Celine Dion revealed Thursday that she has been diagnosed with stiff-person syndrome, a rare and progressive neurological disorder. The diagnosis has forced her to reschedule or cancel the 2023 shows for her “Courage World Tour.”
The 54-year-old Canadian superstar shared the news in a pair of emotional Instagram videos—one in English and one in her native French.
“I’ve been dealing with problems with my health for a long time, and it’s been really difficult for me to face these challenges and to talk about everything I’ve been going to,” she told fans. “These spasms affect every aspect of my daily life, sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to.”
Here’s what to know about stiff-person syndrome, including how the disorder is diagnosed and managed, and the effect it can have on daily life.
Stiff-person syndrome—sometimes known as SPS or Moersch-Woltman syndrome—is a disorder that experts are still working to understand, though research indicates that it has an autoimmune component. It’s been frequently associated with other autoimmune diseases, such as type 1 diabetes, thyroiditis, vitiligo, and pernicious anemia.
It’s estimated that fewer than 5,000 people in the US have the disorder, and symptoms typically appear during adulthood (ages 19–65). Women may also be disproportionately impacted, with stiff-person syndrome affecting twice as many women as men.
Stiff-person syndrome affects the nervous system, specifically parts of the brain and spinal cord that regulate movement. As a result, patients’ muscles contract and stiffen excessively—sometimes so severely that the spasms can fracture bone.
“It typically involves the back and the lower extremities,” Andrew McKeon, MD, professor of neurology at the Mayo Clinic and board member at the Stiff-Person Syndrome Research Foundation, told Health. “But there can be other muscle groups involved in some patients.”
Other symptoms of stiff-person syndrome may include: anxiety, hyperhidrosis difficulty walking, rigidity, and hypothyroidism.
People with stiff-person syndrome may also have “exaggerated startle responses,” said Dr. McKeon—this means they may be extra sensitive to noise or touch, which can then elicit a response putting them at an even greater risk of injury or falls.
Rare diseases like stiff-person syndrome are notoriously difficult to diagnose. Sometimes, a single symptom may be related to various other diseases, or healthcare providers may not have enough experience or knowledge about the disease.
“We have a tough time making this diagnosis a lot of the time,” said Dr. McKeon. “The exam findings can fluctuate, they may or may not be there at certain times in the patient.”
Stiff-person syndrome is commonly misdiagnosed as Parkinson’s disease, multiple sclerosis, or fibromyalgia, among others. It’s usually only after testing a person’s blood for glutamic acid decarboxylase (GAD) antibody levels that a true diagnosis can be made. (Most patients with stiff-person syndrome have higher GAD antibody levels.)
With a proper diagnosis and appropriate treatment—drugs that relax muscles and alleviate spasms, as well as antibody treatments for the immune system— stiff-person syndrome can be managed.
“Typically, stiff-person syndrome is a treatable disorder, but a chronic disorder,” said Dr. McKeon. “M patients continue to work. Many patients continue to have very normal family and social activities once they are treated.”
Though a small minority of people do experience a progressive form of disease that doesn’t get better with treatment, people in treatment for stiff-person syndrome either improve or their symptoms remain managed.
In Dion’s case, though she didn’t go into details about her treatment program, she said she is “working hard with [her] sports medicine therapist every day to build back [her] strength and [her] ability to perform again.”
In her video, Dion said she feels hopeful that she’s on the road to recovery, and that in order for her to get back to performing, she must concentrate on her health at this moment.
“I have to admit, it’s been a struggle. All I know is singing—it’s what I’ve done all my life and it’s what I love to do the most. I miss you so much, I miss seeing all of you, being on the stage, performing for you,” Dion told fans. “I’m doing everything that I can to recuperate.”
As Dion goes through treatment for the physical side of stiff-person syndrome, it wouldn’t be uncommon to tend to her mental health as well.
“Patients with stiff-person syndrome—as with any patient with a neurologic disorder—can develop problems such as anxiety or depression,” said Dr. McKeon. Treatment for the disorder may also have an impact on mental health.
But Dion’s decision to be open about her diagnosis may also be helpful to stiff-person syndrome community—and has already had an impact on other patients.
“It’s a mix of shock and really deep empathy,” Tara Zier, DDS, founder and CEO of the Stiff-Person Syndrome Research Foundation told Health in a statement. “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.”
“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,” Zier, who is also a stiff-person syndrome patient, said in a statement. “Celine’s SPS diagnosis is going to wake people up. Today. She is already using her beautiful voice to shine a bright light on an often-misdiagnosed but devastating disease.”
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