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 seizuresincluding her pediatrician. He chalked up the childs aberrant behavior to temper tantrums. A few months later, Anas 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 Jessicas behavior following the seizures because, Jessica explained, She would be kicking, screaming, bitingshe 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. Thats when some pieces of the puzzle started to come together. Jessica said it wasnt until then that they began to realize, OK, she doesnt know how to do a lot of things. Ana couldnt jump, she couldnt 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 didnt 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 herit wouldnt 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 dont 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 doctors 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 girls mother called Jessica on the phone and on her recommendation, Jessica and Peter took Ana to Dr. Schneider.
Dr. Schneider looked at Anas MRIs and immediately recommended surgery. He told Jessica and Peter that the kind of seizures Ana was havinglaughing, or gelastic seizureswere 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, Im not going to lie to you; this surgery is a risk. Theres a chance she could die on the operating table. He then explained that if she didnt 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. Anas tumor was in her hypothalamus, which controls body temperature, thirst, and hunger but also emotionsincluding 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 techniquesimilar to a global positioning systemto guide him, literally, to the center of the brain. He said, You dont 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, Everythings going to be great! Dont worry about it. Jessica asked if he would come down and let them know how the surgery was going. He said, Nono 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 wouldnt want done to my own child. He added, I couldnt 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. Anas 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 selfbefore the seizures began.
Her mother said, Shes just wonderful.