Journal article

Resection of tuber centers only for seizure control in tuberous sclerosis complex

Sarah EM Stephenson, Wirginia J Maixner, Sarah M Barton, Colleen D'Arcy, Simone A Mandelstam, Duncan MacGregor, Paul J Lockhart, Richard J Leventer, A Simon Harvey

Epilepsy Research | ELSEVIER | Published : 2021

Abstract

Our previous studies suggest the tuber center is the seizure focus in tuberous sclerosis complex (TSC). We report findings from 5 epilepsy surgeries in 4 children with TSC and focal motor seizures from single tubers in primary sensorimotor cortex in which resection was limited to the cortex in the tuber center. Intraoperative electrocorticography showed epileptiform activity in the tuber center, with or without propagation to the tuber rim and surrounding perituberal cortex. Histopathology showed an abundance of dysmorphic neurons in the tuber center compared to the rim in four paired specimens, dysmorphic neurons being the reported epileptogenic cell line in TSC. Associated focal motor seiz..

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Grants

Awarded by Australian Government National Health and Medical Research Council


Funding Acknowledgements

This work was supported by the Australian Government National Health and Medical Research CouncilGNT1128933, the Murdoch Children's Research Institute, the Campbell Edwards Trust and the Brain Foundation. Additional funding was provided by the Independent Research Institute Infrastructure Support Scheme and the Victorian State Government Operational Infrastructure Program. RJL is supported by the Melbourne Children's Clinician Scientist Fellowship scheme. PJL was supported in part by the Vincent Chiodo Charitable Trust.