Articles, Issue 4/2024

The First Case of Radiofrequency Thermocoagulation of Hypothalamic Hamartoma with Drug-resistant Epilepsy in Bulgaria

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К. Minkin, I. Ivanov, I. Pacheva, K. Gabrovski, I. Milenova, P. Karazapryanov, V. Pavlov, P. Dimova

Abstract

Introduction: Epilepsy associated with hypothalamic hamartoma is frequently drug resistant. The modern minimally invasive surgical treatment of this disease is highly effective and has low risks. The aim of our paper is to present the first case of stereotactic radiofrequent thermocoagulation of hypothalamic hamartoma with drug-resistant epilepsy in Bulgaria.

Case description: We present a 4-years-old girl which seizures with smiles and cry have started at the age of 3 years. Some of the seizures were associated with head and eyes version to the right or even activity stop, staring and uncomprehensive speech. The antiseizure medication with valproic acid and clonazepam only reduced seizures which continue to be dayly, even multiple per day (to 6 seizures per day). 

The interictal EEG found bilateral sharps and spikes and slow waves in the posterior regions. The ictal EEG consisted in low-voltage fast activity in the right posterior temporal regions, especially T4-Tp2. The magnetic resonance immaging (MRI) found hypothalamic lesion, 10 mm in diameter, located in the region of the tuber cinereum with the caracteristics of hamartoma. After multidisciplinary discussion, a minimally invasive stereotactic surgery of the hypothalamic hamartoma was proposed. We performed 3 stereotactic thermocoagulations using 3 closed trajectory on the border between the hypothalamic hamartoma and the hypothalamus. During the postoperative period, there were no neurological or psychological deficits. The child remain seizure free and demonstrate significant cognitive development during the follow-up of 1 year.

Conclusion: Drug-resistant epilepsies due to hypothalamic hamartomas could be successfully treated using minimally invasive stereotactic thermocoagulations. The early diagnosis of epileptogenic lesions including hypothalamic hamartomas needs early MRI.

Key words: epilepsy, hypothalamic hamartoma, gelastic seizures, stereotactic thermocoagulations, epilepsy surgery

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Bibliography

  1. Delalande O, Fohlen M. Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification. Neurol Med Chir (Tokyo). 2003;43(2):61-8.
  2. Shirozu H, Masuda H, Kameyama S. Long-term seizure outcomes in patients with hypothalamic hamartoma treated by stereotactic radiofrequency thermocoagulation. Epilepsia. 2021;62(11):2697-706.
  3. Castinetti F, Brue T, Morange I, Carron R, Regis J. Gamma Knife radiosurgery for hypothalamic hamartoma preserves endocrine functions. Epilepsia. 2017;58 Suppl 2:72-6.
  4. Kameyama S, Murakami H, Masuda H, Sugiyama I. Minimally invasive magnetic resonance imaging-guided stereotactic radiofrequency thermocoagulation for epileptogenic hypothalamic hamartomas. Neurosurgery. 2009;65(3):438-49; discussion 49.
  5. Brandberg G, Raininko R, Eeg-Olofsson O. Hypothalamic hamartoma with gelastic seizures in Swedish children and adolescents. Eur J Paediatr Neurol. 2004;8(1):35-44.
  6. Rubino S, Qian J, Pinheiro-Neto CD, Kenning TJ, Adamo MA. A familial syndrome of hypothalamic hamartomas, polydactyly, and SMO mutations: a clinical report of 2 cases. J Neurosurg Pediatr. 2018;23(1):98-103.
  7. Tsumori M, Itonaga T, Oyake M, Hirano N, Oyama N, Ihara K. Infantile Hypothalamic Hamartoma: A Rare Presentation of Isolated Obesity. JCEM Case Rep. 2024;2(3):luae033.
  8. Kahane P, Ryvlin P, Hoffmann D, Minotti L, Benabid AL. From hypothalamic hamartoma to cortex: what can be learnt from depth recordings and stimulation? Epileptic Disord. 2003;5(4):205-17.
  9. Munari C, Tassi L, Berta E, Kahane P, Quarato PP. Case of a child with gelastic seizures and hypothalamic hamartoma. Epilepsia. 1997;38(12):1364-5.
  10. Munari C, Kahane P, Francione S, Hoffmann D, Tassi L, Cusmai R, et al. Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study). Electroencephalogr Clin Neurophysiol. 1995;95(3):154-60.
  11. Kuzniecky R, Guthrie B, Mountz J, Bebin M, Faught E, Gilliam F, et al. Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. Ann Neurol. 1997;42(1):60-7.
  12. Tran TP, Truong VT, Wilk M, Tayah T, Bouthillier A, Mohamed I, et al. Different localizations underlying cortical gelastic epilepsy: case series and review of literature. Epilepsy Behav. 2014;35:34-41.
  13. Palmini A, Chandler C, Andermann F, Costa Da Costa J, Paglioli-Neto E, Polkey C, et al. Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy. Neurology. 2002;58(9):1338-47.
  14. Scholly J, Staack AM, Kahane P, Scavarda D, Regis J, Hirsch E, et al. Hypothalamic hamartoma: Epileptogenesis beyond the lesion? Epilepsia. 2017;58 Suppl 2:32-40.
  15. Freeman JL, Harvey AS, Rosenfeld JV, Wrennall JA, Bailey CA, Berkovic SF. Generalized epilepsy in hypothalamic hamartoma: evolution and postoperative resolution. Neurology. 2003;60(5):762-7.
  16. Calisto A, Dorfmuller G, Fohlen M, Bulteau C, Conti A, Delalande O. Endoscopic disconnection of hypothalamic hamartomas: safety and feasibility of robot-assisted, thulium laser-based procedures. J Neurosurg Pediatr. 2014;14(6):563-72.
  17. Kuzniecky RI, Guthrie BL. Stereotactic surgical approach to hypothalamic hamartomas. Epileptic Disord. 2003;5(4):275-80.
  18.   Regis J, Scavarda D, Tamura M, Villeneuve N, Bartolomei F, Brue T, et al. Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol. 2007;14(2):73-9.
  19. Regis J, Bartolomei F, de Toffol B, Genton P, Kobayashi T, Mori Y, et al. Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Neurosurgery. 2000;47(6):1343-51; discussion 51-2.
  20. Homma J, Kameyama S, Masuda H, Ueno T, Fujimoto A, Oishi M, et al. Stereotactic radiofrequency thermocoagulation for hypothalamic hamartoma with intractable gelastic seizures. Epilepsy Res. 2007;76(1):15-21.
  21.   Du VX, Gandhi SV, Rekate HL, Mehta AD. Laser interstitial thermal therapy: A first line treatment for seizures due to hypothalamic hamartoma? Epilepsia. 2017;58 Suppl 2:77-84.

Address for correspondence: 

Department of Neurosurgery 

University Hospital “St. Ivan Rilski”, Sofia

15, “Akad. Ivan Geshov”, Blvd.

1606, Sofia, Bulgaria

minkin@abv.bg