A dentist's viselike pain signaled a nearly invisible disorder

The Washington Post illustration

For more than a year, Tara Zier waited in vain for the viselike tightness in her chest and the pain in her neck to subside.

The life of the Northern Virginia dentist had imploded in October 2014 when her former husband, the father of her children who were then 11 and 13, killed himself. The shocking act unleashed a cascade of investigations into the investment firm executive and his financial problems.

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Initially, Zier thought her pain might be the product of intense stress coupled with grief. What she couldn't understand was why nothing she did seemed to help and why her neck pain, an occupational hazard among dentists, continued to worsen.

But when she consulted her internist, Zier said he "blew it off." He advised Zier to stop focusing so intently on her health and take an antidepressant.

A similar message would be delivered by several of the many specialists who examined her heart, lungs, spine, neck and brain between 2015 and 2017, as Zier underwent tests and procedures that sought to uncover the origin of her confusing symptoms.

In October 2017, a neurologist, the first Zier had seen, ordered the blood test that identified the little-known malady that had proved so elusive. Because anxiety is often a prominent feature of the disorder, its presence is often misinterpreted, further complicating the diagnostic process.

Zier quickly found that identification was merely the first step. Learning to manage the problem would prove equally challenging.

"It's more of an invisible illness," said Zier, now 50, who lives in a Maryland suburb. "If you looked at me, you wouldn't know I had it."

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In January 2015, Zier was driving back from Vermont with her children after a snowboarding trip she hoped would boost their spirits when she pulled to the side of the Garden State Parkway. Zier had been battling a bad case of bronchitis for several days; increasingly short of breath and dizzy, she felt too sick to drive the rest of the way home. She spent the next four days in a New Jersey hospital where she was treated for pneumonia.

As the year progressed, Zier continued to feel unusually fatigued. At times she had trouble catching her breath. Zier thrived on vigorous exercise and had earned a third-degree black belt in karate, but could scarcely drag herself through a workout. Her chest felt constricted and her neck ached.

But tests of her lungs and heart were normal and multiple MRI scans of her spine and neck found nothing that would account for her symptoms.

In January 2016, Zier saw her internist, who diagnosed severe psychological stress. She remembers that he commended her for "holding up remarkably well" and advised her to take the anti-anxiety medication he had prescribed following her ex-husband's death.

Zier thought physical therapy might help; instead, it made her feel worse. Several sessions of cognitive behavioral therapy with a mental health counselor were not helpful. Neither was a series of steroid injections in her neck administered by an anesthesiologist. A cardiologist, a neurosurgeon and an otolaryngologist found nothing amiss.

In January 2017, Zier's internist prescribed an antidepressant, noting that she was "very anxious about her health and fears she will have a catastrophic illness."

Both statements were true, Zier said. No doctor seemed to know what was wrong and some made it clear they doubted her problem was physical. Uppermost in her mind, she said, was the awareness that her children, who had been devastated by their father's suicide, had only one parent.

"Nothing can happen to me," she remembers thinking frequently.

A few weeks later, Zier was treated in the emergency room of a Maryland hospital for excruciating neck pain that was diagnosed as muscle spasms.

The ER doctor prescribed diazepam, the widely used (and abused) sedative commonly known as Valium, along with a short course of corticosteroids.

For the first time in many months, Zier recalled, "I actually felt better." But the pain quickly returned once she stopping taking the drugs.

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Soon afterward, she saw a second cardiologist and a psychiatrist; both told her she was suffering from chronic anxiety. The psychiatrist, whom she saw five times, noted her plethora of doctors' appointments and suggested Zier spend time "not being a patient."

It was advice she found insulting and frustrating.

"I'm not dismissing a mental health issue at all," Zier said, "but there was something wrong with my body."

In March 2017, she stopped practicing dentistry, unable to keep up with the demands of the profession she loved. She found a new primary care doctor, a rheumatologist she considered helpful and sympathetic. She also saw an endocrinologist, a radiologist and a specialist who diagnosed and treated her for a disorder of the autonomic nervous system, which regulates heart rate, blood pressure and body temperature.

In October 2017, after reporting sudden numbness on the side of her face along with worsening vertigo, her internist sent her to a Washington neurologist.

"I thought he was going to blow me off," Zier recalled, "but he listened to me." The neurologist ordered an MRI of her brain along with a slew of blood tests.

Among them was a test to measure the level of antibodies to glutamic acid decarboxylase (GAD), a protein involved in the production of an important neurotransmitter in the brain. The neurotransmitter, known as gamma-aminobutyric acid (GABA), helps control muscle movement and the regulation of anxiety.

The GAD antibody test is used in the diagnosis of diabetes and epilepsy and the detection of a rare, progressive neurological condition called stiff-person syndrome (SPS), which affects the brain and spinal cord, causing alternating rigidity and painful muscle spasms along with a heightened sensitivity to stress, noise or other stimuli. Most people with SPS have elevated levels of anti-GAD antibodies. Some studies have found they also have lower levels of GABA, which may help explain their persistent anxiety.

"All your tests came back normal, except one," Zier remembers the neurologist telling her. Her GAD65 antibody level measured 25,000 units per milliliter (U/mL); normal is less than 5 U/mL.

The neurologist told Zier he believed she had SPS, although he had seen only one other case in his nearly 40-year career.

SPS was first identified in 1956 by doctors at the Mayo Clinic and is believed to affect roughly 1 person in 1 million, most of them women who have an autoimmune disease such as Type 1 diabetes. (Zier has Graves' disease which causes an overactive thyroid.) The disorder may result from a faulty immune response; it is frequently misdiagnosed as Parkinson's disease, multiple sclerosis, severe anxiety or less often agoraphobia.

Hunched over postures are characteristic of SPS and some people can become too disabled to walk. Others are afraid to leave their homes because the sound of a car horn can trigger violent, unpredictable muscle spasms resulting in a fall.

The neurologist prescribed Valium and baclofen, a muscle relaxant, both of which are mainstay treatments for SPS.

"I was relieved I had a diagnosis," Zier said, "but scared about what it meant."

- - -

Zier's internist helped her secure an appointment at the Mayo Clinic for a consultation. She spent a week in Minnesota undergoing additional tests that confirmed the SPS diagnosis and attended Mayo's pain management program.

Zier ultimately opted for treatment closer to home at Johns Hopkins's Stiff Person Syndrome Center, believed to be the only one of its kind in the world.

For the past few years she has seen Scott Newsome, the neuroimmunologist who heads the center.

Although Zier was diagnosed far more quickly than most people with SPS - the average delay after initial symptoms is about seven years - her experience is fairly typical.

"Early on, people are dismissed and it's thought that they have a mental health issue or are exaggerating," observed Newsome, an associate professor of neurology who estimates that he has seen about 200 people with SPS. Long-standing anxiety, he said, appears to be intrinsic to SPS and has been present in every patient he has seen.

Zier does not have the contorting symptoms caused by muscle spasms that can occur in severe cases. But, Newsome added, "most people [with SPS] are disabled and their lives are turned upside down."

Treatment is aimed at alleviating symptoms, improving mobility and slowing progression of the condition, which has a variable and unpredictable course. In addition to medications ranging from muscle relaxants to immunotherapy, recommended treatments include aqua therapy, stretching and cognitive behavioral therapy.

In the past few years, Zier's condition seems to have improved, although pain is a constant. "Every day is a struggle," she said. "I'm happy I'm breathing and that my dog's on the couch with me."

Zier recently founded the nonprofit group Stiff Person Syndrome Research Foundation, which seeks to raise awareness of SPS along with money for research.

She remains distressed by the skepticism she initially encountered, an experience that appears to be common among those who share her diagnosis.

"The stress that goes on with a diagnostic odyssey is unreal," she said. "I'm not an easily offended person, but to be dismissed over and over was very tough."

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