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Inside the LGS Brain


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Brenda Porter, MD, PhD, offers information about what happens inside the LGS brain

Published in: Health & Medicine
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Inside the LGS Brain

  1. 1. Inside the LGS Brain Brenda Porter MD, PhD
  2. 2. LEFT RIGHT NOT Seizure Background
  3. 3. Tonic Seizure
  4. 4. Atonic Seizure
  5. 5. Three Patients all LGS • Etiology: All different, one had encephalitis at 3 months with prolonged status epilepticus. One has a mutation in the SCN2A gene. One has no known cause. • They all have seizures that are called generalized, that means both sides of the brain seem to have simultaneous discharges. • This is a key feature of LGS-generalize EEG abnormalities.
  6. 6. Hemispheric synchrony
  7. 7. Corpus Callosotomy
  8. 8. Decrease in Drop Seizures Following CC • Graham D Graham D et al. 2017
  9. 9. Development • Adults do not develop LGS. • Infants and young children develop LGS. – There often is a progression of seizure types with age. – Young infants will have focal or infantile spasms then go on to develop the key generalized seizures of LGS, Tonic and Atonic seizures. – Why does the developing brain develop this aberrant synchrony?
  10. 10. Multiple Etiologies • Genetic-25-50% • Lesion Genetic- 10-20% • Lesion injury (early in life)-25% • Somehow a change in the brain, often the cortex that is present early in life and possibly only on one side can cause the whole brain or at least both sides to have a discharge simultaneously.
  11. 11. Hemispheric synchrony
  12. 12. Conceptualizing Lennox–Gastaut syndrome as a secondary network epilepsy Archer et al 2014
  13. 13. Take home points • LGS is due to a usurping of the normal networks that develop in childhood. Abnormalities in the cortex in one or many sites can drive the aberrant connections. The network becomes “to linked” and that causes seizures and cognitive problems.
  14. 14. So what to do? • If there is a cortical driver take it out. – Clear from TS and focal developmental disorders and the first patient I showed with encephalitis you can find cortical drivers in the network and removing them can improve seizures and (cognition?). • Earlier you take it out the better (probably) • What about manipulating the network? A work in progress?
  15. 15. Network Modulation • Electrical-Deep Brain Stimulation- Unclear still what target though the thalamus is the most often targeted site. • Genetic- There are at least 40 companies currently in phase 1-2 trials for gene replacement. Though rare in brain disorders, at least 2 are showing success.
  16. 16. Epilepsy Team • Neurologists • Nurses Neurosurgeons Dieticians • Neurodiagnostic Technologist • Genetic Counselor • Study Coordinators