Oran, 13, suffers from Lennox-Gastaut syndrome. This form of severe and early epilepsy proves resistant to treatment. Until last year, the young boy suffered several dozen seizures daily, impacting his quality of life. In October, he benefited from an intervention aimed at implanting a new deep neurostimulation device. His situation has improved considerably since then.
Lennox-Gastaut syndrome (LGS) is a rare and difficult to treat form of childhood epilepsy. The first seizure most often occurs between the ages of 2 and 6 years. The disease causes “absences”, involuntary muscle contractions, falls, then cognitive impairment. Oran, originally from Somerset, developed the syndrome when he was three years old.
Since then, he suffered dozens of seizures day and night. Several members of his family carry a mutation in the SCN1B gene, causing a form of epilepsy and heart abnormalities. But unlike Oran, today everyone has their crises under control. He was not so lucky, his epilepsy continued to worsen over time. This illness “stole his entire childhood”, according to his mother’s words, reported by the BBC.
A neurostimulator fixed in the skull bone
He frequently collapsed suddenly, shook and lost consciousness. Sometimes the attack led to respiratory arrest requiring emergency intervention. Oran therefore needed 24-hour care, as a crisis could occur at any time. Unfortunately, the young boy also suffers from autistic disorder and ADHD, but his epilepsy is the biggest burden. “ While he was a happy, energetic three-year-old, he struggled to engage in the world because of his medications and seizures “, said his mother.
Fortunately, Oran was able to integrate the CADET project (Children’s Adaptive Deep brain stimulation for Epilepsy Trial). This project implemented by University College London encompasses a series of clinical trials to study the safety and effectiveness of deep brain stimulation on children with LGS. Oran is the first of four children who will be involved in the pilot test.
The project is based on a device called Picostim, manufactured by Amber Therapeutics, designed to control seizures. These are triggered by a sudden abnormal electrical discharge in certain areas of the brain. Picostim emits a constant electrical pulse, in order to block or disrupt these discharges. It is connected to two electrodes that are implanted deep into the brain, until they reach the thalamus – a “hub” of electrical signals in the brain.
Diagram of the implantation of the Picostim deep brain stimulation device, equipped with DyNeuMo-1 software. Credits: Great Ormond Street Institute of Child Health
Oran was operated on in October 2023, at Great Ormond Street Hospital in London. This very delicate intervention lasted almost eight hours. Surgeons removed a piece of bone from the skull and instead inserted the device – a square measuring 3.5 cm on a side and 0.6 cm thick. They then screwed this neurostimulator into the surrounding skull to secure it.
Daytime seizures reduced by around 80%
Note that deep brain stimulation has already been tried to treat childhood epilepsy. However, the neurostimulators were placed in the chest and then connected to the brain by wires. According to Martin Tisdall, the neurosurgeon who led the team, positioning the implant in the skull allows us to avoid certain complications. Indeed, the risks of post-operative infections and device failure (via damage to the wires) are lower.
The team activated the neurostimulator about a month after the operation. Since then, Oran’s brain has received constant electrical stimulation, imperceptible to the boy. The results are very positive. “ He is more alert and no longer has seizures during the day. His nocturnal attacks are also shorter and less severe “, his mother told the BBC. Thanks to Picostim, daytime seizures have decreased by almost 80%, reports the hospital press release.
The device is rechargeable, via wireless headphones. During this time, the young patient can continue their usual activities, such as watching a video. There is therefore no point in planning surgery to replace it every three to five years, as with other devices.
The young boy, now 13 years old, has returned to an almost normal life. At least, his quality of life has greatly improved. His mother says he is much happier, more talkative, more engaged. His son can now express himself better and undertake activities that were previously inaccessible to him. He really enjoys riding lessons – which he does under medical supervision just in case.
Thanks to the neurostimulator, young Oran can now practice the activities he enjoys. Credits: Great Ormond Street Hospital for Children
The team is sincerely happy for this boy and his family. “ LSeeing you ride a horse and regain your independence is absolutely amazing. We couldn’t be happier to be part of their journey said Dr. Tisdall.
A solution to treatment-resistant childhood epilepsy?
This is the first UK clinical trial evaluating this type of treatment for children with LGS. It involved teams from research and development, surgery, nursing, neurology, imaging, anaesthesia, neurophysiology and many others.
The installation of the device does not cure the disease, but considerably improves the quality of life. In addition, it significantly reduces the increased risk of “sudden unexpected death linked to epilepsy”. “ C’is the first real chance we’ve been given in years », Says the mother from Oran. The device also has settings for optimizing seizure patterns, which researchers believe could be useful for LGS patients.
“Oran is the first child in the world to receive this experimental device and we are extremely happy that it has had such a positive impact for him and his family. said Professor Tim Denison, from the University of Oxford, who is the chief engineer. Three more children with LGS will soon be fitted with the deep brain neurostimulator. Then, a second trial will follow, involving 22 other young patients. If the device proves safe and effective, it could become a standard treatment for pediatric epilepsy.
The team also plans to modify the neurostimulator so that it responds in real time to changes in the patient’s brain activity. The goal is to try to block seizures when they are about to occur. “ Every day we see the life-threatening or life-limiting effects of uncontrollable epilepsy. […] Deep brain stimulation brings us closer than ever to stopping seizures for patients with very limited effective treatment options », concludes Dr Tisdall.
