Published: December 12, 2024
The Priar family was enjoying a summer barbecue when they noticed something was wrong with their 2-year-old son, George.
“We were at a pool party, and he started staring off, just kind of went blank, so we called 911,” said Larissa Priar, George’s mother. “George was taken to our local emergency department, and we learned he was having silent seizures. Once he was stable, he was flown to Norton Children’s Hospital.”
The family from Owensboro, Kentucky, spent several days at the Louisville hospital, with George undergoing several tests.
“I was scared — nervous — but we were just trusting the doctors and trusting the teams and the nurses that they were going to do the best that they could and help to get us some answers,” Larissa said. “They took really good care of George, made sure he was comfortable, and they watched him very closely.”
Ultimately, the Norton Children’s Neuroscience Institute team was able to find the answer through an MRI, which revealed George had a rare brain malformation known as periventricular nodular heterotopia (PVNH).
“It is a condition in which some of the nerve cells in the brain, called neurons, did not migrate or travel to where they were supposed to when the brain was developing. These nerve cells got ‘stuck’ along the lining of the ventricles, or the normal fluid-filled spaces of the brain,” said Margaret J. Means, M.D., a pediatric neurologist with Norton Children’s Neurosicence Institute. “There are several possible causes for why migration may be disrupted and result in PVNH, including a genetic change or toxic exposures such as carbon monoxide.”
PVNH is incurable and is considered rare; its exact prevalence is unknown. The disease typically is discovered after a patient begins to have seizures. In some cases, PVNH can cause learning disabilities and developmental delays.
George is currently on anti-seizure medication and is participating in speech therapy. His parents and care team will continue to monitor for any possible delays, but so far, George seems to be progressing well, according to Larissa.
“He’s been doing great. He’s doing well in speech therapy,” Larissa said. “He’s running around being a happy little boy.”
According to Dr. Means, in some cases, if seizures cannot be managed with medication, surgery may be necessary.
For now, George’s parents are taking it one day at a time, grateful Norton Children’s was able to provide the right diagnosis.
“We’re thrilled with the care George has gotten and continues to receive,” Larissa said. “At this point, we feel like Norton Children’s is our second home.”