Emergency Transcallosal Resection of Hypothalamic Hamartoma for “Status Gelasticus”

Hypothalamic hamartomas are rare developmental malformations of the inferior hypothalamus. Typically they result in symptomatic, refractory mixed seizure disorder with trademark gelastic or laughing seizures. We present a 30-month-old boy with a hypothalamic hamartoma and a nearly 2-month history of near-continuous gelastic seizures, which we have labeled "status gelasticus." The seizures were refractory to multiple antiepileptic drugs (AEDs), and emergency transcallosal resection of the hypothalamic hamartoma was performed with resultant near-immediate cessation of the seizures. At 12-month follow-up, the patient was averaging one brief gelastic seizure every 2 weeks, with a longest seizure-free period of 2 months.