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Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas

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Gamma Knife Radiosurgery

Part of the book series: Acta Neurochirurgica Supplements ((NEUROCHIRURGICA,volume 91))

Summary

Objective. Drug resistant epilepsy associated with hypothalamic hamartoma (HH) can be cured by microsurgical resection of the lesion. Morbidity and mortality risks of microsurgery in this area are significant. Gamma Knife Surgery’s (GKS) reduced invasivity seems to be well adapted. In view of the severity of the disease and risks of surgical resection it is crucial to evaluate GKS for this indication. A first retrospective study has shown a very good safety and efficacy level but for a more reliable evaluation a prospective study would be required.

Methods. Between Oct 1999 and July 2002, 30 patients with HH and associated severe epilepsy were included. Seizure semiology (video EEG ) and frequency, behavioural disturbances, neuro-psychological performance, endocrinological status. sleep electroclinical abnormalities, MR imaging, and visual function were systematically evaluated before and after GKS (6, 12, 18, 24, 36 months). Twenty patients had experienced precocious puberty at a median age of 3.7 (0–9). Range of maximum diameter was from 7.5 to 23 mm with only 3 larger than 18 mm. The median marginal dose was 17 gy (14–20).

Results. Sufficient follow up for final evaluation is not yet available. Only 6 patients have a follow-up of more than 12 months and 19 more than 6 months. However a lot of very dramatic changes did occur during that period in this group. Among the 19 patients with more than 6 month s of follow-up, a lot had already experienced an increase of gelastic seizures around 3 months (3), an improvement in their seizure rate (18), behaviour (9), sleep (3), and EEG background activity (3), a cessation of part ial complex seizures (7). No complications have occurred till now except one patient experiencing at 5 months a hyperthermi a without infection and concomitant increase of gelastic seizures both ceasing suddenly and spontaneously after 15 days.

Conclusion. Our first results indicate that GKS is as effective as microsurgical resection and very much safer. GKS also allows to avoid the vascular risk related to radiofrequency lesioning or stimulation. The disadvantage of radiosurgery is its delayed action. Longer follow-up is mandatory for a serious evaluation of the role of GKS. Results are faster and more complete in patients with smaller lesions inside the 3rd ventricle (grade II). The early effect on subclinical discharges turns out to play a major role in the dramatic improvement of sleep quality, behaviour, developmental accelerat ion at school.

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References

  1. Albright A, Lee P (1993) Neurosurgical treatment of hypothalamic hamartomas causing precocious puberty [published erratum appeared in J Neurosurg 1993 Jul;79(1):156]. J Neurosurg 78: 77–82

    Article  PubMed  CAS  Google Scholar 

  2. Albright A, Lee P (1998) Surgery for hypothalamic hamartomas [letter]. J Neurosurg 88: 353

    PubMed  CAS  Google Scholar 

  3. 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

    Article  PubMed  CAS  Google Scholar 

  4. Arita K, Kurisu K. Iida K et al (1998) Subsidence of seizure induced by stereotactic radiation in a patient with hypothalamic hamartoma. Case report. J Neurosurg 89: 645–648

    Article  PubMed  CAS  Google Scholar 

  5. Arroyo S. Santamaria J. Sanmarti F et al (1997) Ictal laughter associated with paroxysmal hypothalamopituitary dysfunction. Epilepsia 38: 114–117

    Article  PubMed  CAS  Google Scholar 

  6. Beningfield S. Bonnici F. Cremin B (1988) Magnetic resonance imaging of hypothalamic hamartomas. Br J Radiol 61: 1177–1180

    Article  PubMed  CAS  Google Scholar 

  7. Berkovic S. Kuzniecky R. Andermann F (1997) Human epileptogenesis and hypothalamic hamartomas: new lessons from an experiment of nature [editorial]. Epilepsia 38: 1–3

    Article  PubMed  CAS  Google Scholar 

  8. Berkovic SF. Andermann F. Melanson D et al (1988) Hypothalamic hamartoma and associated ictal laughter. Evolution of a characteristic epileptic syndrome and diagnostic value of magnetic resonance imaging. Ann Neurol 23: 429–439

    Article  PubMed  CAS  Google Scholar 

  9. Brada M. Ford D. Ashley S et al (1992) Risk of second brain tumor after conservative radiotherapy for pituitary adenoma. Br Med J 304: 1343–1346

    Article  CAS  Google Scholar 

  10. Cascino GD, Andermann F, Berkovic SF et al (1993) Gelastic seizures and hypothalamic hamartomas: evaluation of patients undergoing chronic intracranial EEG monitoring and outcome of surgical treatment. Neurology 43: 747–750

    Article  PubMed  CAS  Google Scholar 

  11. Cerullo A, Tinuper P, Provini F et al (1998) Autonomic and hormonal ictal changes in gelastic seizures from hypothalamic hamartomas. Electroencephalogr Clin Neurophysiol 107: 317–322

    Article  PubMed  CAS  Google Scholar 

  12. Cheng K. Sawarnura Y. Yamauchi T et al (1993) Asymptomatic large hypoth alamic hamartoma associated with polydactyly in an adult. Neuro surgery 32: 458–460: discussion 460

    CAS  Google Scholar 

  13. Commentz JC, Helmke K (1995) Precocious puberty and decreased melatonin secretion due to a hypothalamic hamartoma. Horm Res 44: 271–275

    Article  PubMed  CAS  Google Scholar 

  14. Culler FL, James HE, Simon ML et al (1985) Identification of gonadotropin-releasing hormone in neurons of a hypothalamic hamartoma in a boy with precocious puberty. Neurosurgery 17: 408–412

    Google Scholar 

  15. Debeneix C, Bourgeois M, Trivin C et al (2001) Hypothalamic hamartoma: comparison of clinical presentation and magnetic resonance images. Horm Res 56: 12–18

    Article  PubMed  CAS  Google Scholar 

  16. Degerblad M, Rähn T. Bergstrand G et al (1986) Long-term results of stereotactic radiosurgery to the pituit ary gland in Cushing’s disease. Acta Endocrinol 112: 310–314

    PubMed  CAS  Google Scholar 

  17. Delalande O, Fohlen M, Jalin C et al (1998) Surgical treatment of Epilepsy due to Hypothalamic hamartoma: Technique and preliminary results in five cases. Epilepsia 39: 90–91

    Google Scholar 

  18. Deonna T, Ziegler AL (2000) Hypothalamic hamartoma, precocious puberty and gelastic seizures: a special model of ‘epileptic’ developmental disorder. Epileptic Disord 2: 33–37

    PubMed  CAS  Google Scholar 

  19. DiFazio MP, Davis RG (2000) Utility of early single photon emission computed tomography (SPECT) in neonatal gelastic epilepsy associated with hypothalamic hamartoma. J Child Neurol 15: 414–417

    CAS  Google Scholar 

  20. Donley D, Kuzniecky R, Mountz J et al (1994) Ictal SPECT findings in hypothalamic hamartoma and epilepsy (Abstract). Epilepsia 35 [Suppl]8: 146

    Google Scholar 

  21. Dunoyer C, Ragheb J. Resnick T et al (2002) The usc of stereotactic radiosurgery to treat intractable childhood partial epilepsy. Epilepsia 43: 292–300

    Article  PubMed  Google Scholar 

  22. Flickinger J, Kondziolka D, Lunsford L (1998) Clinical applications of stereotactic radiosurgery. Cancer Treat Res 93: 283–297

    Article  PubMed  CAS  Google Scholar 

  23. Flickinger JC, Kondziolka D, Lunsford LD (1998) Radiosurgery management of brain metastasis from systemic cancer. In: Lunsford LD, Kondziolka D, Flickinger JC (eds) Progress in neurological surgery: gamma knife brain surgery. Karger, Basel. pp 145–159

    Chapter  Google Scholar 

  24. Frattali CM, Liow K, Craig GH et al (2001) Cognitive deficits in children with gelastic seizures and hypothalamic hamartoma. Neurology 57: 43–46

    Article  PubMed  CAS  Google Scholar 

  25. Grob J, Regis J, Laurans R et al (1998) Rad iosurgery without whole brain radiotherapy in melanom a brain metastases. Eur J Cancer 34: 1187–1192

    Article  PubMed  CAS  Google Scholar 

  26. Harada K, Yoshida J, Wakabayashi T et al (1995) A super longacting LH-RH analogue induces regression of hypothalamic hamartoma associated with precocious puberty. Acta Neurochir (Wien) 137: 102–105

    Article  CAS  Google Scholar 

  27. Hayashi M, Bartolomei F, Rey M et al (2002) MR changes after gamma knife radiosurgery for mesial temporal lobe epilepsy: an evidence for the efficacy of subnecrotic doses. In: Kondziolka D (ed) Radiosurgery. Karger, Basel. pp 192–202

    Chapter  Google Scholar 

  28. Kahane P, Munari C, Minotti L et al (1997) Role of the hypothalamic hamart oma in the genesis of gelastic and dacrystic seizures. In: Tuxhorn I, Holthausen H, Boenigk H (eds) Pediatric epilepsy syndromes and their surgical treatment. John Libbey, London. pp 447–461

    Google Scholar 

  29. Kahane P, Tassi L, Hoffmann D et al (1994) Crises dacrystiques et harna rtome hypoth alamique: à propos d’un e observation vidéo-stéréo-EEG. Epilepsies 6: 259–279

    Google Scholar 

  30. Kondziolka D, Lunsford L, McLaughlin M et al (1998) Long-term outcomes after rad iosurgery for acou stic neuromas [see comments]. N Engl J Med 339: 1426–1433

    Article  PubMed  CAS  Google Scholar 

  31. Kondziolka D. Niranjan A. Lunsford L et al (1999) Stereotactic radiosurgery for meningiomas. Neurosurg Clin N Am 10: 317–325

    PubMed  CAS  Google Scholar 

  32. Kuzniecky R, Guthrie B, Mountz J et al (1997) Intrinsic epileptogenesis of hypothalamic hamartomas in gelastic epilepsy. Ann Neurol 42: 60–67

    Article  PubMed  CAS  Google Scholar 

  33. Kuzniecky R, Guthrie B, Mountz J et al (1995) Hypothalamic hamartomas and gelastc seizures: evidence for subcortical seizure generation by ictal SPECT and cerebral stimulation. Epilepsia 36 [Suppl] 3: 266

    Article  Google Scholar 

  34. Kuzniecky R, Mountz J, Wheatley G et al (1993) Ictal Single Photon emission computed tomography demonstrates localized epileptogenesis in cort icaldysplasia. Ann Neurol 34: 627–631

    Article  PubMed  CAS  Google Scholar 

  35. Kyuma Y, Kato E, Sekido K et al (1985) Hypothalamic hamartoma successfully treated by operation. Case report. J Neurosurg 62: 288–290

    Article  PubMed  CAS  Google Scholar 

  36. Laws E, Vance M (1999) Radio surgery for pituitary tumors and craniopharyngiomas. Neuro surg Clin N Am 10: 327–336

    Google Scholar 

  37. Leksell L (1951) The stereotaxic method and radiosurgery of the brain. Acta Chir Scand 102: 316–319

    PubMed  CAS  Google Scholar 

  38. Leksell L (1971) Stereotaxis and radiosurgery. An operative system. In: Thomas CC (ed) Springfield

    Google Scholar 

  39. Lunsford LD (1990) Stereotactic radiosurgery of intracranial arteriovenous malformations. Neurosurgery update. Mc GrawHill, New York. pp 175–185

    Google Scholar 

  40. Machado HR, Hoffmann HJ, Hwang PA (1991) Gelastic seizures treated by resection of a hypothal amic hamartoma. Child’s Nervous System 7: 462–465

    Article  PubMed  CAS  Google Scholar 

  41. Mattia O, Olivier A, Avoli M (1995) Seizure-like discharges recorded in human dysplastic neocortex maint ained in vitro. Neurology 45: 1391–1395

    Article  PubMed  CAS  Google Scholar 

  42. Mori Y, Kondziolka D, Flickinger J et al (1998) Stereotactic radiosurgery for brain metastasis from renal cell carcinoma [see comments]. Cancer 83: 344–353

    Article  PubMed  CAS  Google Scholar 

  43. Munari C, Kahane P, Francione S et al (1995) Role of the hypothalamic hamartoma in the genesis of gelastic fits (a videostereo-EEG study). Electroencephalogr Clin Neurophysiol 95: 154–160

    Article  PubMed  CAS  Google Scholar 

  44. Murphy JV, Wheless JW, Schmoll CM (2000) Left vagal nerve stimulation in six patients with hypothalamic hamartomas. Pediatr Neurol 23: 167–168

    Article  PubMed  CAS  Google Scholar 

  45. Nishio S, Fujiwara S, Aiko Y et al (1989) Hypothalamic hamartoma. Report of two cases. J Neurosurg 70: 640–645

    Article  PubMed  CAS  Google Scholar 

  46. Nishio S, Morioka T, Fukui M et al (1994) Surgical treatment of intractable seizures due to hypothalamic hamartoma. Epilepsia 35: 514–519

    Article  PubMed  CAS  Google Scholar 

  47. Nishio S, Shigeto H, Fukui M (1993) Hypothalami c hamartoma: the role of surgery. Neurosurg Rev 16: 157–160

    Article  PubMed  CAS  Google Scholar 

  48. Noren G (1996) Gamma knife radiosurgery for acoustic neurinomas. In: Gildenberg PL Tasker RR (eds) Textbook of stereotactic and functional neurosurgery. Mc Graw-Hill, New York. pp 835–844

    Google Scholar 

  49. Paillas JE, Roger G, Toga M et al (1969) Hamarthome de l’hypothalamus. Rev Neurol 120: 177–194

    PubMed  CAS  Google Scholar 

  50. Pallini R, Bozzini V, Colicchio G et al (1993) Callosotomy for generalized seizures associated with hypothalamic hamartoma. Neurol Res 15: 139–141

    PubMed  CAS  Google Scholar 

  51. Palmini A, Andermann F, Olivier A (1991) Focal neuronal migration disorders and intractable par tial epilepsy: results of surgical treatment. Ann Neurol 30: 750–757

    Article  PubMed  CAS  Google Scholar 

  52. Palmini A, Chandler C, Andermann F et al (2002) Resection of the lesion in patien ts with hypothalamic hamartomas and catastrophic epilepsy. Neurology 58: 1338–1347

    Article  PubMed  CAS  Google Scholar 

  53. Palmini A. Gambardella A. Andermann F (1995) Intrinsic epileptogenicity of human dysplastic cortex as suggested by corticography and surgical results. Ann Neurol 37: 476–487

    Article  PubMed  CAS  Google Scholar 

  54. Parrent AG (1999) Stereotacti c radiofrequency ablation for the treatment of gelastic seizures associated with hypothalamic hamartoma. Case report. J Neurosurg 91: 881–884

    Article  PubMed  CAS  Google Scholar 

  55. Pascual-Castroviejo I, Moneo JH, Viano J et al (2000) [Hypothalamic hamartomas: cont rol of seizures after part ial removal in one case]. Rev Neurol 31: 119–122

    PubMed  CAS  Google Scholar 

  56. Plouin P, Ponsot G. Dulac O et al (1983) Hamartomes hypothalamiques et crises de rire. Rev. EEG Neurophysiol 13: 312–316

    CAS  Google Scholar 

  57. Pollock BE, Kondziolka D, Lunsford LD el al (1994) Stereotactic radiosurgery for pituitary adenomas: imaging. visual and endocrine results. Acta Neurochir (Wien) 62: 33–38

    Article  CAS  Google Scholar 

  58. Prasad D, Steiner M, Steiner L (2000) Gamma surgery for vestibular schwannoma [see comments]. J Neurosurg 92: 745–759

    Article  PubMed  CAS  Google Scholar 

  59. Regis J, Bartolomei F, de Toffol B et al (2000) Gamma knife surgery for epilepsy related to hypothalamic hamartomas. Neurosurgery 47: 1343–1351; discussion 1351-1342

    Article  PubMed  CAS  Google Scholar 

  60. Regis J, Bartolomei F, Hayashi M et al (2000) The role of gamma knife surgery in the treatm ent of severe epilepsies. Epileptic Disord 2: 113–122

    PubMed  CAS  Google Scholar 

  61. Régis J, Bartolomei F, Kida Y et al (2000) Radiosurgery of Epilepsy Associated with Caverno us Malformati on: Retrospective study in 49 patients. Neurosurgery 47: 1091–1097

    Article  PubMed  Google Scholar 

  62. Regis J, Bartolomei F, Metellus P et al (1999) Radiosurgery for trigeminal neuralgia and epilepsy. Neurosurg Clin N Am 10: 359–377

    PubMed  CAS  Google Scholar 

  63. Régis J, Peragut J, Rey M et al (1994) First selective amygdalehippocampic radiosurgery for mesial temp oral lobe epilepsy. Stereo tact and Functional Neurosurg 64[Suppl] 1: 191–201

    Google Scholar 

  64. Régis J, Peragut JC, Rey M et al (1994) First selective amygda lohippocampic radiosurgery for mesial temporal lobe epilepsy. Stereotact Funct Neuro surg 64: 191–201

    Google Scholar 

  65. Regis J, Semah F, Bryan R et al (1999) Early and delayed MR and PET changes after selective lemporomesial radiosurgery in mesial temporal lobe epilepsy. AJNR Am J Neuroradiol 20: 213–216

    PubMed  CAS  Google Scholar 

  66. Roche PH, Regis J, Dufour H et al (2000) Gamm a knife Radiosur gery in the man agement of caverno us sinus men ingiomas. J Neurosurg 93: 68–73

    Article  PubMed  Google Scholar 

  67. Rosenfeld JV, Harvey AS, Wrennall J et al (2001) Transcallosal resection of hypoth alamic hamartomas, with control of seizures, in children with gelastic epilepsy. Neuro surgery 48: 108–11 8

    CAS  Google Scholar 

  68. Rutigliano MJ, Lunsford LD, Kondziolka D et al (1995) Th e cost effectiveness of stereotactic radiosurgery versus surgical resection in the treatment of solitary meta static brain tumors. Neurosurgery 37: 445–455

    Article  PubMed  CAS  Google Scholar 

  69. Sato M, Ushio Y, Arita N et al (1985) Hypothalamic hamartoma: report of two cases. Neurosurgery 16: 198–206

    Article  PubMed  CAS  Google Scholar 

  70. Simmons NE, Laws E (1998) Gliomas Occurence after sellar Irradiation: case report and review. Neurosurgery 42: 172–178

    Article  PubMed  CAS  Google Scholar 

  71. Steiner L, Lindquist C, Adler J et al ( 1992) Clinical outcome of radiosurgery for cerebral arte riovenous malformations. J Neurosurg 77: 1–8

    Article  PubMed  CAS  Google Scholar 

  72. Stewart L, Steinbok P, Daaboul J (1998) Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty. Report of six cases. J Neurosurg 88: 340–345

    Article  PubMed  CAS  Google Scholar 

  73. Tasch E, Cendes F, Li LM et al (1998) Hypothalamic hamar tomas and gclastic epilepsy: a spectroscopic study. Neurology 51: 1046–1050

    Article  PubMed  CAS  Google Scholar 

  74. Tassi L, Quarato P, Francione S (1995) Chronological relationships between ictal laughing and smiling and intralesional discharges: stereo-EEG study of a patient with gelastic epilepsy and hypothalamic hamartoma. Epilepsia 36: 239–240

    Google Scholar 

  75. Tassinari CA, Riguzzi P, Rizzi R et al (1997) Gelastic seizures. In: Tuxhorn I, Holthausen H, Boenigk H (eds) Current problems in epilepsy. John Libbey, London. pp 429–446

    Google Scholar 

  76. Turjman F, Xavier J, Froment J et al (1996) Late MR follow-up of hypothalamic hamartomas. Childs Nerv Syst 12: 63–68

    Article  PubMed  CAS  Google Scholar 

  77. Unger F, Schrottner O, Haselsberger K et al (2000) Gamma knife radiosurgery for hypothalamic hamartom as in patients with medically intractable epilepsy and precocious puberty. Report of two cases. J Neurosurg 92: 726–731

    Article  PubMed  CAS  Google Scholar 

  78. Valdueza J, Cristante L, Dammann O et al (1994) Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery. Neurosurgery 34: 949–958; discussion 958

    Article  PubMed  CAS  Google Scholar 

  79. Watanabe T, Enomoto T, Uemura K et al (1998) [Gelastic seizures treated by partial resection of a hypothalamic hamartoma]. No Shinkei Geka 26: 923–928

    PubMed  CAS  Google Scholar 

  80. Weissenberger AA, Dell ML, Liow K et al (2001) Aggression and psychiatric comorbidity in children with hypothalamic hamartomas and their unaffected siblings. J Am Acad Child Adolesc Psychiatry 40: 696–703

    Article  PubMed  CAS  Google Scholar 

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Authors and Affiliations

  1. Stereotactic and Functional Neurosurgery Department, Timone Hospital, Marseilles, France

    Dr. J. Régis, M. Hayashi & L. P. Eupierre

  2. Endocrinology Department, Timone Hospital, Marseilles, France

    N. Villeneuve, F. Bartolomei, T. Brue & P. Chauvel

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  1. Dr. J. Régis
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Correspondence to J. Régis .

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Editors and Affiliations

  1. German Gamma Knife Centre, Munich, Germany

    B. Wowra  & A. Muacevic  & 

  2. Neurochirurgische Klinik und Poliklinik, Klinikum Großhadern, Munich, Germany

    J.-C. Tonn

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Régis, J. et al. (2004). Gamma Knife Surgery for Epilepsy Related to Hypothalamic Hamartomas. In: Wowra, B., Muacevic, A., Tonn, JC. (eds) Gamma Knife Radiosurgery. Acta Neurochirurgica Supplements, vol 91. Springer, Vienna. https://doi.org/10.1007/978-3-7091-0583-2_4

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