Life Reprogrammed: Deep Brain Stimulation Helps Control Excessive and Involuntary Movements in Boy with Rare Disorder

Medical Mysteries is a series that explores rare diseases or unusual medical conditions.

Adrian is the first child in Asia with GNAO1 to undergo deep brain stimulation (DBS) surgery to treat the condition.

ST PHOTO: NG SOR LUAN

Summary

• Adrian, 11, was initially misdiagnosed with cerebral palsy due to a rare gene mutation called GNAO1, which causes movement disorders and developmental delays.
• After a severe influenza A infection worsened his condition, genetic testing identified GNAO1, which led to deep brain stimulation surgery.
• The surgery, performed by Dr. Nicolas Kon, was successful, improving Adrian's seizures by 90%, allowing him to return to school.

SINGAPORE – Fewer than 500 people worldwide are known to have a rare genetic disorder called GNAO1 mutation, which disrupts brain signals and affects movement and development.

That's why Adrian, an 11-year-old boy, had involuntary jerking movements and slow development, which were initially thought to be cerebral palsy - a group of diseases related to movement and posture caused by brain damage during development, often occurring before birth.

His mother, Madam Felicia Tan, 43, told The Straits Times that Adrian was born prematurely at 34 weeks and had to stay in the neonatal intensive care unit for three weeks.

“Nothing too serious except for a small hole in the heart, which was treated with medication,” she said.

However, when Adrian was nine months old, he was not meeting the developmental milestones appropriate for his age. He could not sit up, crawl, or babble repetitive syllables like “mama.”

“He was also not communicating, and that was when he was transferred to KK Women's and Children's Hospital (KKH), where he was diagnosed with cerebral palsy and treated until he was 10 years old,” said Ms Tan.

The condition worsened after a bout of flu.

The situation worsened when Adrian contracted influenza A in June 2025. He developed a high fever and severe tremors in his legs.

“We took him to NUH (National University Hospital), the closest one to our house. He tested positive for influenza A, was given Tamiflu and sent home,” she said.

But despite treatment, Adrian remained very weak.
“He lay motionless in bed. He couldn’t sit up. Almost two weeks later, the recurring tremors started in his legs. He had no control over his body… He was so stiff that we couldn’t put him in a wheelchair,” she said.

At this time, Ms Tan's sister-in-law – who worked at KKH – introduced the family to pediatric neurologist Dr Simon Ling.

Adrian was hospitalized in mid-2025 and underwent genetic testing.

In July, she was diagnosed with the GNAO1 mutation – which affects proteins that act as molecular “switches” in the brain, disrupting signal transmission.

Doctors say children with the condition often have severe movement disorders, muscle stiffness and developmental delays – symptoms that closely resemble cerebral palsy. Only genetic testing can confirm the diagnosis.

According to medical literature, the first children with GNAO1 were only discovered in 2013 by Japanese scientists.

The Only Chance: Deep Brain Stimulation Surgery

Following the diagnosis, Dr Ling referred the family to see paediatric neurologist Dr Yeo Tong Hong (Parkway East Hospital), who assessed that Adrian was suitable for deep brain stimulation (DBS) surgery.

“This is an advanced method that uses two thin wires (electrodes) placed in specific locations of the brain. They are connected under the skin to a small battery-powered device placed on the chest or abdomen,” Dr Yeo explained.

The device is about the same size as the Apple Watch face. Adrian and his mother Felicia Tan pose with neurosurgeon Nicolas Kon from the Hospital Mount Elizabeth (left) and Dr Yeo Tong Hong, paediatric neurologist from Parkway East Hospital.

ST PHOTO: NG SOR LUAN

The device generates small electrical pulses that help regulate abnormal brain activity and reduce symptoms, especially dystonia, which causes continuous twisting or jerking of the muscles.

This surgery is also used for Parkinson's disease when medications are no longer effective.

According to studies, DBS is particularly effective in primary dystonia with improvement rates of up to 90%.

In Adrian, the condition worsened with life-threatening “dystonic storms” – repeated, uncontrollable muscle spasms throughout the body.

Dr Yeo had treated the first GNAO1 patient in Scotland so he understood that DBS was Adrian's only hope.

He had been preparing to advise the family to go to the UK or the US for surgery, until he learned that neurosurgeon Nicolas Kon at Mount Elizabeth Hospital had expertise in performing the procedure on children.

Pioneering surgery in Asia

Adrian had been in intensive care for a long time and was not responding to medication, complications arose. Therefore, surgery had to be performed urgently.

The surgery lasted 4 hours on July 19. Adrian became the first child in Asia with GNAO1 to receive DBS implantation.

“We inserted two electrodes 7cm deep into the target locations in the brain, based on CT and MRI images to locate. Both hemispheres of the brain are affected, so two electrodes are needed,” said Dr. Kon.

The pulse generator is placed under the skin in the area below the collarbone, connected to the electrodes via a wire inserted under the skin.

As soon as the device was turned on, Adrian's jerking movements stopped.

The device’s battery can last 15 years and needs to be charged wirelessly every week. The family usually charges it while she sleeps to avoid movement.

Four Months Later: Back to School

Thanks to intensive physical therapy, after 4 months Adrian returned to school and used a walker. The number of involuntary movements decreased significantly.

The family is extremely grateful and hopes Adrian's story will help other parents feel confident in having their children genetically tested when they encounter an unexplained abnormality.

Deep brain stimulation (DBS)

DBS is a method of implanting a device that generates electrical pulses into the brain to control neurological symptoms such as Parkinson's and recently GNAO1. DBS helps regulate brain activity, reduce tremors, stiffness and slowness of movement.

Surgery usually lasts 4–6 hours and the child is given general anesthesia. The side view of Adrian's head, showing a DBS electrode implanted deep in the brain.

PHOTO: DR. NICOLAS KON

Surgical procedure

Part 1: Placing Electrodes in the Brain

• Using a brain GPS system to deliver electrodes to the motor control area
• Combining postoperative CT and preoperative MRI to create digital brain models
• Drill a small hole in the skull, place a signal-testing electrode, and then replace it with a permanent electrode.
• Repeat with the other side of the brain.

Part 2: Connecting the wires and battery pack

• The wire is threaded under the skin, behind the ear, down the neck to connect to the battery pack in the chest.

Postoperative

• Infusion of antibiotics, pain relievers, antiemetics
• CT scan to check device position
• Turn on DBS before discharge and adjust according to the follow-up schedule

Source: https://www.straitstimes.com/singapore

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