Independent Bilateral Hypothalamic Hamartomas and Gelastic Seizures: What Treatment to Offer?

Abstract

We report a case of a 9-year-old boy with normal cognitive and neurological status. Pharmacoresistant epilepsy started at 2 years of age when he woke up from sleep with epigastric painful sensations. During the course, somatosensory seizures with pain in the legs and, from 3.2 years on, gelastic seizures and dyscognitive seizures occurred. High-resolution magnetic resonance image (MRI) of the brain revealed bilateral independent hypothalamic hamartomas in the supramamillary region. After presurgical evaluation, a decision for an endoscopic resection of the bigger, right-sided hamartoma was made. Seizures improved transiently after surgery (Engel 3a). However, no significant improvement was noted 6 months after surgery. As the only negative consequence, a marked weight gain followed surgery. Cognitive functions remained stable. On the basis of this case, we discuss the therapeutic options for this rare condition which include resection of the contralateral hamartoma or stereotactic laser ablation.