Abstract
Hypothalamic hamartomas (HHs) are intrinsically epileptogenic lesions associated to medically intractable focal epilepsy mainly characterized by gelastic and focal seizures. Intralesional recording with deep electrodes has documented the presence of ictal discharge arising from inside the lesion. Nevertheless interictal and ictal scalp EEG is poorly informative and non-localizing in a great deal of cases. HH disconnection leads to seizure remission in most cases. To describe the intralesional EEG recordings and to compare them with concomitant scalp EEG and with previous cases reported in literature. We reviewed the medical records of 17 children affected by drug-resistant focal epilepsy associated to HH. We recorded intralesional electrical activity during stereo-endoscopic disconnection in three cases and during deep brain stimulation implantation in one. We also correlated it with the simultaneous scalp-EEG recording. Acute intralesional recordings in our cases confirmed the presence of epileptiform abnormalities intermingled with low-voltage activity, mostly on the same side of the HH attachment. Paroxysmal activity recorded inside the HH was always evident. Mapping of HH epileptogenic activity could be useful to confirm the usefulness of disconnection procedure. This should consider on-site recording from the HH and if abnormalities are detected safely proceed to disconnection of the HH.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Coons SW, Rekate HL, Prenger EC et al (2007) The histopathology of hypothalamic hamartomas: study of 57 cases. J Neuropathol Exp Neurol 66:131–141
Oehl B, Brandt A, Fauser S et al (2010) Semiologic aspects of epileptic seizures in 31 patients with hypothalamic hamartoma. Epilepsia 51:2116–2123
Parvizi J, Le S, Foster BL, Bourgeois B et al (2011) Gelastic epilepsy and hypothalamic hamartomas: neuroanatomical analysis of brain lesions in 100 patients. Brain 134:2960–2968
Delalande O, Fohlen M (2003) Disconnecting surgical treatment of hypothalamic hamartoma in children and adults with refractory epilepsy and proposal of a new classification. Neurol Med Chir 43:61–68
Fohlen M, Lellouch A, Delalande O (2003) Hypothalamic hamartoma with refractory epilepsy: surgical procedures and results in 18 patients. Epileptic Disord 5:267–273
Dorfmüller G, Fohlen M, Bulteau C et al (2008) Surgical disconnection of hypothalamic hamartomas. Neurochirurgie 54:315–319
Harvey AS, Freeman JL (2007) Epilepsy in hypothalamic hamartoma: clinical and EEG features. Semin Pediatr Neurol 14:60–64
Mathieu D, Deacon C, Pinard CA et al (2010) Gamma knife surgery for hypothalamic hamartomas causing refractory epilepsy: preliminary results from a prospective observational study. J Neurosurg 113:215–221
Schulze-Bonhage A, Homberg V, Trippel M et al (2004) Interstitial radiosurgery in the treatment of gelastic epilepsy due to hypothalamic hamartomas. Neurology 62:644–647
Régis J, Scavarda D, Tamura M et al (2007) Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Semin Pediatr Neurol 14:73–79
Arita K, Ikawa F, Kurisu K et al (1999) The relationship between magnetic resonance imaging findings and clinical manifestations of hypothalamic hamartoma. J Neurosurg 91:212–220
Troester M, Haine-Schlagel R, Ng YT et al (2011) EEG and video-EEG seizure monitoring has limited utility in patients with hypothalamic hamartoma and epilepsy. Epilepsia 52:1137–1143
Kahane P, Ryvlin P, Hoffmann D et al (2003) From hypothalamic hamartoma to cortex: what can be learnt from depth recordings and stimulation? Epileptic Disord 5:205–217
Munari C, Kahane P, Francione S et al (1995) Role of the hypothalamic hamartoma in the genesis of gelastic fits (a video-stereo-EEG study). Electroencephalogr Clin Neurophysiol 95:154–160
Blume WT, Lüders HO, Mizrahi E et al (2001) Glossary of descriptive terminology for ictal semiology: report of the ILAE task force on classification and terminology. Epilepsia 42:1212–1218
Marras CE, Rizzi M, Villani F et al (2011) Deep brain stimulation for the treatment of drug-refractory epilepsy in a patient with a hypothalamic hamartoma. Case report. Neurosurg Focus 30:E4
Kuzniecky R, Guthrie B, Mountz J et al (1997) Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. Ann Neurol 42:60–67
Roberts CM, Thompson EM, Selden NR (2011) Transendoscopic intraoperative recording of gelastic seizures from a hypothalamic hamartoma. Pediatr Neurosurg 47:147–151
Conflict of interest
The authors declare that they have no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Electronic supplementary material
Below is the link to the electronic supplementary material.
Rights and permissions
About this article
Cite this article
Specchio, N., Rizzi, M., Trivisano, M. et al. Acute intralesional recording in hypothalamic hamartoma: description of 4 cases. Acta Neurol Belg 115, 233–239 (2015). https://doi.org/10.1007/s13760-014-0374-z
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s13760-014-0374-z