A teenager with severe epilepsy has become the first patient in the world to have his seizures controlled using a skull implant.
Oran Knowlson has seen his daytime seizures reduced by 80 percent as a result of the neurotransmitter sending electrical signals deep in his brain.
This 13-year-old boy from Somerset has suffered from Lennox-Gastaut syndrome, a form of treatment-resistant epilepsy, since he was three.
He had daily seizures that in some cases caused him to fall to the ground, shake violently, and lose consciousness.
But now, eight months after his life-changing operation as part of a trial at Great Ormand Street Hospital in London, The adolescent’s future seems “hopeful” and he is more “talkative” and “engaged.”
Oran Knowlson, 13, has seen his daytime seizures reduced by 80 percent as a result of the neurotransmitter sending electrical signals deep in his brain.
Oran’s seizures began two weeks after his third birthday and until the trial he had not had a single day without seizures.
After some seizures I would need emergency medication to resuscitate him as he had stopped breathing.
Due to the severity of his epilepsy, he required 24-hour care for most of his life, as seizures could occur at any time of the day and he was at significantly increased risk of sudden unexpected death in epilepsy (SUDEP).
‘Before the seizures started, Oran was hitting all of its milestones, but as the seizures became more severe, we lost more and more of Oran. From being a happy and energetic three-year-old, he struggled to engage in the world because of his medication and seizures, but he still retains his sense of humor,” said Justine Knowlson, Oran’s mother.
He added: “We’ve tried everything, but this is the first real opportunity we’ve been given in years, there hasn’t been any ‘what’s next’ until now.” Unless someone takes the first step in a trial like this, there will never be anything better and there has to be something better for our family.”
Lennox-Gastaut syndrome affects about one to two in 100 children with epilepsy that usually begins when the child is between three months and five years old.
The rare epilepsy syndrome causes periodic seizures and learning difficulties.
Oran is part of the CADET (Children’s Adaptive Deep Brain Stimulation Trial for Epilepsy) project, which consists of a series of trials evaluating the safety and effectiveness of deep brain stimulation for severe epilepsy.
The partnership involves Great Ormond Street Hospital, University College London, King’s College Hospital and the University of Oxford.
Epilepsy seizures are caused by abnormal bursts of electrical activity in the brain.
But the rechargeable device, the Picostim neurotransmitter made by British company Amber Therapeutics, works by emitting a constant pulse of current to block these abnormal signals.
It is mounted on the skull and connected to electrodes deep in the brain to reduce seizure activity.
Oran underwent surgery in October 2023 to insert the device, and once he recovered from the procedure, they “turned it on,” delivering constant electrical stimulation to Oran’s brain. Since then, the life of Oran and his family has changed completely.
Mrs Knowlson said: “We have been online since December and have seen a huge improvement, the seizures have reduced and are less severe.” It’s been great, but the improved quality of life has been invaluable for Oran.
“He’s a lot more talkative, he’s more engaged. He turned 13 and I’m definitely a teenager now; he’s happy to tell me no. But it increases his quality of life, when he can express himself better.
‘The team really has your back. We never felt alone, since last August (when we joined the rehearsal). They made us feel part of the team and so did Oran.
‘The future seems hopeful, something that would not have occurred to me to say six months ago. For Oran, having hope brings excitement. He makes the future brighter and even more attainable. “I am very happy that Oran can live this experience.”
Typically, deep brain stimulation (DBS), which is a treatment that involves surgery to insert a small device that stimulates specific parts of the brain, involves mounting devices on the chest with wires that run through the neck to the brain.
But this device is mounted on the skull, which means the wires are less likely to break or erode as the child grows.
The device that can be recharged through portable headphones (pictured) works by emitting a constant pulse of current to block these abnormal signals.
Since receiving the implant, Orán has a better quality of life and has become more “talkative and engaged”
It can also be recharged through portable headphones, which can be used while watching a video or interacting with a tablet.
This also means that patients will not need to undergo surgery every three to five years to replace it.
The implant targets the thalamus, which is an electrical signaling center in the brain.
The device is expected to block electrical pathways and consequently stop the spread of seizures.
It also has settings for optimization of seizure patterns, which although not used in this trial, could be used in the future for patients with Lennox-Gastaut syndrome.
‘Every day we see the life-threatening and life-limiting impacts of uncontrollable epilepsy. “It can make school, hobbies or even just watching a favorite TV show completely impossible,” said Martin Tisdall, consultant pediatric neurosurgeon at GOSH and honorary associate professor at UCL.
‘For Oran and his family, epilepsy completely changed their lives and so seeing him ride a horse and regain his independence is absolutely amazing. We couldn’t be happier to be a part of his journey.
‘Deep brain stimulation brings us closer than ever to stopping seizures in patients who have very limited effective treatment options.
“We are excited to build the evidence base to demonstrate the ability of deep brain stimulation to treat pediatric epilepsy and hope that in the coming years it will be a standard treatment that we can offer.”
The CADET trial is now recruiting three additional patients with Lennox-Gastaut syndrome for the pilot, funded by the Royal Academy of Engineering. 22 patients will then take part in the full trial, funded by GOSH Charity and LifeArc and sponsored by University College London.
