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SALT LAKE CITY — Taryn Hoffman, 35, started noticing distortions and twisting in her muscles 12 years ago. Three years later, doctors diagnosed the condition as dystonia, a neurological disease that produces radical and often immobilizing contractions of the muscles.
Victims of the disease suffer from cramps and shaking. They're often bent over because muscles will no longer hold the body in an upright position. The neck may turn or pull involuntarily, and spasms will sometimes cause the eyes to close.
Hoffman had a form of the disease that mostly affected her neck and facial muscles. The neck and head would abruptly bend to one side, and her face would distort and grimace, almost as if she had been hit with some kind of paralysis.
As a resident of Boise, Hoffman worked in the health care industry, and though her employer understood the condition, others did not. One day, the spasms hit while she was helping a patient. The patient, believing she was an addict, told her she might want to lay off the drugs.
"I burst into tears because I couldn't believe how hurtful people can be when they don't understand," Hoffman said. "I said (to the patient), 'Please don't judge me. I have a disorder that causes my body to do things that I don't want it to do. And I don't mean to offend you, but that was not a very nice thing to say to me.’ ”
Hoffman battled her disease for nine years before hearing about deep brain stimulation (DBS). It's a procedure where doctors implant a pacemaker-like device just under the skin. Electrical impulses move up small wire leads to precise areas on both sides of the brain where short circuits interfere with normal muscle movements.
"Deep brain stimulation involves implanting electrodes within certain areas of your brain. These electrodes produce electrical impulses that regulate abnormal impulses. Or, the electrical impulses can affect certain cells and chemicals within the brain. The amount of stimulation in deep brain stimulation is controlled by a pacemaker-like device placed under the skin in your upper chest. A wire that travels under your skin connects this device to the electrodes in your brain."
-Source: MayoClinic.org
"Deep brain stimulation, in theory, comes in and normalizes that extra noise that's blocking the ability of the normal, voluntary movement to happen," said Dr. Lauren Schrock, a neurologist at the University of Utah.
For Hoffman, the DBS implant at the University of Utah's Clinical Neurosciences Center has altered her life dramatically.
"(I'm in) absolute, complete disbelief; just utter shock," Hoffman said. "This is what it's like to feel normal."
As we watched, Hoffman demonstrated the remarkable effect of DBS. Using a handheld remote, she turned off her implanted device. In less than five seconds, the twisting of the neck muscles, the facial distortion and grimacing returned. When she turned the device back on, the symptoms disappeared.
With the implant, Hoffman does anything she wants because the spasms — "posturing" as it's called — are blocked.
"I was very self-conscious about myself," Hoffman said, recalling her life before DBS. "I wouldn't want to go out. I wouldn't want to do things. I wouldn't want to be social at all."
The initial surgery for the implant takes four to five hours, and during part of that procedure patients remain conscious. They have to respond when stimulated through small openings in the skull to make sure the permanent probes are precisely located where they'll do the most good. Even after surgery, some patients discover the right stimulation or waiting for the brain to make its own adjustments may take time.
Related:
Brain stimulation controls tremors caused by Parkinson's
Doctors are becoming increasingly encouraged with an implant that's eliminating tremors from Parkinson's disease."In a sense, we believe it changes or remodels the brain and causes a change in plasticity," Schrock said.
University of Utah Clinical Neurosciences is now one of the country's major centers for DBS, performing two to three procedures each week. From Parkinson's disease, tremors, obsessive-compulsive disorders, dystonia and more, DBS appears to block symptoms in different ways.
"In some scenarios you may want to block abnormal patterns, but in other situations you may be trying to enhance normal patterns," explained Dr. Paul House, a neurosurgeon at the University of Utah.
Not all patients respond the same, and not all do as well as Hoffman. DBS allowed her to go off medications — some of which she believed were too risky for pregnancy.
But what a change after the implant. No longer needing medications to control the disease, Hoffmann got pregnant and gave birth to a baby girl, who joined her adopted brother as an addition Hoffman and her husband thought they would never experience.
To family, the Neurosciences Center, her support group and many others, Hoffman expresses a big thanks.
"They are a huge part of my life," she said.
The data for DBS implants looks better and better with each passing year. Down the road, U. researchers could become participants in trials testing the procedure to control depression and other neuropsychiatric disorders.
