When singer Celine Dion was diagnosed with stiff person syndrome, Melissa Meyer was sad but relieved.
The North Phoenix resident said she didn’t feel so alone. Meyer and Dion suffer from the syndrome, a rare, progressive neurological disorder. Symptoms may include stiff muscles in the torso, arms, and legs; and greater sensitivity to noise, touch and emotional distress, which can set off muscle spasms.
“I had mixed emotions,” she said. “I felt really sad for her. In her video, it seemed like she had so much emotion and hope. I remembered what it was like to be there. There’s just so much unknown about it because it’s rare.”
Stiff person syndrome affects twice as many women as men, and is frequently associated with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo and pernicious anemia. Scientists don’t yet understand what causes stiff person syndrome, but research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord.
Stiff person syndrome is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia. A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies.
“I burst into tears when I heard the news about Celine Dion and SPS,” said Dr. Tara Zier, founder/chief executive officer of The Stiff Person Syndrome Research Foundation and SPS patient.
“I had heard she was struggling to find a diagnosis, but I hoped it would not be SPS. We actually reached out to her on Instagram one month ago to offer resources, because her symptoms sounded all too familiar. For me, it’s a mix of shock and really deep empathy. Listening to her talking about her kids supporting her, empathizing with how lonely it can feel and how hard it is when you can’t participate in what you love doing really resonates with me.”
Zier said she is moved by Dion’s positivity.
“When you’re facing an incurable disease, it can feel hopeless. But Celine will give thousands of patients hope. Maybe now people will take the disease seriously. Part of the reason the average time to diagnosis is an agonizing seven years is that stiff person syndrome has a ridiculous name. But it is a debilitating disease. People aren’t just stiff. It impacts vocal cords. Breathing. Walking. Going out in public. It’s impacting Celine’s life and every patient who suffers from SPS can relate to that.”
Meyer can recall symptoms as far back as her 20s. On her father’s 60th birthday, the family traveled to Vegas. While she was there, her legs felt heavy and stiff. She couldn’t figure out what was wrong with her.
Meyer was diagnosed three years ago, on Dec. 23, 2019, after she started feeling weak. She regularly carried 4 gallons of water upstairs without issue. Eventually, she had to bear hug 1 gallon.
“I was in a doctorate program. I was stressed and tired,” she said. “I had some joint pain. I just didn’t feel right. I couldn’t pinpoint what was going on. I saw a neurologist and he said, ‘I don’t know why you’re in my office.’”
In her late thirties at the time, she saw a rheumatologist and he chalked the symptoms up to aging.
“I was ready to give up, which isn’t uncommon for people with stiff person,” she said. “You start to wonder if the symptoms are actually happening.”
As a student, she met someone during an internship who referred her to a neurologist. He ordered lab work and, after three months, she had her troubling diagnosis.
Meyer called the last three years a “bit of a journey.” Meyer, who works as a psychiatric nurse practitioner for children, has fleeting excruciating muscle spasms, while others have rigid muscles, broken bones due to falls or dislocations, or are bedridden.
“The spasms are in my core,” she said. “Sometimes they’re in my limbs. I have the random eye twitch that likes to happen in the middle of telemed sessions. The weakness has returned.
“It’s so difficult. My worst flare up was a couple months after the initial diagnosis, in June 2020, right around the height of COVID. It was the most excruciating pain. It felt like someone was grabbing my core and crushing as tight as they could. It lasted 14 hours.”
Several symptoms improve with oral diazepam (an anti-anxiety and muscle relaxant drug) or with medication that alleviate muscle spasms, such as baclofen or gabapentin.
A study funded by the National Institute of Neurological Disorders and Stroke (NINDS) showed that intravenous immunoglobulin (IVIg) treatment is effective in reducing stiffness, sensitivity to noise, touch, and stress and for improving gait and balance for people with SPS. IVIg contains immunoglobulins (natural antibodies produced by the immune system) derived from thousands of healthy donors.
Meyer is undergoing subcutaneous immunoglobulin therapy. She also kickboxes three times a week.
“Balance is a big issue with stiff person syndrome,” she said. “I’m very careful. I can’t do heavy weights or else I can cause a flare up. I had physical therapy, but I had a flare up right after. I’m too nervous to go back. The kickboxing is working well for me.”
Psychologically, stiff person syndrome can be hard.
“It’s tough for people who don’t get diagnosed for a long time,” said the 43-year-old former Scottsdale resident. “You really do start to wonder where the symptoms are coming from. I see a counselor, who helped me accept the diagnosis for what it is. I never know what tomorrow is going to look like and that’s OK.
“I’m controlling what I can and accepting what I can.”
Stiff Person Syndrome