When Shaun Ryan was 8 years old, his mother, Rose, noticed him making a few facial gestures she hadn’t seen before. He would squeeze his eyes shut repeatedly or grimace at odd moments.
She didn’t think much of it until his irregular movements grew markedly worse during a family vacation. Something was wrong. The Ryan family cancelled the rest of their holiday and embarked on what would become a six-year struggle to find help. They found the answer at North Shore University Hospital (NSUH).
Trapped by Tourette's
Shaun had Tourette’s syndrome — a neurological disorder in which irregular brain signals cause involuntary movements, or tics. Symptoms can be mild or, as in Shaun’s case, very severe.
Shaun’s symptoms quickly progressed. He experienced constant tics and could no longer sit still. Sometimes he spun in circles. His arms jerked outward unexpectedly. He grunted loudly. His eyes rolled back for an hour at a time. Shaun even began involuntarily swearing — a rare symptom of Tourette’s.
“He knew what was happening, but couldn’t stop it,” explained Ms. Ryan. “The only time Shaun was free from his tics was when he slept.”
Hope of Relief
Shaun saw countless neurologists throughout his childhood. He also tried numerous treatments, including medication, Botox injections into his vocal cords and behavioral therapy.
“Some therapies worked for a short time, but never lasted,” said Ms. Ryan. “After we had exhausted every possible option, our neurologist suggested we meet with experts at North Shore University Hospital.”
Shaun’s neurologist had heard NSUH neurologists deliver a lecture on deep brain stimulation (DBS) for patients with Tourette’s.
Understanding Deep Brain Stimulation
Although it’s not fully understood how DBS works, it can be thought of as something like a pacemaker for the brain, resetting abnormal rhythms to get brain circuitry working in a more normal manner. A generator implanted under the collarbone sends signals to electrodes placed in the brain; a medical specialist can adjust the electrodes.
Using DBS to treat Tourette’s syndrome is still very rare. The therapy is FDA-approved only for Parkinson’s disease, tremors and dystonia. However, research shows that DBS can offer excellent outcomes for some Tourette’s patients.
Alon Mogilner, MD, PhD, chief of functional and restorative neurosurgery, and Michael Pourfar, MD, movement disorder specialist of North Shore-LIJ’s Harvey Cushing Institutes of Neuroscience, collaborate to provide DBS therapy to patients. In the past seven years, they have treated hundreds of adults using DBS.
“DBS isn’t appropriate for the majority of children with Tourette’s syndrome because many do not have severe enough symptoms to warrant brain surgery or they gain adequate control with oral medications. In addition, many may outgrow the disorder on their own,” said Dr. Mogilner.
Until meeting Shaun, Drs. Mogilner and Pourfar had not offered DBS to a child with Tourette’s for these reasons. However, after carefully evaluating Shaun for six months, they concluded that it might be his only hope for a normal life given the severity of his symptoms and the number of medications and treatments he had already tried.
A Life Reclaimed
Dr. Mogilner performed the complex surgery to implant the devices. Dr. Pourfar worked closely to adjust the signals sent to the brain.
“There are thousands of different ways to program this technology,” explained Dr. Pourfar. “It’s as much an art as it is a science. That’s why a team with strong experience in DBS is so important.”
Shaun’s surgery was one year ago. Today, he’s a different person.
“People who meet Shaun for the first time can’t tell he even has Tourette’s. It’s amazing,” said Ms. Ryan. “His tics are virtually gone. He now has his driver’s license and goes to school full-time. He’s even an emergency medical service volunteer and junior firefighter volunteer. Shaun never let the syndrome stop him. Drs. Mogilner and Pourfar gave him his life back.”
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