Hypothalamic hamartoma clinically manifested with gelastic intractable epilepsy: a case report with review of the literature
Bulgarian Medical Journal, 2023, 17(2), 51-55.
D. Metodiev1, K. Minkin2, S. Nachev1
1 Neuropathological Laboratory, University Hospital “Sv. Ivan Rilski” – Sofia
2 Department of Neurosurgery, Epilepsy Surgery Center, University Hospital “Sv. Ivan Rilski” – Sofia
Abstract. Hypothalamic hamartoma is one of the rarest epileptogenic lesions associated with drug-resistant epilepsy. The authors present a 17-year-old girl with hypothalamic hamartoma and drug-resistant epilepsy with gelastic seizures and behavior problems. Partial hamartoma resection with significant seizure frequency reduction was performed. The preoperative and postoperative magnetic resonance imaging (MRI) were presented. The histological examination found groups of relatively small neurons forming small nodules, which were partially separated by glial cells with a sparse neuropil. The immunohistochemical examination showed positive labeling of neurons with NeuN and S-100 protein and low proliferative index. The different clinical manifestations and therapeutic options and their relation to the anatomical features of hypothalamic hamartoma were discussed. A review of the literature was performed.
Key words: drug-resistant epilepsy, gelastic seizures, glial cells, neurons, hypothalamic hamartoma
Address for correspondence: Dr. Dimitar Metodiev, e-mail: dimitarmetodievvv@gmail.com