Journey to the Center of the Brain

Adapted from an article by Lisa Boylan, Managing Editor, EpilepsyUSA magazine

Anatasia “Ana” LaGalla, 3, had what are known as “laughing seizures,” however there was nothing remotely amusing about them. Beginning in May of 2006, she began having seizures once every two weeks. No one knew at the time they were seizures—including her pediatrician. He chalked up the child’s aberrant behavior to temper tantrums. A few months later, Ana’s seizures became markedly worse, then in January she had a severe seizure where she became non-responsive, her whole body was stiff and her eyes were glassy. Her parents, Jessica and Peter, took her back to the pediatrician. He told them it was a seizure and referred them to a neurologist. The neurologist observed Jessica’s behavior following the seizures because, Jessica explained, “She would be kicking, screaming, biting—she would bite herself.” The neurologist concluded that she needed to have her behavior evaluated. Jessica persisted. She said, “Listen, we think she just had a seizure; the behavior will come second. We have to get one thing under control at a time.”

An EEG revealed she did have seizure activity in her brain but it was localized in the hypothalamus, which controls speech and motor skills. That’s when some pieces of the puzzle started to come together. Jessica said it wasn’t until then that they began to realize, “OK, she doesn’t know how to do a lot of things.” Ana couldn’t jump, she couldn’t take off her clothes by herself; however these developmental milestones got buried under the everyday necessity of getting out the door on time. Jessica said, “I had to get to work in the morning, so it was much easier for me to get her dressed myself, not even realizing that she didn’t know how to do it herself.”

‘The last think you ever expect anyone to tell you about your child.’

MRIs confirmed Ana had a tumor on her hypothalamus. Jessica could not comprehend what the physicians were saying to her—it wouldn’t sink in. She said, “It was the last thing you ever expect anyone to tell you about your child.” The next step was to consult with a neurosurgeon to find out the best way to treat the tumor. The first neurosurgeon they met with told them the hypothalamus was a very high-risk area of the brain to do surgery and he recommended a biopsy of the tumor and then chemotherapy. Jessica was not satisfied with this option. She reasoned, “If you want to go in to do the biopsy, why don’t you just take the whole thing out?” She knew she needed another opinion.

Degrees of separation

Jessica attributes her tenacity in finding the right diagnosis for her daughter to working in a doctor’s office. As it turns out, the doctor she worked for told her about his neighbor, whose daughter had successful surgery performed by a pediatric neurosurgeon named Dr. Steven Schneider. The girl’s mother called Jessica on the phone and on her recommendation, Jessica and Peter took Ana to Dr. Schneider.
Dr. Schneider looked at Ana’s MRIs and immediately recommended surgery. He told Jessica and Peter that the kind of seizures Ana was having—laughing, or gelastic seizures—were very rare. He explained that their rarity was the reason they were having such a hard time getting a correct diagnosis. He estimated there are 1 in 500 cases of gelastic seizures in cases of epilepsy and only a handful of cases a year in the Unites States. Dr. Schneider told them, “Listen, this is something your daughter absolutely needs in order to lead a normal life in society.” He added, “I’m not going to lie to you; this surgery is a risk. There’s a chance she could die on the operating table.” He then explained that if she didn’t have the surgery her seizures would persist, in all likelihood leaving her in a vegetative state. Jessica and Peter knew right then what they had to do; surgery was the only option. Fortunately for the LaGalla family, Dr. Schneider has 20 years of experience with this type of microscopic, deep-seated lesion surgery. Ana’s tumor was in her hypothalamus, which controls body temperature, thirst, and hunger but also emotions—including involuntary emotions, such as embarrassment. Seizures triggered in the hypothalamus are associated with short outbursts of laughter, crying and other behavioral abnormalities. By the time Dr. Schneider operated on Ana, she was having up to 60 episodes a day.

Journey to the center of the brain

In order to perform surgery on Jessica, Dr. Schneider modified a surgical technique to allow him to go much further into the hypothalamus without causing injury. It was the first time he had employed it. He used an open approach where, he said, “you actually go between the right and left halves of the brain” without having to sacrifice any blood vessels on the way in. He used a stereotaxic navigation technique—similar to a global positioning system—to guide him, literally, to the center of the brain. He said, “You don’t have any margin for error.” The navigation system makes it possible to safely perform surgery in areas that were not possible in the past.

‘No news is good news’

The day of the surgery arrived far too quickly. While Ana lay sleeping, Jessica and Peter braced themselves for what they knew would be the longest four hours of their lives. Dr. Schneider came in beaming and said, “Everything’s going to be great! Don’t worry about it.” Jessica asked if he would come down and let them know how the surgery was going. He said, “No—no news is good news.” He told them, however, that if for any reason he thought he should not continue the surgery, he would stop. He said, “I have a son her age; I would never, ever do anything I wouldn’t want done to my own child.” He added, “I couldn’t live with myself knowing that I plowed through when I knew I should have stopped.” Jessica and Peter kissed their sleeping little girl goodbye, then she was wheeled away into the operating room.

The surgery was dramatically successful. Ana’s seizures stopped within 24-48 hours after her surgery. Jessica was overwhelmed with gratitude. She told Dr. Schneider she wished there was something she could do for him. He told her the best thing she could do for him would be to get the word out about this kind of surgery to help other people who might benefit from it.

Ana is now fully recovered and like her old self—before the seizures began.
Her mother said, “She’s just wonderful.”