Epileptic encephalopathies secondary to hypothalamic hamartomas treated with radiosurgery: A case series

• Published in: Epileptic disorders Vol. 26; no. 5; pp. 581 - 590
• Main Authors: Jaramillo‐Jiménez, Esteban, Sandoval‐Barrios, Juliana, Walsh, Fergus John, Jaramillo‐Jiménez, María Clara, Echeverri‐Sánchez, Juan David, Rodríguez‐Márquez, Iader Alfonso, Barrientos‐Montoya, Hernán Darío, Ascencio‐Lancheros, José Luis, Giraldo‐Palacio, John Freddy, Sierra‐Arrieta, Iván Manuel, Gómez‐Duque, David Ignacio, Pérez‐López, Simón, Bustamante, Mariana Torres
• Format: Journal Article
• Language: English
• Published: Boston, USA Wiley Periodicals, Inc 01-10-2024; Wiley Subscription Services, Inc
• Subjects: Adolescent; Child; Child, Preschool; Convulsions & seizures; Drug Resistant Epilepsy - etiology; Drug Resistant Epilepsy - surgery; encephalopathy; Epilepsy; Female; hamartoma; Hamartoma - complications; Hamartoma - surgery; Humans; Hypothalamic Diseases - complications; Hypothalamic Diseases - surgery; Hypothalamus; Male; Morbidity; Neoplasia; Neurodevelopmental disorders; Patients; Radiosurgery; Radiosurgery - methods; Seizures; Visual pathways; epilepsy; radiosurgery; encephalopathy; hamartoma; hypothalamus
• ISSN: 1294-9361; 1950-6945; 1950-6945
• DOI: 10.1002/epd2.20246

Objective
Hypothalamic hamartomas are congenital lesions that typically present with gelastic seizures, refractory epilepsy, neurodevelopmental delay, and severe cognitive impairment. Surgical procedures have been reported to be effective in removing the hamartomas, however, they are associated with significant morbidity. Therefore, it is not considered a safe therapeutic modality. Image‐guided robotic radiosurgery (CyberKnife® Radiosurgery System) has been shown to provide good outcomes without lasting complications.
Methods
This series of cases describes the clinical, radiological, radiotherapeutic, and postsurgical outcomes of five patients with epileptic encephalopathies secondary to hypothalamic hamartomas who were treated with CyberKnife®.
Results
All patients exhibited refractory epilepsy with gelastic seizures and were unsuitable candidates for surgical resection The prescribed dose ranged between 16 and 25 Gy, delivered in a single fraction for four patients and five fractions for one patient while adhering strictly to visual pathway constraints. After radiosurgery, four patients maintained seizure control (one with an Engel class Ia, three with an Engel class 1d), and another presented sporadic, nondisabling gelastic seizures (with an Engel class IIa). After 24–26 months of follow‐up, in three patients, their intelligence quotient scores increased. No complications were reported.
Significance
This report suggests that Cyberknife may be a good option for treating hypothalamic hamartoma, particularly in cases where other noninvasive alternatives are unavailable. Nevertheless, additional studies are essential in order to evaluate the effectiveness of the technique in these cases.