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Epileptogenicity in tuberous sclerosis complex: A stereoelectroencephalographic study.

Andrew Neal1,2,3, Karine Ostrowsky-Coste2,4, Julien Jung1,2

  • 1National Institute of Health and Medical Research U1028/National Center for Scientific Research, Mixed Unit of Research 5292, Lyon Neuroscience Research Center, Lyon, France.

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Stereo-EEG reveals distinct epileptogenic zone organizations in tuberous sclerosis complex (TSC) epilepsy. Identifying focal tubers improves surgical outcomes for drug-resistant epilepsy in TSC patients.

Keywords:
electroclinical syndromesepilepsy surgeryepileptogenic zonestereo-EEGtuberous sclerosis complex

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Area of Science:

  • Neuroscience
  • Epileptology
  • Medical Imaging

Background:

  • Drug-resistant epilepsy is a significant challenge in tuberous sclerosis complex (TSC).
  • Optimal invasive electroencephalography (EEG) and surgical strategies for TSC-associated epilepsy remain unclear.
  • Stereo-EEG (SEEG) offers detailed insights into brain activity.

Purpose of the Study:

  • To investigate the role of SEEG in TSC-associated drug-resistant epilepsy.
  • To analyze tuber and surrounding cortex epileptogenicity using SEEG data.
  • To differentiate epileptogenic zone (EZ) organization patterns in TSC.

Main Methods:

  • Analysis of SEEG data from 18 patients with TSC, including 110 seizures.
  • Calculation of epileptogenicity index (EI) for defined regions of interest (ROIs): dominant tuber, perituber cortex, secondary tuber, nearby cortex, and distant cortex.
  • Classification of EZ organization into focal tuber (EZ confined to dominant tuber) and complex patterns.

Main Results:

  • The dominant tuber was the most epileptogenic region (highest EI).
  • Seven patients exhibited a focal tuber EZ organization with 80% Engel IA postsurgical outcome.
  • Complex EZ organization, with nearby cortex as most epileptogenic, resulted in 40% Engel IA outcome.
  • Specific tuber characteristics (FLAIR hypointense center, continuous IEDs, stimulation-induced seizures) predicted focal EZ organization with 98% specificity.

Conclusions:

  • Distinct EZ organizations exist in TSC-associated epilepsy, with focal tuber EZs yielding better surgical outcomes.
  • Focal tubers with specific characteristics resemble type II focal cortical dysplasia.
  • Understanding EZ organization is crucial for guiding SEEG approach and resection strategies in TSC epilepsy.