An Unusual Case of Hypothalamic Hamartoma With Nongelastic Seizures and Posterior Cortex Connectivity

Background: To describe a rare seizure semiology originating from a hypothalamic hamartoma in a child, along with unusual ictal onset and connectivity pattern, and provide a review of the pathophysiology of epilepsy associated with hypothalamic hamartoma and management. Methods: A detailed retrospective chart review and literature search were performed using Pubmed and Embase. Results: We present a case of a three -year -old male who presented with dyscognitive seizures with onset at age 22 months. Stereoelectroencephalography exploration con firmed the onset in hypothalamic hamartoma with rapid propagation to the temporal -parietal -occipital association cortex and precuneus. The patient's epilepsy was cured with laser ablation of the hamartoma. Conclusion: Published literature mostly describes a more anterior frontal or temporal epileptic network with primarily gelastic seizures being the hallmark type of seizures associated with hypothalamic hamartoma. We highlight a rare posterior cortex network with an atypical presentation of focal nonmotor seizures with impaired awareness in the setting of a hypothalamic hamartoma. Stereotactic laser ablation of the hamartoma rendered seizure freedom. Early diagnosis and appropriate treatment can lead to seizure freedom. (c) 2024 Published by Elsevier Inc.