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Savannah Salazar went to bed one night in 1995 as a typical toddler. At two and a half years old, she could count to three and knew most of her colors, although she still mixed up black and brown. As far as her parents could see, she was developing pretty much the same way her four-year-old brother had.

And then, in the middle of that night, everything changed. Her parents, Ruben Salazar and Tracy Dixon-Salazar (who is now Dr. Dixon-Salazar), awoke to a sound every parent dreads: their daughter was choking. “She was gagging, hacking, and making frothing noises,” Dr. Dixon-Salazar recalls. “I thought my child was dying. By the time the paramedics came, Savannah was okay. But I'll never forget what one of them said next: ‘Her airway is clear, but what you've just described sounds like a seizure.’”

Savannah didn't go to the hospital that night. Her exhausted parents tried to go back to bed. But when Savannah had another seizure a couple of weeks later—and a total of four within a period of two months—the family embarked on a seemingly endless roller coaster of scans, tests, and doctor visits: blood tests, magnetic resonance imaging (MRI), computed tomography (CT) scans, electroencephalography (EEG)—but they all came back normal.

For a while, it looked like the seizures were going away as mysteriously as they had appeared. “All of the seizures occurred in the middle of the night, when Savannah was sleeping,” says Dr. Dixon-Salazar. “After those first four seizures, she went six months where she didn't have any seizures. I don't think we missed any because she was sleeping with us at that point. We were hypervigilant, and the seizures were hard to miss.”

But then they came back. And they got worse. Much worse.

he first few had been tonic-clonic seizures, which often begin with a stiffening of the limbs (the tonic phase) followed by jerking movements (the clonic phase). But then Savannah began having hundreds of seizures of different types in a typical week.

MORE THAN SEIZURES

For two years, the Salazars had no diagnosis for Savannah's condition. “We knew she was having these spells, but all her eight EEGs were normal, and the doctors were reluctant to be definitive about what her condition was without better information,” Dr. Dixon-Salazar says. Apart from the patient history and the neurologic examination, the EEG is the most influential tool in the diagnosis of seizures and epilepsy. It provides a record of ongoing electrical activity in the brain.

When Savannah was five, she started kindergarten, and her parents began to realize that more was wrong than just the seizures. “My son couldn't read when he started kindergarten, but by the end he was reading little books with rhyming words and sight words,” says Dr. Dixon-Salazar. “But we saw that Savannah just wasn't adding to what she could do. When she got around her peers and people started measuring progress more, we realized that she wasn't typical.”

The delays, along with new changes on Savannah's EEG, finally helped to identify her condition as Lennox-Gastaut syndrome (LGS), which has three primary defining characteristics: multiple seizure types, a distinctive brain wave pattern that doctors call a “slow spike-and-wave,” and mental and developmental delays that can range from mild to profound. It usually develops between about 3 and 8 years of age.

“It is one of the severe pediatric epilepsies,” says Jacqueline French, M.D., director of the Epilepsy Study Consortium, professor of neurology at New York University Langone Medical Center, and Fellow of the American Academy of Neurology (AAN). “There are a number of them, unfortunately. Lennox-Gastaut syndrome is a group term.”

Sometimes LGS has a structural cause. For example, children with lissencephaly, a rare brain disorder in which the folds in the surface of the brain (otherwise known as gyri) fail to develop, can go on to develop LGS. The condition can also develop after birth trauma, head injury, or exposure to toxins (such as maternal consumption of alcohol during pregnancy leading to fetal alcohol syndrome). Infantile spasms, which typically begin in babies less than a year old, can also sometimes progress to LGS.

But none of those things were true of Savannah. The mystery of what had happened to her daughter haunted Dr. Dixon-Salazar. She began a quest for answers. “I couldn't wrap my brain around the fact that nobody could tell me why one day my daughter was absolutely fine and the next day she was not fine. I had to know,” she says.

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CURE For questions, please contact the CURE office, 312.255.1801, or email info@cureepilepsy.org.

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