When we hear the word stroke, we think of older people, not of babies in the womb. Yet neonatal strokes, although rare, do happen. It happened with Ozzy, an exuberant, science-loving 7-year-old who is constantly busy playing with his little sister, making up stories about his favorite stuffed animal named Bunny, and dreaming about what he wants to be when he grows up.
“I want to be a zoologist or paleontologist. But sometimes I want to be a police officer or firefighter,” Ozzy says.
A few hours after a long and difficult delivery, a nurse noticed that Ozzy had rhythmic arm and leg movements on one side and that his eyes were moving rapidly back and forth. He was transferred to the Neonatal Intensive Care Unit at Stanford Children’s Health. Within 24 hours after birth, his parents were told that he had experienced a large middle cerebral artery (MCA) stroke, likely in the final week of pregnancy, which destroyed 85% of the left hemisphere of his brain.
“We were in shock,” says Ozzy’s mom, Stefanie.
With a neonatal stroke, scar tissue forms in the brain. Sometimes it short-circuits nerves, causing seizures, with approximately 30% to 40% of children eventually developing epilepsy. Everyone still held out hope that Ozzy would be on the right side of the odds. He received care regularly at the Epilepsy Center at Stanford Children’s Health.
When Ozzy was 4 months old, his parents noticed that he was easily startled and that this startle seemed to come in clusters. He was diagnosed with infantile spasms (IS), a rare seizure disorder that can progress to epilepsy. Under the care of Stanford Children’s Health doctors, Ozzy was able to beat IS and was seizure free between the ages of 1 and 4. Life felt normal for Ozzy and his family. He was a happy, curious, and thriving kid.
“It seemed like he bobbed and weaved around epilepsy for a few years there,” Stefanie says.
When Ozzy turned 4 years old, Stefanie started seeing odd behaviors. He became defocused, frazzled, and sometimes spontaneously destructive. He had trouble finding words and stringing sentences together. Processing new information and remembering old information became difficult at times. All of this made him frustrated. His teachers started noticing things, too. He seemed to be in his own world and stopped engaging with classmates in preschool. Ozzy was experiencing seizures, but they were not obvious to the untrained eye.
Ozzy’s odd behavior culminated on one dark day. He awoke groggy, had trouble dressing, and a little later struggled to sit up. Then, he started convulsing in the back seat of the car. His dad, Eran, called 911 and an ambulance rushed Ozzy to the local hospital, where he was treated for a large seizure.
“It was really scary, but when he woke up in the hospital the next day, he seemed fine. Mostly, he was upset that he didn’t remember the ambulance ride, because he loves ambulances and fire trucks,” says Eran.
To find out what caused the seizure, Ozzy’s parents brought him back to Stanford Children’s Health for an examination and an electroencephalogram (EEG), which records brain activity and helps doctors make care decisions for children with epilepsy. They discussed their concerns with Ozzy’s school performance and behavior.
“During the exam, Ozzy looked at a picture of a boy pointing at a rainbow with his dog, and he could say ‘rainbow’ and ‘dog,’ but he couldn’t tell us what was happening,” says Emily Spelbrink, MD, PhD, an epileptologist who has cared for Ozzy for years, first as a resident physician.
Ozzy’s EEG revealed encephalopathy with status epilepticus during sleep (ESES), which means that all night long his brain was having tiny seizures, leaving him mentally and physically exhausted.
“If you think of a seizure as an electrical fire in the brain, then ESES is little sparks going off every second during sleep. It tires the brain out,” Dr. Spelbrink says.
A solution had to be found. An epilepsy team of more than 20 experts, including pediatric epileptologists, neurosurgeons, neurologists, fellows, residents, nurse practitioners, nurses, case managers, and neurodiagnostic technologists, came together to review every detail of Ozzy’s medical journey, from his earlier stroke to his current seizure and his ESES episodes, to decide the best path forward.
“We tried all possible medicines over the course of two years, to no avail,” Stefanie says. “We saw more regression. He had a hard time dressing himself. His teacher would call to say he spent the whole day lying on the floor. We knew we couldn’t go on this way.”
The Complex Primary Care Clinic at Stanford Children’s Health stepped in to help coordinate Ozzy’s care and provide support for the family. His parents say that they would have been lost if it hadn’t been for the extraordinary support from David Bergman, MD, at the clinic, because it is incredibly overwhelming to care for a medically complex child.
Since medicines were not working for Ozzy, his doctors suggested another approach, which sounded unbelievable—a surgery to disconnect the two sides of Ozzy’s brain to stop the seizures. It’s called a hemispherotomy, and most parents can’t believe that something like it is even possible.
“Disconnecting half of a child’s brain seems pretty insane, but it’s in the normal scope of insane for treating severe epilepsy,” Dr. Spelbrink says.
It’s a highly complex surgery that’s reserved for the most severe cases of epilepsy. Think of it as flipping the circuit breaker in a section of your house. Without power, there’s no electricity in certain rooms, and they remain dark and quiet. When the connection between the healthy and unhealthy halves of the brain is severed, seizures cannot spread. The surgery would essentially disconnect the faulty wiring from Ozzy’s stroke-scarred brain tissue, stopping it from traveling to the other side and interrupting his healthy brain activity.
“It was a huge decision, and we debated whether or not Ozzy should get the surgery. It wasn’t super clear-cut because even though he had some odd behavior, he also tested normal on a neuropsychology test. Nonetheless, his seizures continued, and our quality of life continued to deteriorate along with it,” Eran says. “The Stanford team advised us but never pushed us. Instead, they empowered us to make the decision.”
Hemispherotomy is often confused with its cousin, hemispherectomy. The first disconnects tracts between the two sides of the brain or makes small holes in the brain to disengage damaged parts, while the second, hemispherectomy, removes damaged brain tissue. These remarkable brain surgeries are offered only at large pediatric epilepsy centers, like the one at Stanford Children’s Health.
“On a scale of 1 to 10 for complex brain surgeries, hemispherotomy is a 10. It’s the most complex, most invasive brain surgery done for epilepsy,” says Gerald Grant, MD, renowned neurosurgeon and co-director of the Epilepsy Center at Stanford Children’s Health.
What finally tipped the scale on an impossible decision for Stefanie and Eran was the worry of sudden unexpected death in epilepsy (SUDEP). The more seizures a person has, the greater the risk of SUDEP. Plus, Ozzy’s function was deteriorating even more.
“There’s a window of opportunity with the brain. The longer you wait, the harder it can become for the brain to rewire its tracts, and a child can lose function that can’t be regained,” Dr. Grant says.
Because Ozzy had a stroke before birth that essentially destroyed the left side of his brain, the right side took over all functions early on, making him the perfect candidate for a hemispherotomy. This meant that functions like speech, memory, and vision were now performed by his healthy right side. Doctors ran several tests, including a functional MRI, which maps important brain functions, to confirm it. Ozzy is bilingual, so the team tested him for both English and German, and they saw that his brain was exclusively lighting up in the right hemisphere.
“Not every epilepsy center would try a hemispherotomy, but we have a large team with a lot of experience that communicates well and trusts each other to deliver spectacular outcomes,” says Brenda Porter, MD, epileptologist and co-director of the Epilepsy Center, which is accredited by the National Association of Epilepsy Centers as a level 4 epilepsy center. “And we have a top-notch EEG lab to guide us.”
It took several hours for Dr. Grant to perform the hemispherotomy because he had to disconnect every tract crossing from left to right along the corpus callosum—the band of nerves that connects the two cerebral hemispheres, and the brain stem. A tract is a neural pathway made up of several nerve fibers grouped together that carry information between parts of the brain, the brain stem, and the spinal cord—and from the spinal cord to the body.
His work was guided by the latest and greatest neurosurgery tools, Synaptive. The tools include an ultra-high-resolution camera-telescope/microscope mounted on a robotic arm that provides a high-quality 3-D view of the brain. For the hemispherotomy, Dr. Grant used its tractography feature, which empowered him to visualize the left-to-right-running tracts and to make sure that he disconnected all of them. Stanford Children’s Health is the first pediatric hospital in the world to use Synaptive.
“It’s an exciting time to be a pediatric epileptologist because there are several tools we can now use to treat and diagnose epilepsy. Besides Synaptive, we are using lasers to surgically ablate areas of the brain that cause seizures, placing stereo EEGs in babies, and we are one of the first in the country to implant devices to provide deep brain stimulation,” Dr. Porter says. “The earlier we can treat, the better cognitive outcomes for kids like Ozzy.”
Even with extensive testing and planning, the moment of truth is when a child wakes up from hemispherotomy surgery. Stefanie will never forget that first visit with Dr. Grant when he gave her two thumbs up after visiting Ozzy just a few hours after the surgery.
“We trusted that the brain moved everything to the right side, but the brain can work in mysterious ways, so it felt great to see him moving and talking just fine after surgery,” Dr. Grant says.
Ozzy completed several months of intensive rehabilitation to continue to rewire new pathways and strengthen existing ones. Dr. Grant believes he will continue to make strides for years.
From his neonatal stroke, Ozzy has spastic hemiplegia, a muscle disorder that affects the entire right side of his body. To overcome this challenge, Ozzy attends daily physical and occupational therapy sessions, in conjunction with neuromuscular electrical stimulation (e-stim). He has received serial casting, which holds and stretches muscles in his leg to increase range of motion and flexibility. Ozzy has improved in many unexpected ways following his hemispherotomy. His quality of life is dramatically different, and today he’s a healthy 7-year-old living a full, happy life.
Today, if you show Ozzy a picture and ask him what’s happening, he goes on and on, making up spontaneous stories. His EEG went from 90% ESES activity on the right side of his brain to clean with zero abnormal activity. His left side still shows little discharges, but since the brain is disconnected, they cannot travel to the functional right side of his brain and are completely harmless.
“He gained back much more than we let ourselves hope for,” Dr. Spelbrink adds.
Dr. Grant describes a child’s state after a hemispherotomy as almost manic. They go from a limited world to one that’s wide open, full of new sights, sounds, and thoughts. The healthy part of the brain is free to work efficiently, no longer tormented by the damaged part.
“After surgery, Ozzy was so excited by all the input. His mind was working so much faster, and he couldn’t stop talking and asking questions. He embraces the world in a way he never could before,” Dr. Grant says. “At his follow-up visit, we could almost see his brain rewiring right in front of us.”
The sky’s the limit for Ozzy. Looking back in years to come, his doctors say that people will never know he had a hemispherotomy. Best yet, he’s seizure free. Maybe his sister, Sofie, age 4, described his change most poignantly when she said, “I can play with him, now.”
“It was like a switch was flipped after the surgery. We are in disbelief with the change,” Stefanie says.
Doctors gave them their smart, funny, driven kid back, and they couldn’t be more grateful.