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Stereotactic disconnection of hypothalamic hamartoma to control seizure and behavior disturbance: case report and literature review

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Abstract

An 18-year-old boy with refractory epilepsy and aggressiveness associated to a hypothalamic hamartoma was submitted to a stereotactically guided lesion by thermocoagulation. The target was based on magnetic resonance (MR) images merged with computed tomography scan images taken on the day of surgery while patient was on a stereotactic frame. In order to reveal structures not discernible in MR images, the Schaltenbrand digital brain atlas was merged onto the patient’s images. Target and trajectory of the depth electrode were chosen based on three-dimensional imaging reconstructions. A surgical plan was devised to disconnect the hypothalamic hamartoma from the hypothalamus, medial forebrain bundle, fasciculus princeps, and dorsal longitudinal fasciculus. Our target was placed at the inferior portion of the posterolateral component of the hamartoma, bordering the normal hypothalamus. The patient evolved with marked lessening of aggressiveness. Seizure frequency was reduced from several seizures per day to less than one tonic-clonic seizure during sleep per month and only two episodes suggestive of partial complex seizures during daytime. These results have remained consistent over a 24-month postoperative follow-up. Functional neuroanatomy of hypothalamic connections involved in seizure propagation and aggressive behavior was reviewed.

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Acknowledgements

We thank Dr. Gary Gronich, Dr. Carmen L. Jorge, and Dr. Luis H. Castro for preoperative and postoperative assistance in neurophysiological investigation.

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Authors and Affiliations

  1. Departamento de Neurologia, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

    Antonio Nogueira de Almeida, Erich Talamoni Fonoff, Gerson Ballester, Manoel Jacobsen Teixeira & Raul Marino Jr

  2. Instituto Neurológico de São Paulo, Rua Maestro Cardim, 808 Liberdade, São Paulo, SP, 01323-001, Brazil

    Antonio Nogueira de Almeida

Authors
  1. Antonio Nogueira de Almeida
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  2. Erich Talamoni Fonoff
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  3. Gerson Ballester
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  4. Manoel Jacobsen Teixeira
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  5. Raul Marino Jr
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Correspondence to Antonio Nogueira de Almeida.

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Comments

Felix Rosenow, Marburg, Germany

Hypothalamic hamartomas (HHs) are a rare brain malformation comprising a tumor-like mass of neuronal and glial tissue in the suprasellar region, typically attached to the mamillary on one or both sides and extending antero-inferiorly from the tuber cinereum, with or without extension into the third ventricle above [1, 4]. Clinically, HH frequently present with medication-refractory epilepsy, precocious puberty (PP), and more or less severe behavioral and cognitive disturbances. HH presenting with epilepsy usually have an intraventricular component and sessile attachment to the hypothalamus, whereas those associated with PP alone are either pedunculated or do not distort the hypothalamus and third ventricle. The basis of the epilepsy seems to be intrinsic, and the electroclincal expression appears to be mediated by connection of the HH to adjacent structures [4, 6]. Due to the severe clinical manifestations, frequently, an urgent need for surgical treatment exists. On the other hand, the localization in the middle of the skull in tight connection with fornix, mamillary bodies, and the hypothalamus imply a high risk of surgical therapy with a significant proportion of patients suffering complications such as stroke, cranial neuropathy, or endocrine disturbance [8]. For these reasons, various surgical and nonsurgical treatment strategies have been applied and reported in case reports or small case series including subfrontal, transsylvian, transcallosal resection, open or endoscopic disconnections [2, 4], radiosurgery by gamma-knife [11], LINAC or interstitial iodine seed implantation [12]. Complete seizure control is only achieved in a minority of the patients treated by these different techniques. Radiosurgery appears to carry a relatively low complication rate but takes time to work. Furthermore, the risk of late effects of radiation in the mostly young patients is unknown. Considering the problems with other surgical approaches, the technique of stereotactic disconnection by radiofrequency ablation or thermocoagulation provides a minimally invasive approach which may carry lower risks than other techniques used. In this case report by de Almeida et al., the technique is clearly described, the pros and cons are discussed adequately, and the reader is left with the impression that this technique, so far described in single cases [3, 7, 9] and one recent series of five patients with marked reductions in seizure frequency obtained in all cases, three of which became seizure free [5], deserves evaluation in a larger multicenter study. A comparison with endoscopic disconnection as described by the group of Delalande [2, 10] would be of great interest.

References

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2. 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 (Tokyo) 43:61–68

3. Fukuda M, Kameyama S, Wachi M, Tanaka R (1999) Stereotaxy for hypothalamic hamartoma with intractable gelastic seizures: technical case report. Neurosugery 44:1347–1350

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7. Mullatti N, Selway R, Nashef L, Elwes R, Honavar M, Chandler C, Morris R, Jarosz J, Buchanan C, Polkey C (2003) The clinical spectrum of epilepsy in children and adults with hypothalamic hamartoma. Epilepsia 44:1310–1319

8. Palmini A, Chandler C, Andermann F, Costa da Costa J, Paglioli–Neto E, Polkey C, Rosenblatt B, Montes J, Martínez JV, Farmer JP, Sinclair B, Aronyk K, Paglioli E, Coutinho L, Raupp S, Portuguez M (2002) Resection of the lesion in patients with hypothalamic hamartomas and catastrophic epilepsy. Neurology 58:1338–1347

9. Parent AG (1999) Stereotactic radiofrequency ablation for the treatment of gelastic seizures associated with hypothalamic hamartoma. J Neurosurg 91:881–884

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11. Regis J, Bartolomei F, de Toffol B, Genton P, Kobayashi T, Mori T, Takakura K, Hori T, Inoue H, Schröttner O, Pendl G, Wolf A, Arita K, Chauvel P (2000) Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Neurosurgery 47:1343–1352

12. Schulze-Bonhage A, Homberg V, Trippel M, Keimer R, Elger CE, Warnke PC, Ostertag C (2004) Interstitial radiosurgery in the treatment of gelastic epilepsy due to hypothalamic hamartomas. Neurology 62:644–647

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de Almeida, A.N., Fonoff, E.T., Ballester, G. et al. Stereotactic disconnection of hypothalamic hamartoma to control seizure and behavior disturbance: case report and literature review. Neurosurg Rev 31, 343–349 (2008). https://doi.org/10.1007/s10143-008-0142-8

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