What is stiff-person syndrome, the neurological disorder Celine Dion has been diagnosed with?
Following Celine Dion’s diagnosis with stiff-person syndrome, BAZAAR explains the rare neurological disease which can affect all ages.
By Hayley Peppin
CELINE DION has sadly cancelled all remaining live shows for her Courage World Tour after being diagnosed with a rare neurological illness called Stiff Person Syndrome (SPS) last year.
She told fans via Twitter she was “tremendously disappointed” to “let them down” — with all scheduled shows for 2023 and 2024 affected.
“I’m so sorry to disappoint all of you once again.
“Even though it breaks my heart, it’s best that we cancel everything until I’m really ready to be back on stage.”
Dion concluded: “I’m not giving up… and I can’t wait to see you again!”
Back on December 8, 2022, Dion revealed during a teary announcement video she posted to Instagram that she had been diagnosed with the incurable condition of SPS — also known as Moersch-Woltman syndrome. Stiff-person syndrome gives people painful muscle spasms and fluctuating stiffness, but what triggers the condition and how will it affect Dion? BAZAAR explains the rare disorder below.
Celine Dion reveals stiff-person syndrome diagnosis
Celine Dion, famed for her power ballads — including the iconic ‘My Heart Will Go On’ from the 1996 film Titanic — revealed to Instagram on December 8, 2022 she had to cancel her tour in Germany, Switzerland, Poland, Hungary, Croatia, Austria and the UK from February 2023 due to her diagnosis of Stiff Person Syndrome (SPS). Earlier that year, Dion also cancelled her North American tour and her long-running residency at Caesars Palace in Las Vegas.
During an emotional announcement video, Dion called herself an “open book” — saying she was ready to share the health problems she’s been dealing with “for a long time.”
“Recently I’ve been diagnosed with a very rare neurological disorder called the stiff person syndrome, which affects one in a million people,” the five-time Grammy winner said. While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having.”
“Unfortunately these spasms affect every aspect of my daily life, sometimes causing difficulties when I walk and not allowing me to use my vocal chords to sing the way I’m used to.”
While Dion hasn’t given any indication as to when she might resume touring, she suggested she’s not ready to retire yet.
“I always give 100 per cent when I do my shows, my condition is not allowing me to give you that right now,” she said. “For me to reach you again I have no choice but to concentrate on my health at this moment and I have hope that I’m on the road to recovery.
“This is my focus and I’m doing everything I can to recuperate.”
What is stiff-person syndrome?
Following Celine Dion’s emotional announcement, Dr. Leah Croll, an assistant professor of neurology at the Lewis Katz School of Medicine at Temple University in Philadelphia, told Good Morning America that stiff-person syndrome (SPS) is an “exceptionally rare disease” and “occurs only in about one to two people per million.”
It’s a chronic neurological disorder which impacts the central nervous system and causes fluctuating muscle stiffness and muscle spasms, according to the US National Institute of Neurological Disorders and Stokes (NINDS). It can also affect posture, balance, greater sensitivity to noise, touch and emotional distress and the ability to use certain muscles.
Andrew McKeon, professor of neurology at the Mayo Clinic, told the Washington Post in a nutshell, SPS affects neurons in the brain that regulate movement. When the nervous system becomes overly excited, it can send too many signals to the muscles that causes them to stiffen or spasm. A person’s “whole body can seize up when startled or in other situations,” putting them at risk for falls and injuries, he explained.
It’s also common to see SPS alongside other autoimmune conditions such as diabetes, thyroid disorders and pernicious anemia.
Who can get stiff-person syndrome?
First described in 1956 as stiff-man syndrome, the medical community later changed the term to reflect the majority of SPS’s diagnosed patients — that being middle-aged women.
Dr. Leah Croll told Good Morning America that women are “two to three times” more likely to have stiff-person syndrome than men. While it’s not conclusive — SPS is sadly under-researched — Dr Croll believed it’s because of the disease’s “autoimmune component.”
“In general, women are higher risk for autoimmune diseases. But this is theoretical. We’re not totally certain as of yet.”
While those in their 40s and 50s are most commonly diagnosed with SPS, it’s also affecting young adults in their 20. Even rarer, the disease can appear in children and the elderly.
Despite being an autoimmune disorder, here is no clear mechanism as to why SPS antibodies are generated.
What are the symptoms of stiff-person syndrome?
During her Instagram video, Celine Dion shared some of her symptoms to be “difficulties” walking and using her vocal chords. “Unfortunately, these spasms affect every aspect of my daily life,” the singer said. Other symptoms of stiff-person syndrome include feeling rigid and stiff in certain areas of the body, a feeling of unsteadiness, and muscle spasms — often in the lower back and legs, hence the troubles some SPS patients have with walking.
“Typically, patients will present with stiffness in the muscles of the trunk, neck and back, and also … the shoulders and the hips. In some cases, patients may have the disease that only implicates maybe just one limb, so like just one leg is affected,” Dr. Leah Croll shared with Good Morning America.
Kunal Desai, assistant professor of neurology at Yale University, explained to the Washington Post the particular muscle spasms are called “stimulus sensitive” and can be triggered by a sudden noise, light touch or even emotional distress. He called the condition “extremely uncomfortable” and “very painful” — and can lead to difficulties opening one’s eyes, talking or singing (as Dion noted) and even swallowing.
“And when the pain occurs it’s very debilitating no matter what position. There’s basically no comfortable position for them to stay, either sitting or laying.”
While it doesn’t usually affect one’s cognition, those with SPS can also develop other conditions such as anxiety and phobias due to their physical limitations.
Is stiff-person syndrome treatable?
While there are treatments available to manage and lessen the severity, there is no cure for stiff-person syndrome as yet. However, the US National Institute of Neurological Disorders and Stoke (NINDS) says “several symptoms improve” with oral diazepam (an anti-anxiety and muscle relaxant drug) or other drugs which alleviate muscle spasms, such as baclofen or gabapentin.
A study also funded by NINDS revealed intravenous immunoglobulin (IVIg) — a treatment derived from thousands of healthy donors, which gives you antibodies to help fight infections — is also effective in reducing stiffness, sensitivity to noise, touch and stress, and improves balance and walking ability.
“Most patients, as a first line, will be given medications that are meant to help relax the muscles. And in some patients, their doctors may also choose to pursue certain therapies that are meant to modulate the immune system,” Dr. Leah Croll told Good Morning America.
“It’s important though, to point out that these are therapies that are meant to lessen the severity of symptoms or potentially slow the progression of symptoms, but we don’t have a therapy available that specifically targets this disease.”
Patients may also be referred to pain psychologists, physical therapists and other health professionals “to try to improve their overall quality of life and decrease the suffering associated with pain,” according to Pavan Tankha, medical director of Comprehensive Pain Recovery at the Cleveland Clinic, per the Washington Post.
“Pain simply isn’t what you feel; it’s also what it does to you,” he continued.
Furthermore, Dr. Scott Newsome, a neurologist at the Johns Hopkins University School of Medicine told CTV News in the US is to look at treatment from a “multifaceted treatment approach.”
“The gold standard is treating people with muscle relaxers, but given that it’s an autoimmune condition, we do use immune-related therapies to help and then other non-pharmacologic therapies (like) occupational therapy.”
Does stiff-person syndrome affect life expectancy?
As stiff-person syndrome is rare, there’s been insufficient research and major clinical trials for the disease — thereby making it difficult to determine whether it affects one’s life expectancy. However, Dr. Leah Croll told Good Morning America some patients have lived for a few years after diagnosis, while others have gone on to live for decades.
Only in “very rare circumstances” is breathing and swallowing compromised, writes the Washington Post.
Experts say when well-controlled, people with SPS can live “relatively normal” lives, however their quality of life will never go back to pre-diagnosis days. Some often require a walker or wheelchair to keep from falling or injuring themselves.
What to do if you feel like you have stiff-person syndrome symtoms?
While experts say it’s not a condition commonly seen amongst the general public , if individuals are concerned about their physical ability, they should consult with their GP who can provide a referral to a neurologist.
For more information on stiff-person syndrome, see Rare Diseases Australia, the National Blood Authority, Genetic Alliance Australia and the US National Institute of Neurological Disorders and Stoke (NINDS).