Objective: Hypothalamic hamartomas (HHs) are often associated with early-onset gelastic seizures, thus configuring a well recognized and usually severe case of childhood epilepsy syndrome. We present a treatment modality for intractable epilepsy in hypothalamic hamartomatous lesions.
Methods: This study presents 14 patients with medically refractory seizure associated with HHs treated between 1995 and 2005. The HHs were diagnosed on the basis of magnetic resonance imaging, except in the case of one patient in whom hamartoma was confirmed histologically. There were seven boys and seven girls in this study. The most frequent clinical presentations were seizures. To identify the epileptic focus, we performed comprehensive epilepsy investigations, including electroencephalographic recording using a depth electrode into the hamartoma.
Results: To control the seizure, we performed surgical resection in one patient, gamma knife radiosurgery in four patients, and endoscopic disconnection in 11 patients. Seizure outcome was scored according to Engel's classification throughout a mean follow-up period of 27.4 months (range, 3-54 mo). Of the 11 patients who underwent endoscopic disconnection, six were seizure-free immediately after surgery. Two patients were already diagnosed as having an HH and underwent gamma knife radiosurgery, but seizure control was not achieved. Their gelastic seizure disappeared after endoscopic disconnection.
Conclusion: We confirmed that HHs are intrinsically epileptogenic. Therefore, we suggest that HH-related seizures may be controlled by blocking the seizure propagation from epileptogenic HHs through simple disconnection, regardless of the treatment modality, and the endoscopic disconnection of HHs is safer and more effective than other modalities.