Second Opinion Leads to Dystonia Diagnosis, Botox and a Road to Independence
Looking at Beth Oringher now, you would never suspect that, at one point, she couldn't lift a spoon to put food into her mouth or confidently pour a glass of lemonade.
Before botulinum toxin injections controlled the shaking in Oringher’s hands and head, she avoided public places at all cost. Her jerking motions were frustrating and embarrassing.
"I didn’t socialize, and I wouldn’t go out with anyone unless they were a really close friend," the 67-year-old said. "It was horrible. I hated it. I hated it. I hated it."
The solution came in the form of controlled injections that relax excessive muscle contractions, relieving her symptoms. By using electromyography equipment, experts at Cedars-Sinai were able to measure activity in the muscles to help locate the exact point of nerve disorder.
"This type of treatment has the ability to enhance a patient’s quality of life and is the difference between shaking or not shaking, not being able to use your hand, or walk," said Michele Tagliati, MD, director of the Cedars-Sinai Movement Disorders Program.
Oringher was an elementary school teacher in her early 30s when the muscle contractions started in her left hand. She taught at a time when chalkboards were in every classroom and cursive was an art form, and she took time to comment on every student’s report cards. At home, she was in charge of writing checks and personal notes inside birthday cards.
When formulating neat letters became fatiguing and stressful, Oringher taught herself how to write with her right hand. She was able to get by with good penmanship, but signing her name was another beast."
The shaking progressed until it finally took over one afternoon in 2010, when she was at the DMV counter and her hands were moving so fast she couldn’t fill out a form.
"I got into my car and broke down. I was hysterical," Oringher said. "I called my internist and said, ‘I need the name of a neurologist. I can’t do this.’ At that point it became too much. I had to do something."
Oringher found herself in the hands of a neurologist who diagnosed her with essential tremor, with early signs of Parkinson’s disease, a diagnosis she felt was a "death sentence." Treatment included heavy doses of medications that slowed the tremors but left her sleeping all day.
When all six drugs were combined, Oringher said she "felt like a zombie. I still had the tremors, and then my head started shaking a mile a minute. Then my voice became weak, almost breathy."
At that time, Oringher’s sister and the pediatrician she was working for received an invitation for a meet and greet with Tagliati, who had just joined Cedars-Sinai. They insisted Oringher meet the neurologist.
During a 2-½ hour appointment, Tagliati had Oringher talk, walk, hop, skip, write and draw circles, triangles and numbers.
Tagliati then delivered life-altering news: Oringher did not have Parkinson’s disease or essential tremor. Instead, she had dystonia, a movement disorder that causes involuntary contractions and spasms of the muscles.
The new diagnosis opened different therapeutic options, including changing her medications and using botulinum toxin injections, which are most effective in patients with dystonia to slow down overactive muscles.
"If Beth did have essential tremor, she would have been very responsive to the medications, but the fact that she wasn’t was an indication that something else was happening," Tagliati said. "We made sure to ask the right questions and observe Beth while listening to her. All of this helped determine what Beth needed at the end of the day, and that was the injections."
Botulinum toxin injections may be familiar for cosmetic use — getting rid of wrinkles — but they have been used primarily to help patients with dystonia and spasticity, in which there are involuntary muscle movements because of damage to the portion of the brain or spinal cord that controls voluntary movement. Spasticity can occur in a person who has had a stroke, multiple sclerosis, traumatic brain injury or cerebral palsy.
The injections have also been successful for patients who attend the weekly Spasticity Clinic at Cedars-Sinai. The clinic — which takes place every Friday from 8 a.m. to noon — provides relief to patients who need assistance with tightened muscles. The botulinum toxin injections help patients improve their ability to move, and — combined with physical and occupational therapy — enable them to increase their flexibility as they move their arms and legs and stretch their bodies.
"We have patients who, obviously after a devastating stroke or traumatic brain injury, are unable to walk, move or feed themselves," said Echo E. Tan, MD, a neurologist at Cedars-Sinai. The injections can allow a patient to open their fist so their palms can be cleaned, which can prevent skin erosion, and their nails can be clipped to prevent them from cutting into their palms.
In addition to their medical benefits, the injections allow patients to feel independent.
For Oringher, the new diagnosis and botulinum toxin treatments were "a breath of fresh air." She receives injections to her arms and the back of her neck every three months. She can see the shaking in her hands reverse within 48 hours and can project her voice within 24.
"Nothing comes before my appointments with Dr. Tagliati. He’s such a wonderful doctor, and he’s so compassionate," Oringher said. "I have so much faith in him that I can’t think of a word beyond trust. And the injections — they are amazing. I’ll never stop doing it."
Oringher hasn’t fully given up her pen, though she prefers a keyboard: She continues to sign her grandchildren’s birthday cards after her husband has written a thoughtful note on their behalf. She has also returned to an active social life. She now takes her Shih Tzu, Lola, out for walks and is even making plans to travel.