A 12-year-old boy has become the first ever person with epilepsy to have a device implanted into his skull to reduce epileptic seizures.
Oran Knowlson, who is autistic and has ADHD, has had severe epileptic seizures since he was three years old. His seizures have become more severe over time, often leaving him unable to breathe and needing resuscitation.
Onran has Lennox-Gastaut syndrome, a type of epilepsy which cannot be controlled with standard medication. Since his seizures first started, Onran’s mum, Justine, says her son “struggled to engage in the world”. He needed fulltime care and he was at increased risk of Sudden Unexpected Death in Epilepsy (SUDEP).
Now, eight months on from when the device (known as a neurostimulator) was fitted, Onran’s seizures have reduced dramatically both in severity and frequency.
A neurostimulator is a device that is mounted onto the skull and attached to electrodes deep in the brain to reduce seizure activity. It differs from other Deep Brain Stimulation (DBS) devices which are typically mounted on the chest with wires running up the neck to the brain. By mounting the device in the skull, the researchers say the leads are less likely to break or erode as the child grows.
The device targets the thalamus, which is a hub for electrical signals in the brain. It is hoped that the device will block electrical pathways and consequently stop seizures from spreading. The device also has settings for optimisation towards seizure patterns and is rechargeable with wearable headphones, which means it does not require surgery to replace it every few years.
The Children’s Adaptive Deep brain stimulation for Epilepsy Trial (CADET) is the first ever trial to test our the neurostimulator on children with epileptic seizures, and following Onran’s success, three additional patients with Lennox-Gastaut syndrome are set to be recruited.
Oran’s Mum Justine said: “[Since December] we’ve seen a big improvement; seizures have reduced and are less severe. That’s been great but the quality of life improvement has been invaluable for Oran.
“He’s a lot more chatty, he’s more engaged. He’s turned 13 and I definitely now have a teenager – he’s happy to tell me no. But that adds to his quality of life, when he can express himself better.”
Justine says the device has not only improve Onran’s quality of life but it has also made the future “brighter and more attainable” for both him and the rest of the family.
Epilepsy is common in people with learning disabilities, with around one in three (32%) with a mild to moderate learning disability also having epileptic seizures.
Uncontrolled epilepsy carries significant safety concerns, and mortality rates among people with epilepsy are three times higher than the general population. SUDEP (Sudden Unexpected Death in Epilepsy) is the leading cause of epilepsy-related mortality, and having uncontrolled seizures can increase the risk.
Epilepsy can usually be controlled with anti-epileptic drugs (AEDs), however, some people, like Oran, have drug resistant epilepsy (DRE) which cannot be controlled with standard medication. Scientists therefore hope that this new device will become a standard treatment and improve the lives of thousands of people with DRE.
Martin Tisdall, Honorary Associate Professor at UCL and Consultant Paediatric Neurosurgeon at GOSH, said: “Every single day we see the life-threatening and life-limiting impacts of uncontrollable epilepsy. It can make school, hobbies or even just watching a favourite TV show utterly impossible.
“For Oran and his family, epilepsy completely changed their lives and so to see him riding a horse and getting his independence back is absolutely astounding. We couldn’t be happier to be part of their journey.
“Deep brain stimulation brings us closer than ever before to stopping epileptic seizures for 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 paediatric epilepsy and hope in years to come it will be a standard treatment we can offer.”
