Abstracts

Rationale: Gelastic epilepsy is often associated with hypothalamic hamartomas. We describe the experience of two university centers treating children with hypothalamic hamartomas. Patient data was reviewed to determine the characteristic onset and course of this disorder. Methods: We performed retrospective chart analysis of the existing medical record of the children between the ages of 0-21 years who were seen in the Epilepsy Centers for intractable epilepsy at the two university hospitals. Five patients were identified with hypothalamic hamartomas. Results: Male to female ratio was 1:4. None of the children had Pallister Hall syndrome and one has precocious puberty. The average age at presentation to the epilepsy center was 4.5 years (range 1.9-7.10 years). Average age of onset of gelastic seizure was 2.4 years (range 0.5-5years); average age at the diagnosis of hypothalamic hemartoma was 4.2 years (range 2.1-7 years. Four children had daily gelastic seizures. All five children had other seizure types. Three children had gelastic seizures preceding the other seizure types. Four children had failed multiple antiepileptic drugs. Only one patient had seizure control on initial monotherapy and did not require surgery. The EEG findings during gelastic seizures in four patients were subtle and non-specific. These four patients under went surgery. Post operatively, the gelastic seizures were resolved. Two patients still have other seizure types, which are under control on monotherapy. Conclusions: The diagnosis of hypothalamic hamartoma is often made following recognition of gelastic epilepsy. However, recognition of gelastic seizures may be delayed due to their clinical appearance and subtle EEG findings. In our series of patients, onset of gelastic seizures preceded the diagnosis of hypothalamic hamartoma by up to 4.5 years. SIGNIFICANCE: Earlier recognition of the seizure type may lead to earlier diagnosis and better treatment of hypothalamic hemartoma.