Abstract
Aim
The aim of the study was to evaluate the surgical treatment of epilepsy and detection of possible early surgery predictive elements in patients with tuberous sclerosis complex (TSC).
Materials and methods
Forty-two TSC patients with epilepsy were selected and divided into two main groups: definite and fruste forms. Definite forms were divided into different groups: patients with pharmacologically controlled epilepsy, patients with pharmacoresistant epilepsy excluded from surgery after an extensive presurgical assessment, and patients with a pharmacoresistant epilepsy who underwent surgery. We compared the definite TSC groups to identify elements that predict surgical candidacy. Second, we compared all operated patients to assess surgical outcome.
Conclusion
We found several factors that could predict a surgical intervention even if identification of patients with refractory epilepsy who can benefit from surgery is an evolving process. Also, several positive factors for good surgical outcome were identified. Patients with the fruste form had excellent surgical outcome.
Similar content being viewed by others
References
Palmini A, Andermann F, Olivier A, Tampieri D, Robitaille Y, Andermann E, Wright G (1991) Focal neuronal migration disorders and intractable epilepsy: a study of 30 patients. Ann Neurol 3:741–749
Francione S, Kahane P, Tassi L, Munari C (1998) La chirurgie de l’épilepsie dans la sclérose tubéreuse de Bourneville. In: Bureau M, Kahane P, Munari C (eds) Epilepsies partielles graves pharmaco-résistantes e l’enfant: stratégies diagnostiques et traitements chirurgicaux. John Libbey eurotext, pp 278–295
Roach ES, Gomez MR, Northup H (1998) Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria. J Child Neurol 13:624–628
Jozwiak S, Schwartz RA, Janniger CK, Bielicka-Cymerman J (2000) Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients. J Child Neurol 15:652–659
Houser OW, Gomez MR (1992) CT and MR imaging of intracranial tuberous sclerosis. J Dermatol 19(11):904–908
Holmes GL, Stafstrom CE, Tuberous Sclerosis Study Group (2007) Tuberous sclerosis complex and epilepsy: recent developments and future challenges. Epilepsia 48(4):617–630
Curatolo P, Bombardieri R, Verdecchia M, Seri S (2005) Intractable seizures in tuberous sclerosis complex: from molecular pathogenesis to the rational for treatment. J Child Neurol 20:318–325
Curatolo P, Bombardieri R, Cerminara C (2006) Current management for epilepsy in tuberous sclerosis complex. Curr Opin Neurol 19:119–123
Weiner HL, Carlson C, Ridgway EB, Zaroff CM, Miles D, LaJoie J, Devinsky O (2006) Epilepsy surgery in young children with tuberous sclerosis: results of a novel approach. Pediatrics 117:1494–1502
Engel JJ, Van Ness PC, Rasmussen T, Ojemann LM (1993) Outcome with respect to epileptic seizures. In: Engel JJ (ed) Surgical treatment of the epilepsies. 2nd edn. Raven, New York, pp 609–621
Wechsler D (1998) WISC-R: Wechsler intelligence scale for children (Italian Version). Giunti Organizzazioni Speciali, Florence
Wechsler D (1997) WAIS-R: Wechsler adult intelligence scale (Italian Version). Giunti Organizzazioni Speciali, Florence
Griffiths R (1984) Griffiths mental development scales for testing babies and young children from birth to eight years of age (Italian Version). Giunti Organizzazioni Speciali, Florence
Schopler E, Reichler RJ, Renner BR (1988) The Childhood Autism Rating Scale (CARS). Western Psychological Services, Los Angeles
Conners KC (1995) Continuous performance test user’s manual. Multi-Health Systems, Toronto
Achenbach TM (1991a) Manual for the child behavior checklist/4-18. University of Vermont Department of Psychiatry, Burlington
Yagishita A, Arai N (1999) Cortical tubers without other stigmata of tuberous sclerosis: imaging and pathological findings. Neuroradiology 41:428–432
Curatolo P, Seri S, Verdecchia M, Bombardieri R (2001) Infantile spasms in tuberous sclerosis complex. Brain Dev 173:502–507
Shepherd CW, Houser OW, Gomez MR (1995) MR findings in tuberous sclerosis complex and correlation with seizure development and mental impairment. AJNR Am J Neuroradiol 16(1):149–155
Jambaque I (1994) Neuropsychological aspects. In: Dulac O, Chugani HT, Dalla Bernardina B (eds) Infantile spasms and West Syndrome. Saunders, London, pp 82–87
O’Callaghan FJK, Harris T, Joinson C, Bolton P, Noakes M, Presdee D, Renowden S, Shiell A, Martyn CN, Osborne JP (2004) The relation of infantile spasms, tubers and intelligence in tuberous sclerosis complex. Arch Dis Child 89(6):530–533
Humphrey A, Neville BGR, Clarke A, Bolton PF (2006) Autistic regression associated with seizure onset in an infant with tuberous sclerosis. Dev Med Child Neurol 48:609–611
Wieshmann UC, Woermann FG, Lemieux L, Free SL, Bartlett PA, Smith SJ, Duncan JS, Stevens JM, Shorvon SD (1997) Development of hippocampal atrophy: a serial magnetic resonance imaging study in a patient who developed epilepsy. Epilepsia 38(11):1238–1241
Madhavan D, Schaffer S, Yankovsky A, Arzimanoglou A, Renaldo F, Zaroff CM, LaJoie J, Weiner HL, Andermann E, Franz SN, Leonard J, Connolly M, Cascino GD, Devinsky O (2007) Surgical outcome in tuberous sclerosis complex: a multicenter study. Epilepsia 48(8):1625–1628
Jarrar RG, Buchhalter JR, Raffael C (2004) Long-term outcome of epilepsy surgery in patients with tuberous sclerosis. Neurology 62:479–481
Jansen FE, van Huffelen AC, Bourez-Swart M, van Nieuwnhuizen O (2005) Consistent localization of interictal epileptiform activity on EEGs of patients with tuberous sclerosis complex. Epilepsia 46(3):415–419
Jambaqué I, Cusmai R, Curatolo P (1991) Neuropsychological aspects of tuberous sclerosis in relation to epilepsy and MRI findings. Dev Med Child Neurol 33(8):698–705
Goodman M, Lamm SH, Engel A, Shepherd CW, Houser OW, Gomez MR (1996) Cortical tuber count: a biomarker indicating neurological severity of tuberous sclerosis complex. J Child Neurology 12:85–90
Doherty C, Goh S, Poussaint TJ, Erdag N, Thiele A (2005) Prognostic significance of tuber count and location in tuberous sclerosis complex. Neurology 20:837–841
Bebin EM, Kelly PJ, Gomez MR (1993) Surgical treatment for epilepsy in cerebral tuberous sclerosis. Epilepsia 34(4):651–657
Avellino AM, Berger MS, Rostomily RC, Shaw CM, Ojemann A (1997) Surgical management and seizure outcome in patients with tuberous sclerosis. J Neurosurg 87:391–396
Guerreiro MM, Andermann F, Andermann E, Palmini A, Hwang P, Hoffman HJ, Otsubo H, Bastos A, Dubeau F, Snipes GJ, Olivier A, Rasmussen T (1998) Surgical treatment of epilepsy in tuberous sclerosis: strategies and results in 18 patients. Neurology 51:1263–1269
Karenfort M, Kruse B, Freitag H, Pannek H, Tuxhorn I (2002) Epilepsy surgery outcome in children with focal epilepsy due to tuberous sclerosis complex. Neuropediatrics 33:255–261
Renaldo F, Kahane P, Bourgeois M, Viviana P, Chiron C, Landré E, Chassoux F, Motte J, Echenne B, Arzimanoglou A (2005) Tuberous sclerosis complex: results of surgery. Epilepsia 46(Suppl. 6):82
Koh S, Jayakar P, Dunoyer C, Whiting SE, Resnik TJ, Alvarez LA, Morrison G, Ragheb J, Prats A, Dean P, Gilman J, Duchowny MS (2000) Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome. Epilepsia 41(9):1206–1213
Palmini A, Gambardella A, Andermann F, Dubeau F, Costa da Costa J, Olivier A, Tampieri D, Robitaille Y, Paglioli E, Paglioli Neto E, Coutinho L, Kim H (1994) Operative strategies for patients with cortical dysplastic lesions and intractable epilepsy. Epilepsia 35(Suppl. 6):S57–S71
Baumgartner JE, Wheless JW, Kulkarni S, Northrup H, Au KS, Smith A, Brookshire B (1997) On the surgical treatment of refractory epilepsy in tuberous sclerosis complex. Pediatr Neurosurg 27:311–318
Thiele EA, Duffy FH, Poussaint TY (2001) Intractable epilepsy and TSC: the role of epilepsy surgery in the pediatric population (abstract). J Child Neurol 16:681
Vigliano P, Canavese C, Bobba B, Genitori L, Papalia F, Padovan S, Forni M (2002) Transmantle dysplasia in tuberous sclerosis: clinical features and surgical outcome in four children. J Child Neurol 17:752–758
Jansen FE, Van Huffelen AC, Van Rijen PC, Leijten FSS, Jennekens-Schinkel A, Gosselaar P, Van Nieuwenhuizen O (2007) Epilepsy surgery in tuberous sclerosis: the Dutch experience. Seizure 16:445–453
Lucarelli P, Palmimiello S, Bottini N (2003) Association study of the autistic disorder and chromosome 16p. Am J Hum Genet 119A:242–246
Zaroff CM, Barr WB, Carlson C, LaJoie J, Madhavan D, Miles DK, Nass R, Devinsky O (2006) Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex. Seizure 15:558–562
Hartman AL, Kossoff EH (2006) Epilepsy surgery for the neurocutaneous disorders. Semin Pediatr Neurol 13:63–67
Madhavan D, Weiner HL, Carlson C, Devinsky O, Kuzniecky R (2007) Local epileptogenic networks in tuberous sclerosis complex: a case review. Epilepsy Behav 11:140–146
Author information
Authors and Affiliations
Corresponding author
Appendix
Appendix
Rights and permissions
About this article
Cite this article
Teutonico, F., Mai, R., Devinsky, O. et al. Epilepsy surgery in tuberous sclerosis complex: early predictive elements and outcome. Childs Nerv Syst 24, 1437–1445 (2008). https://doi.org/10.1007/s00381-008-0679-4
Received:
Revised:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00381-008-0679-4