significant seizure control even with subtotal resection.
- This case highlights the potential efficacy of tailored surgical strategies in managing complex pediatric epilepsy syndromes.
Abstract:
A 13-year-old girl developed atonic seizure at 2 years of age. At the age of 10 years, gelastic seizures were noted. Magnetic resonance imaging (MRI) revealed a hypothalamic mass protruding down into the basal cistern and up into the third ventricle. An interictal electroencephalogram (EEG) showed paroxysmal spike and wave complex discharges. Since the seizures failed to respond to medical therapy, it was decided to try to control them by removing the mass. The operation was carried out through an interhemispheric trans-lamina terminalis approach. The lesion was so similar to normal brain tissue that the resection had to be limited enough to avoid complications. Histological examination of the mass showed a hamartoma. Postoperative MRI showed residual mass, but no seizure has been noted since the operation. The EEG recorded one year after the operation showed no spike and wave complex discharge, although she was still on anticonvulsant drugs. The authors propose that surgical therapy should be considered as a treatment for intractable gelastic epilepsy with hypothalamic hamartoma and that the first operation should be conservative enough to avoid complications, because it can bring about good results even if it is only a partial resection.