Celine Dion’s Stiff-Person Syndrome Is ‘a Really Difficult and Disabling Disease,’ Expert Says

Celine Dion has spent the past couple years battling health issues that have kept her off the stage. She pushed her 2022 tour to 2023, and then on Thursday announced that those European dates would have to be postponed too due to an exceptionally rare neurological condition called stiff-person syndrome. 

When Dion postponed the opening of her Las Vegas residency last fall, she cited “severe and persistent muscle spasms.” The same issue forced her to cancel her North American tour in Jan. 2022, too. As she shared in a new video on Instagram, Nov. 8, she’s since learned that stiff-person syndrome was causing those spams. 

“Unfortunately, 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,” Dion explained. 

While Dion didn’t divulge any further details, Dr. Scott Newsome, the director of the Stiff-Person Syndrome Center at Johns Hopkins Medicine, tells Rolling Stone that SPS spasms can completely upend a person’s life. 

“If you’ve ever had a charley horse, our patients can have those anywhere there’s a muscle, including the abdominal region and back,” he says. “And they can be continuous for minutes to hours. If you amplify it by 1000, or more, that’s what our patients are experiencing.” 

Newsome adds that these spasms can be intermittent, or be triggered by unpredictable events like loud noises. “We’ve had patients where they’re walking across the street, someone honks their horn, they go into a horrific spasm, and they fall,” Newsome says. “They can have head injuries, orthopedic injuries. It’s a really difficult and disabling disease.” (Dion did not say whether she was experiencing such triggered spasms, though as a musician, she is frequently surrounded by loud noises.)

Stiff-person syndrome, Newsome says, was first described in the mid-1950s at the Mayo Clinic. The classic symptoms described then — and which remain prevalent today — are muscular-skeletal, spanning from spasms to a fixed spine curvature deformity called hyperlordosis.

In the 70-odd years since then, neurologists and doctors have discovered that SPS, like many autoimmune conditions, exists on a spectrum. There’s SPS-plus, which involves the classic symptoms, plus “brainstem and/or cerebella involvement symptom signs,” such as wobbly walking, speech issues, and double vision. There’s even partial SPS, where symptoms might be experienced in just a single limb. 

While much has been gleaned about SPS since the 1950s, there’s still so much that isn’t known. For starters, an exact cause remains unknown. SPS, like other autoimmune diseases, does tend to affect more women than men, with a typical onset in middle age. Even still, Newsome calls it a “non-biased condition,” saying he’s seen it pop up in young children, as well as people in their 70s and 80s. (As for its rarity, the frequently touted stat is that SPS only affects one person out of a million, though Newsome thinks it’s probably a bit more common than that.)

Newsome also notes that SPS is diagnosed by exclusion, where other conditions that mimic SPS are ruled out. Blood tests can be done, and there is an antibody (GAD65) that frequently shows up in people with SPS; but even that’s only a tell if GAD65 antibody levels are extremely high and appear in the right clinical context. MRIs, CAT scans, and lumbar punctures can be useful in diagnosing SPS too, as well as electromyography (EMG) studies to examine how the muscles are behaving. 

“The best example I can give is when someone is contracting the bicep muscle, the tricep should relax,” Newsom3 says. ”In stiff-person syndrome, they’re contracting together, like they’re at war with each other. We can see that on an EMG.”

Just as there’s no definitely-known cause for SPS, there’s also no definitive cure — though Newsome hopes that by identifying “the main start of the disease within the immune system, we can then develop more targeted treatments.” Still, there are ways to treat SPS and alleviate symptoms, with Newsome suggesting a multi-faceted approach is ideal. This can include everything from muscle relaxants to immune-based treatments, such as intravenous immune globulin (IVIG) therapy, as well as non-pharmacological treatments (yoga, massage therapy, aqua therapy, even cognitive behavioral therapy).

“It’s trial and error, just like with anything in medicine,” Newsome says. “What I tell people is, if you find something that works, let’s stick with it.”

As for living with SPS, and whether it’s possible for someone like Dion to manage the condition well enough to return to the stage, Newsome (who is not Dion’s doctor) acknowledges that “it’s hard to answer.”

“People are affected by this in different ways,” he says. “We’ve had some people who’ve been able to get back some normalcy, not perfect; I wouldn’t say that — at least the patients I’ve seen — that they get back to their pre-stiff-person baseline. I don’t know, for Celine, whether she’ll get back to performing like she did in years past. But I think with a combination treatment approach, that gives people the best odds to get back to a higher quality of life, even though it may be different. People can live with this disease.” 

When it comes to finding cures and better treatments for rare conditions like SPS, Newsome says it always “starts at awareness” and “making people aware of how disabling this condition can be.” To that end, he praises Dion for speaking out about her diagnosis and what she’s been going through. 

“I give her a lot of credit,” he says. “Given where she is in the limelight, it takes a lot of courage to come out with this.”

From Rolling Stone US.