Hypothalamic Hamartomas and Gelastic Seizures. Chilean Experience in a Pediatric Epilepsy Surgery Program during 13 years. Review of the literature

Hypothalamic Hamartomas (HH) are benign tumors consist of hyperplastic heterotopic tissue growing in a disorganized fashion. They are associated to epilepsy usually refractory to medical treatment. The classical description of seizures are “Gelastic”, however HH can produce different types of focal seizures or even generalized symptoms. Objectives: To determine the pre surgical aspects as well as operative techniques with the best outcomes in HH associated to gelastic seizures and epilepsy refractory to medical treatment.

Methods: We studied pediatric patients with HH who underwent Epilepsy surgery between 2004 and 2017. It was analyzed the type of seizures before surgery, the neurological comorbidity, the type of hamartoma seen in MRI, the electroencephalography records and the type of surgery and  outcome according to the Engel’s and ILAE’s classification. Also we compare the neuropsychological evaluation before and after surgery.

Conclusions: Indistinctly of the open surgery technique used, the possibility to remove the totality of the tumor was only 14%. Nonetheless the results evaluated with ILAE’s and Engel’s outcome score were not so bad, possibly because the surgery gets the splitting of the hamartoma from the rest of the hypothalamus.

Author(s):

Maximiliano Paez Nova

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