A six-year-old girl with a rare seizure condition had a 10-hour surgical procedure to disconnect half her brain
She is expected to recover and lead a normal life with minimal complications
Hemispherectomies are often successful as a treatment for intractable pediatric seizure conditions
After a 10-hour surgical procedure, a 6-year-old girl in Southern California, Brianna Bodley, will live the rest of her life with half of her brain disconnected. Brianna will need rehabilitation, and some fine motor and visual skills will never return. However, the hope is her personality will not change, and she is expected to live a normal life.
Brianna began experiencing seizures in 2022. In August 2022, she was diagnosed with epilepsy. In September 2022, doctors determined that epilepsy was a misdiagnosis and that Brianna had a rarer condition, Rasmussen’s encephalitis. Rasmussen’s encephalitis is progressive and typically affects only half of the brain. In addition to daily, often severe seizures, children with Rasmussen’s encephalitis experience a progressive loss of neurological function, leading to eventual paralysis.
The condition is most commonly diagnosed in children under 10. It has a diagnosis rate of less than 3 in 10 million children under 18. Treatment is often focused on symptoms and might include antiepileptics and corticosteroids. Surgical treatment has been used for children, like Brianna, with a high recovery potential.
Pediatric neurosurgeon Dr. Aaron Robison recommended the surgical procedure, a hemispherectomy. He was concerned that the disease progression and the daily seizures were damaging Brianna’s brain and advised the Bodleys that a hemispherectomy could prevent further damage and might stop the seizures. Brianna had already tried 6 different antiepileptics and Rituximab as treatments without success. She was receiving intravenous immunoglobulin for pain management.
Modern hemispherectomies can be performed without removing the diseased brain tissue. Surgeons today can disconnect half the brain tissue. This reduces the side effects and complications associated with total removal.
“The one treatment that does lead to predictability in Rasmussen’s encephalitis is hemispherectomy. It’s a definitive option,” explains neurosurgeon Micheal Doherty, MD. “It stops the seizures, by and large. It offers predictability to patients and their families. Timing is influenced by age and by the severity of the neurologic effects.”
It took surgeons 10 hours to disconnect the right half of Brianna’s brain. Surgery was performed on September 28, 2023. Brianna was able to leave the ICU on October 2nd.
Hemispherectomies to treat seizures
Hemispherectomies have been used as a treatment for brain tumors since the 1920s. Today, they’re also used for intractable pediatric seizures and for certain other injuries or abnormalities confined to half of the brain. In their nearly century of use, medical professionals have gained surprising information about brain plasticity from hemispherectomies. This knowledge has allowed the treatment to advance and has given neurologists valuable insights about the brain.
The brain’s abilities following a hemispherectomy can be astonishing. Many children who have this procedure can relearn how to walk, talk, and speak. They’re able to attend school and gain employment as adults. This has been true historically, and since the mid-1970s, when neurosurgeons began sometimes disconnecting half the brain rather than removing it, long-term results have improved.
Success rates of hemispherectomies as a seizure cure can vary depending on the study, the population, and the underlying seizure cause before hemispherectomy. Some data shows a post-hemispherectomy seizure-free rate of over 80%.
Traditionally, it’s been suggested that hemispherectomies are best performed on children under about the age of 5. Still, newer understandings of neuroplasticity and brain development suggest that firm age cut-offs might not be as beneficial as previously thought. Instead, the age of seizure onset and severity of seizure activity might better indicate surgical appropriateness. Studies of adults who underwent hemispherectomies as children are helping to contribute to this knowledge.