Nine-year-old Zane Johnson recently received the “most improved award” in his class and for the first time, he is on the A/B honor roll. These accomplishments mean a lot to Zane and his Benton County family because so much of his life has been interrupted by constant seizures.
Zane suffered a stroke in utero, before he was born. By the time he was three months old, his parents suspected there was something wrong.
“His head leaned to the left side,” his mother Sherri Beth Johnson said. “We would continually turn it to the right, and it would fall back to the left. We could hold a toy near his face on the right side and it was as if he didn’t see it.”
The Johnsons were referred to an eye specialist. Zane had an outpatient surgery to open a tear duct. His head was misshapen and doctors fitted for a helmet he would wear 23 hours a day. In addition, Zane was seeing a physical therapist to strengthen his neck muscles and help him sit up.
“It took nine long months and a number of diagnoses before an MRI confirmed Zane had hemiplegia cerebral palsy,” Johnson said.
Zane’s stroke had damaged the left frontal lobe of his brain, affecting his motor skills and the use of his right side.
Zane had a few seizures when he was 4, which doctors thought was due to a viral illness.
“They prescribed medication but it made him very violent, so they took him off and we didn’t see any more seizures,” Johnson said.
The seizures began again two years later. This time, they were happening more frequently. Doctors no longer believed they were due to a viral illness. Zane was prescribed medication again, but the seizures continued.
“They had to raise the dosage. And when they did that, he shut down,” Johnson said. “He didn’t laugh anymore. And over the next year he gradually started declining. And it wasn’t just little things. He would wake up and not remember how to use the bathroom. We looked for a second opinion.”
In 2013, the Johnsons saw Erin Willis, M.D., a neurologist and assistant professor in the Department of Pediatrics in the UAMS College of Medicine.
Willis set him up for an overnight test that would measure the activity in his brain called an electroencephalography (EEG) and MRI. While the family waited for that appointment, Zane’s seizures intensified and doubled in frequency.
“He would have drop seizures, he would be at school and just drop to the floor,” Johnson said. “He was back in diapers. We were watching him decline before our very eyes. By this time he had also lost all information he’d learned: numbers, letters and sight words. Mentally, he was operating as an 18-month-old child.”
In February 2014, Zane and his family traveled to Little Rock for their overnight EEG appointment. During that admission he was diagnosed with epileptic encephalopathy with continuous spike and wave during sleep, a rare epileptic disorder that is associated with frequent seizures and developmental regressions. Initially Zane was given anti-epileptic medications, but his seizures continued to worsen and he developed nonconvulsive status epilepticus, a near-constant state of uncontrollable seizures.
He was admitted to the intensive care unit for urgent treatment with further anti-epileptic medications, in an attempt to stop the persistent state of seizure activity that was preventing him from waking up. A team of UAMS neurologists and neurosurgeons met with the family to decide on the best way to stop his seizures.
“Given his history of bad seizures, a stroke and an MRI we felt that a hemispherotomy would be the best option for him,” said Gregory Albert, M.D., pediatric neurosurgeon at Arkansas Children’s Hospital and assistant professor in the Department of Neurosurgery in the UAMS College of Medicine.
A rare procedure, a hemispherotomy involves disconnecting one cerebral hemisphere (half of the brain) from the other. The surgery was scheduled for April 1, 2014. After seven hours, the family received news the surgery was successful.
“His seizures are now controlled,” said Demitre Serletis, M.D., Ph.D., assistant professor in the Department of Neurosurgery in the UAMS College of Medicine. “They stopped at the time of surgery. We were recording from the good side, while operating on the bad side. The seizure activity that was flowing to that good side had completely stopped with the last disconnection. And since that time, we have not recorded a single seizure.”
Three days after surgery, Zane moved the right side of his body. His mother says they were all surprised and overjoyed when he lifted his right arm for the first time.
“When I first met him, Zane had been regressing,” Albert said. “Since his surgery he has regained all his knowledge and surpassed where he was before. His recovery is happening much faster than we expected. This is fantastic and it’s what we hope for all our patients.”
UAMS and Arkansas Children’s Hospital bring together a team of experts from different medical specialties including neurology, neurosurgery, neuropsychology and neuroradiology. The team works to find the most appropriate epilepsy treatment for each patient.
Today, Zane is back up to a kindergarten level. He is now able to answer simple math problems, tell time and count money. His mother says he’s using his imagination and for the first time was able to understand the concept of Christmas and become excited about it. Zane’s days are filled with dancing, singing and laughter again.
“Anytime we can get children back to where the parents had once seen them, it’s the best feeling in the world,” Willis said. “It’s why we come to work every day.”