Surgical Treatment

The surface of the brain (cortex) is organised into specific areas that deal with special functions such as movement or language. Brain cells (neurones) in this surface layer, have important fibres that pass through the brain substance to carry messages to control the rest of the body. These brain cells also have small fibres that branch out and connect them to the other brain cells in the surface layer. In LKS, one area of the brain’s surface develops electrical discharges or seizures. This area then spreads the seizures to other areas of the surface, through its network of small fibres, and thereby becomes ‘dominant’ and ‘drives’ the rest of the surface or cortex into discharges that ‘tie-up’ the brain cells and prevents them from carrying out their specialised function, such as language.

Brain surgery for LKS aims to prevent spread of seizures through this surface network by making tiny cuts over the surface where the seizures originate, preventing the discharges travelling sideways to other surface areas, whilst preserving the long fibres that carry the specialist messages to the rest of the body. This surgery is called ‘multiple subpial transection’ and requires specialised assessment to identify the ‘dominant driving’ area of the brain surface to be targeted.

Brain surgery may be used for children who have active disease with poor recovery of skills and EEG evidence of continuous seizures in sleep, or for those who require unacceptably high doses of steroids to maintain their recovery. It does not aim to cure the child, but to limit any further loss of skills and allow some recovery.

Less than half of the children who are assessed for surgery, are found to be suitable on investigation. Brain surgery inevitably has some risks. However, the experience in reputable centres is that more than half of the children experience significant improvement, not simply in language, but often most markedly in behaviour, particularly autistic features. Brain surgery however, is not curative and the children will have some remaining impairments, although experience to date is that no children have been made worse by the procedure.

Surgery (MST) aims to cut the surface fibres (solid arrows) and hence prevent the spread of seizures to other surface areas, whilst still preserving the long fibres (dashed arrows) which take messages to the rest of the body.