Dystonia is a movement disorder in which muscles spasm
Approximately 250,000 Americans have dystonia
If you’re scanning through radio stations, you may soon hear a familiar but oddly mechanical voice reporting the latest news from Capitol Hill.
Cox radio veteran Jamie Dupree is back on the air Monday after a two-year absence. But the melodious tones that graced the airways for over 30 years sound a bit different today.
Dupree isn’t speaking. Instead, he is typing into a text-to-speech application that uses years of his archived audio to create an artificial voice. He calls it “Jamie Dupree version 2.0.”
“It will be a computer-generated synthesis of my voice,” he told audiences, announcing his return.
A bit robotic, yes, but for Dupree, it’s an answer to a prayer. He abruptly lost the ability to speak in spring 2016, and it hasn’t come back.
Jamie Dupree is the radio man without a voice.
‘The sound would just crack in half’
In March 2016, Dupree was taking a well-deserved break from the grind of covering the presidential campaign. In London on vacation with his wife and three children, he was struck down by a stomach bug.
His health didn’t improve when he got home. Constant diarrhea, a racing pulse and general malaise plagued him. Doctors couldn’t pinpoint the cause; that would soon become a common refrain.
The oddest symptom was his voice. It had suddenly become breathy, squeaky, unreliable.
“I would try to say regular words, and the sound would just crack in half,” Dupree wrote in an email, his preferred method of communicating.
Over the next few months, his speech got worse. When Dupree opened his mouth to talk, his tongue would thrust out against his will. The more he tried to talk, the more it protruded, making it impossible to express himself.
“The first couple of words that I say are fine,” Dupree explained, “but as I try to string together a sentence, then the tongue becomes more prominent and the throat clenches, leading to a strangled, strained sound of my voice.”
Trying to whisper produced the same effect, but it didn’t affect his ability to eat or drink. It was like his tongue had a mind of its own.
The search for answers
A merry-go-round of doctors finally found relief for his stomach and heart issues but the most precious thing – his voice – continued to elude them. As did the cause of all his symptoms.
“Like my voice – no one had any answers as to WHY it was happening,” Dupree wrote. “Why was my heart going so fast? Why was my food moving so fast? Something in my body was not working right. But no one could tell me why.”
The Mayo Clinic rejected his appointment application. A week after the Trump inauguration, a doctor at another prominent hospital injected botulinum toxin into Dupree’s vocal cords, hoping to relax his voice.
“That was a personal disaster,” Dupree wrote. “The procedure did nothing for my voice – in fact, it took away what voice I had – and it then resulted in about 8 weeks of swallowing difficulties.”
A visit to the Cleveland Clinic put a name to his condition: a rare mandibular dystonia called lingual protrusion dystonia. It’s part of a group of movement disorders characterized by involuntary muscle contractions that cause strange, repetitive motions and postures. The most common form, cervical dystonia, affects the neck, causing wobbling or worse, the inability to hold the head upright. Blepharospasm, another kind, is a forced squeezing shut of the eyelids that reduces or blocks vision. Still other dystonias can focus on the legs, hands and feet, even the entire body.
Nearly 250,000 Americans have some type of dystonia, according to the American Association of Neurological Surgeons, making it the third most common movement disorder. Only essential tremor and Parkinson’s disease affect more people.
For Dupree, the relief of having a diagnosis quickly faded.
“[The doctor] told me … very few answers were available. He gave me no real guidance on other doctors to see,” he wrote. “That was a long drive home.”
A club no one wants to join
For a reason no doctor understands, focal dystonia often attacks the part of the body a person uses most for his or her profession. A nationally known radio talk show host, Diane Rehm, has a form of dystonia that affects her vocal cords. Trumpet players will suddenly be unable to use their lips. Guitar players, writers and pianists will lose control of fingers or an entire hand.
“How can you have a disorder where everything about the brain and the hand functions normally except the hand can’t perform one exquisite task like writing or playing a musical instrument?” asked New York neurologist Dr. Steven Frucht, director of the Fresco Institute for Parkinson’s and Movement Disorders at NYU Langone Health. A violinist and pianist himself, he treats many musicians with the disorder.
“You would think that this cannot happen,” Frucht said. “And yet after seeing hundreds of patients, I can say it most certainly can. And it does.”
“It’s precipitated by a voluntary movement. When they’re perfectly at rest, not speaking or playing, you can’t tell there’s anything wrong,” said Boston neurologist Dr. Daniel Tarsy, former chairman of the Movement Disorders Section of the American Academy of Neurology. “It’s often mistaken as having a psychological basis, which is not doing much of a service to the person who has one.”
It’s the brain’s fault
While it appears that it’s Dupree’s tongue and vocal muscles that are malfunctioning, the actual culprit is his brain.
“The problem is in the brain and how it controls the muscles,” said neurologist Dr. Hyder Jinnah, who is treating Dupree at the Emory Brain Health Center in Atlanta. “Exactly what’s going on in the brain that makes it do that, we’re not really sure.”
“When you look at the areas of the brain that are inappropriately activating, they’re not just one region,” Frucht said. “This is actually a widely distributed motor network disorder. It’s not like there’s a lesion in a three-millimeter location, and we could just take care of that.”
Doctors believe that there is a genetic component for dystonia, a predisposition that is triggered by an event in life. Like Dupree, many patients recall an illness, injury or stressful event and then “boom, the problem seems to have arisen,” explained Jinnah, who runs the Dystonia Coalition, a network of researchers and advocacy groups dedicated to researching the disorder.
“We’re tempted to say those things cause the dystonia,” he added, “but many people have those things every day, and they don’t have dystonia, so establishing a relationship between something like Jamie’s gastrointestinal illness and his dystonia is very hard to do.”
Tricking the brain
Focal dystonia often begins in one body part and then travels to an adjacent body part before settling in. In Dupree’s case, the problem began in his vocal cords and traveled to his mouth and tongue.
“I have no problems with it on anything except speech – and only on certain sounds, not all of them,” Dupree wrote.
Hard-to-say sounds include B, C, D, E, G, H, J, K, P, Q, T, U, V, W and Z, Dupree said. F, L, M, N and O are easy. A, I, S, X and Y can cause some problems. That suggests to doctors that his brain is targeting only a few of the many muscles in his tongue.
If he yawns, gags or puts a pen in his mouth, he can fool his brain a bit. Experts call it a sensory trick.
“If I talk through a yawn, I can get out about 7 words that are just fine,” Dupree wrote. “I’ve become quite an expert at talking through my yawns to say something. As soon as that yawn is over, then my voice is back to a struggle.
“I can get out a few more words by holding a pen in my mouth,” he continued. “It relaxes things a little in my throat, and allows out a little more speech. If I make myself gag, that opens my throat a little more and allows out some normal speech.”
One of Dupree’s most successful tricks is reserved for friends and family. If he raises his voice and tries to talk like a Jim Henson Muppet, he can complete entire sentences.
“You’re like the Swedish Chef!” a friend joked at a recent Atlanta reunion. “That’s right. Yurdy, yurdy, yurdy!” Dupree replied, laughing.
“If someone has a true sensory trick, it is dystonia, in my opinion. Nothing else will do this,” Frucht said. “It’s a bizarre thing that early on fed the fire of ‘this must be a psychological disorder, right?’
“It turns out nothing could be further from the truth,” he continued. “You can even show by sophisticated brain imaging that the application of the jest, or trick, or even thinking about the jest alters brain circuitry.”
Unfortunately, the brain often gets wise to sensory tricks, and they become less effective.
‘It’s like shooting ducks’
Treatments for dystonia are limited. Some medications can help distract the brain, and those are usually tried first, especially for focal dystonia. Deep-brain stimulation is a last resort for all but those with widespread dystonia in many body parts, which, sadly, are often children. Physical and speech therapy are usually a part of the treatment plan; acupuncture and medication may also help.
“A small number of people, maybe 5%, will find that it goes away just like it came, and we don’t really know why,” Jinnah said. “Most people have it for most of their life once it starts, and so we have to figure out ways to treat it.”
Injections of botulism toxin relax muscles; it is often the treatment of choice for some types of dystonia, such as blepharospasm.
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“It doesn’t treat the disorder; it treats their phenomena,” said Dr. Andrew Blitzer, an otolaryngologist at Mount Sinai in New York City. “We can’t change the brain signal coming in to the muscle, but we can weaken the muscle so it can’t go into spasm.”
Blitzer is a pioneer in the use of botulinum toxin for dystonia. Since giving “the world’s first botulinum toxin injection for dystonia in 1984,” he said, he has learned that the success of the treatment can vary “day to day and month to month” depending on the patient’s level of stress and the site of the injection.
“The brain on stress is worse, so it’s like shooting ducks, moving targets, because you’re always trying to anticipate how the patient is going to be,” he said.
Because there are so many variables, Blitzer recommends finding a doctor who is extremely familiar with the procedure and starting with small doses, increasing the level of toxin as needed.
‘I’m not giving up’
On a recent steamy day in Atlanta, Dupree relaxed onto a table in the Emory Brain Center for a botulinum injection. It was the first since the disastrous experience in 2016 that affected his ability to swallow. This time, the target of the injection was Dupree’s tongue, the dosage small.
“We’re trying to target the muscles that push your tongue out first, because that is the most annoying,” Jinnah explained. “First, I’m going to attach a little device that helps us listen to those muscles.”
Pointing to a spot in Dupree’s neck just under the jaw, Jinnah explained that the muscles that push the tongue forward are deep in the throat, so an injection in the neck is easiest.
“Ready?” Jinnah asked. After a thumbs-up, he inserted the small needle in one side of Dupree’s throat and then the other. “Hear that?” Jinnah asked Dupree. “That little rumbling noise is the muscle.”
Afterward, the two shook hands. “I really hope it works,” Jinnah said.
Weeks went by, and unfortunately, there was not much improvement. But there were also no side effects. It’s possible the amount of toxin was too small, so an appointment was set up for a stronger dose. In the meantime, additional medications can be tried.
“I knew there would not be a magic answer,” Dupree said. “It’s been a hard ride down, and it will not come up like …” He snapped his fingers. “I know that there is a voice in there. I’m not giving up.”