Abstract
Approximately 50% of patients with tuberous sclerosis complex (TSC) present intractable epilepsy, and surgery is an option for those patients. Hereby, we analyze long-term seizure control and neuropsychological outcomes of epilepsy surgery in patients with TSC. Clinical data were retrospectively collected from 66 patients with TSC and epilepsy followed up over 5 years, 51 of whom underwent epilepsy surgery between 2001 and 2011. Reductions in the number of seizures were analyzed at 1-year (1FU), 5-year (5FU), and 10-year (10FU) follow-ups visits after the operation. Influential factors on postoperative seizure free and intelligence quotient (IQ) and quality-of-life (QOL) outcomes were evaluated at 5FU. Resective procedures included 26 tuber resections, 15 lobectomies, and 10 tuber resections and lobectomies. Corpus callosotomies were performed as the adjunctive approach in 11 cases with low IQ. The percentages of seizure-free cases were 74.5% at 1FU, 58.8% at 5FU, and 47.8% at 10FU, and the predictive factor for long-term postoperative seizure freedom was the history of preoperative seizures and preoperative full-scale IQ. Significant improvements were found in performance IQ, full-scale IQ, and QOL in patients from the surgery group, particularly those who were seizure free after the operation. Our study showed that epilepsy surgery in TSC with epilepsy rendered improvements in seizure control, full-scale IQ, and QOL. Satisfactory long-term seizure control was often achieved with an early operation and without mental retardation, and improvements in QOL and IQ were frequently observed in postoperative patients who remained seizure free.
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Li H, Jin F, Jiang K et al (2016) mTORC1-mediated down regulation of COX2 restrains tumor growth caused by TSC2 deficiency. Oncotarget 7:28435–28447
Kingswood JC, Bruzzi P, Curatolo P et al (2014) TOSCA—first international registry to address knowledge gaps in the natural history and management of tuberous sclerosis complex. Orphanet J Rare Dis 9:182
Curatolo P, Maria BL (2013) Tuberous sclerosis. Handb Clin Neurol 111:323–331
Hunold A, Haueisen J, Ahtam B et al (2014) Localization of the epileptogenic foci in tuberous sclerosis complex: a pediatric case report. Front Hum Neurosci 8:175
Crino PB, Nathanson KL, Henske EP (2006) The tuberous sclerosis complex. N Engl J Med 355:1345–1356
Curatolo P, Bombardieri R, Jozwiak S (2008) Tuberous sclerosis. Lancet 372:657–668
Overwater IE, Bindels-de Heus K, Rietman AB et al (2015) Epilepsy in children with tuberous sclerosis complex: chance of remission and response to antiepileptic drugs. Epilepsia 56:1239–1245
Krueger D, Wilfong A, Holland-Bouley K et al (2013) Everolimus treatment of refractory epilepsy in tuberous sclerosis complex. Ann Neurol 74:679–687
Jansen FE, van Huffelen AC, Algra A et al (2007) Epilepsy surgery in tuberous sclerosis: a systematic review. Epilepsia 48:1477–1484
Zhang K, Hu WH, Zhang C et al (2013) Predictors of seizure freedom after surgical management of tuberous sclerosis complex: a systematic review and meta analysis. Epilepsy Res 105:377–383
Fallah A, Guyatt GH, Snead OC III et al (2014) Predictors of seizure outcomes in children with tuberous sclerosis complex and intractable epilepsy undergoing resective epilepsy surgery: an individual participant data meta-analysis. PLoS One 8:e53565
Liang S, Li A, Zhao M et al (2010) Epilepsy surgery in tuberous sclerosis complex: emphasis on surgical candidate and neuropsychology. Epilepsia 51:2316–2321
Shahid A (2013) Resecting the epileptogenic tuber: what happens in the long term? Epilepsia 54:135–138
Roach ES, Sparagana SP (2004) Diagnosis of tuberous sclerosis complex. J Child Neurol 19:643–649
Koh S, Jayakar P, Dunoyer C et al (2000) Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome. Epilepsia 41:1206–1213
Jansen FE, van Huffelen AC, Bourez-Swart M et al (2005) Consistent localization of interictal epileptiform activity on EEGs of patients with tuberous sclerosis complex. Epilepsia 46:415–419
Liang S, Wang S, Zhang J et al (2012) Long-term outcomes of epilepsy surgery in school-aged children with partial epilepsy. Pediatr Neurol 47:284–290
Bollo RJ, Kalhorn SP, Carlson C et al (2008) Epilepsy surgery and tuberous sclerosis complex: special considerations. Neurosurg Focus 25:13
Weiner HL, Carlson C, Ridgway EB et al (2006) Epilepsy surgery in young children with tuberous sclerosis: results of a novel approach. Pediatrics 117:1494–1502
Arya R, Tenney JR, Horn PS et al (2015) Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions. J Neurosurg Pediatr 15:26–33
Krsek P, Jahodova A, Kyncl M, Kudr M, Komarek V, Jezdik P, Jayakar P, Miller I, Korman B, Rey G, Resnick T, Duchowny M (2013) Predictors of seizure-free outcome after epilepsy surgery for pediatric tuberous sclerosis complex. Epilepsia 54(11):1913–1921
Song P, Joo EY, Seo DW et al (2012) Seizure localization in patients with multiple tubers: presurgical evaluation in tuberous sclerosis. J Epilepsy Res 2:16–20
Wu JY, Salamon N, Kirsch HE et al (2010) Noninvasive testing, early surgery, and seizure freedom in tuberous sclerosis complex. Neurology 74:392–398
Yogi A, Hirata Y, Karavaeva E et al (2015) DTI of tuber and perituberal tissue can predict epileptogenicity in tuberous sclerosis complex. Neurology 85:2011–2015
Abdijadid S, Mathern GW, Levine MS et al (2015) Basic mechanisms of epileptogenesis in pediatric cortical dysplasia CNS. Neurosci Ther 21:92–103
Cepeda C, Andre VM, Yamazaki I et al (2010) Comparative study of cellular and synaptic abnormalities in brain tissue samples from pediatric tuberous sclerosis complex and cortical dysplasia type II. Epilepsia 51:160–165
Mühlebner A, van Scheppingen J, Hulshof HM et al (2016) Novel histopathological patterns in cortical tubers of epilepsy surgery patients with tuberous sclerosis complex. PLoS One 11:e0157396
Ma TS, Elliott RE, Ruppe V et al (2012) Electrocorticographic evidence of perituberal cortex epileptogenicity in tuberous sclerosis complex clinical article. J Neurosurg Pediatrics 10:376–382
Ruppe V, Dilsiz P, Reiss CS et al (2014) Developmental brain abnormalities in tuberous sclerosis complex: a comparative tissue analysis of cortical tubers and perituberal cortex. Epilepsia 55:539–550
Oh S, Stewart T, Miller I et al (2011) In vivo optical properties of cortical tubers in children with tuberous sclerosis complex (TSC): a preliminary investigation. Epilepsia 52:1699–1704
Acknowledgements
The authors would like to show gratitude to the patients and their families for their long-term cooperation, Prof. J. F. Yang for MRI image interpretation, and Prof. Q. H. Ouyang for PET image interpretation. We also appreciate the contribution provided by the following people: Dr. C. Gao, and Dr. H. B. Kong from Neurosurgery Department, and Dr. S. S. Liang, Mrs. X. M. Yu, and all technicians in the Neurophysiologic Laboratory of Capital Epilepsy Therapy Center.
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Liang, S., Zhang, J., Yang, Z. et al. Long-term outcomes of epilepsy surgery in tuberous sclerosis complex. J Neurol 264, 1146–1154 (2017). https://doi.org/10.1007/s00415-017-8507-y
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DOI: https://doi.org/10.1007/s00415-017-8507-y