CyberKnife® Radiosurgery as First-line Treatment for Catastrophic Epilepsy Caused by Hypothalamic Hamartoma

Hypothalamic hamartomas (HH) are deep-seated lesions often associated with catastrophic epilepsy (an epileptic syndrome characterized by severe, drug-refractory seizures eventually leading to mental retardation and death). Radical microsurgical resection is not feasible for lesions located within the wall of the third ventricle inside the hypothalamus. Frame-based stereotactic radiosurgery has been reported as an effective treatment modality for small- to medium-size intrahypothalamic hamartomas, providing excellent seizure outcomes without lasting complications. This report describes the use of frameless image-guided robotic radiosurgery (CyberKnife® Radiosurgery System) as a first-line treatment in two children with catastrophic epilepsy induced by HH. Both patients experienced multiple-daily complex partial and gelastic seizures, as well as almost daily generalized seizures. The prescribed dose was 16 Gy (to the 65% isodose for case I; to the 70% isodose for case II). Lesional volume was 11.5 cc (case I) and 8.9 cc (case II). A steady reduction of the seizure frequency and severity was achieved after the treatment, starting about three months after the treatment. The generalized seizures disappeared within one year, while complete resolution of the gelastic seizures required up to 18 months. No seizure recurrence and no radiation-induced side effects or complications were witnessed over a follow-up period of ten years and eight months (case I) and nine years and seven months (case II) since the treatment. CyberKnife radiosurgery proved to be a safe and effective non-invasive first-line treatment in these two children with catastrophic epilepsy caused by HH.