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

Area of Science:

• Pediatric Neurology
• Epileptology
• Neuroimaging

Background:

• Hypothalamic hamartomas are rare developmental tumors associated with epilepsy.
• Epilepsy in this context often involves gelastic seizures and anteriorly located epileptic networks.
• This study focuses on a rare case with atypical seizure semiology and network involvement.

Purpose of the Study:

• To describe a rare seizure semiology in a child with a hypothalamic hamartoma.
• To investigate an unusual ictal onset and connectivity pattern.
• To review the pathophysiology and management of epilepsy related to hypothalamic hamartomas.

Main Methods:

• Retrospective chart review of a pediatric patient.
• Comprehensive literature search using PubMed and Embase databases.
• Stereoelectroencephalography (SEEG) for ictal onset zone localization.

Main Results:

• A 3-year-old male presented with dyscognitive seizures starting at 22 months.
• SEEG confirmed seizure onset within the hypothalamic hamartoma.
• Seizure propagation involved the temporal-parietal-occipital cortex and precuneus.
• Epilepsy was resolved following stereotactic laser ablation of the hamartoma.

Conclusions:

• This case highlights a rare posterior cortical network epilepsy associated with hypothalamic hamartoma.
• Atypical focal non-motor seizures with impaired awareness were observed.
• Stereotactic laser ablation is an effective treatment, leading to seizure freedom.
• Early diagnosis and prompt treatment are crucial for optimal outcomes in hypothalamic hamartoma-related epilepsy.