She’s one of the hardest-working singers in the business and beloved by all, but Celine Dion has had to cancel a string of shows after being diagnosed with a rare neurological disease. So, what is Stiff Person Syndrome, the disease that’s putting her career on pause?
In a tearful video shared to Instagram on December 8, 2022, the Canadian pop star explained that she had been “dealing with problems with my health for a long time and it’s been really difficult for me to face these challenges and talk about everything I’ve been going through,” the 54-year-old explained. “It hurts me to tell you that I won’t be ready to restart my tour in Europe in February. She continued that she was suffering painful spasms that “affect her daily life” and that she’d recently been diagnosed with Stiff Person Syndrome, a rare neurological disorder that affects around one in a million people. Here’s what you need to know about Stiff Person Syndrome and whether there’s a cure.
What is Stiff Person Syndrome?
Stiff Person Syndrome (SPS) is a rare neurological and neuromuscular condition that causes progressive muscle stiffness and painful spasms, according to John Hopkins Medical Center. Spasms can be triggered by a range of things including but not limited to sudden movement, cold temperatures or unexpected loud noises. The cause is not presently known; however, research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord according to the National Institute of Neurological Disorders and Stroke. SPS is also frequently associated with other autoimmune diseases such as diabetes, thyroiditis, vitiligo and pernicious anemia.
John Hopkins neurologist Dr Scott Newsome explained in a video that it’s a “devastating” and incurable disease that affects around one in one million people worldwide, though as the medical community gains a better understanding of the condition it’s “probably more common than that.” The current statistic is based on what he deemed the “classic” symptoms of Stiff Person Syndrome, which were first described in the ‘50s as patients presenting with “rigid” torso muscles, tightness in their legs and “horrific spasms in really any muscle in the body,” though it’s most common in the lower body. A definitive diagnosis is obtained via a blood test, a lumbar puncture and electromyography.
According to the Stiff Person Syndrome Research Foundation, “patients can be disabled, wheelchair-bound or bed-ridden, unable to work and care for themselves,” they say, adding that the neurological disease with autoimmune features can include symptoms like hyper-rigidity, debilitating pain, chronic anxiety,” and muscle spasms “so violent they can dislocate joints and even break bones.” Because of its early symptoms, Stiff Person Syndrome is often mistaken as Multiple Sclerosis, Parkinson’s, Fibromyalgia, psychosomatic illness, anxiety, phobia and other autoimmune diseases.
“Sometimes they get labeled ‘crazy’,” Dr. Newsome said, “because on exam, early on, there aren’t the hallmark features of Stiff Person Syndrome… But on average, because of how rare it is and how early on in the disease it can mimic a lot of other conditions, it takes about seven years for people to get diagnosed, from symptom onset to diagnosis.” Dion explained in her video to Instagram that “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.” She announced that she would have to postpone a string of tour dates until 2024.
It’s now clear this debilitating disease was the reason for her cancellations in January 2022 as well, with a press release citing “severe and persistent muscle spasms” that had been preventing her from performing. Thankfully, Dion has “a great team of doctors working alongside me to help me get better. And my precious children who are supporting me and giving me hope,” she said. “I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again, but I have to admit, it’s been a struggle.”
What is the treatment for Stiff Person Syndrome?
Treatment for Stiff Person Syndrome can be arduous and though it’s a currently incurable condition, it can be managed with a range of medications and therapies. People with SPS tend to respond to high doses of diazepam and several anticonvulsants like gabapentin and tiagabine. Valium can also be effective as it’s a muscle relaxant. There’s also another somewhat surprising medication. “Often, we will give Botox to patients with Stiff Person Syndrome,” explained Dr. Newsome in another video, “especially when they have difficulties with spasticity or tightness… which is interesting in and of itself but it helps with some of the rigidity and the spasms our patients can suffer with.” In one report from 2003, a 41-year-old patient was shown to have “significant” improvement in her symptoms after receiving a Botox injection in her muscles.
Dr. Newsome explained that physical therapy can be a great supplement to medications, though “one has to be very careful with the type of physical therapy” because some treatments can worsen Stiff Person Syndrome. He recommends deep tissue techniques, ultra-sound, heat and aqua therapies in a warm pool because cold “can exacerbate” the symptoms. As the NINDS and Dion noted, people with SPS also have challenges in daily life: “Most individuals with SPS have frequent falls and because they lack the normal defensive reflexes; injuries can be severe. With appropriate treatment, the symptoms are usually well controlled.”
Concluding her emotional announcement, Dion said she missed her fans and being on stage to perform for them. “I always give 100 percent when I do my shows, but 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. I want to thank you so much for your encouraging wishes of love and support on my social media. This means a lot to me.” In a tearful sign-off, she said: “Take care of yourselves. Be well. I love you guys so much and I really hope I can see you again real soon. Thank you.”
For more about Celine Dion, you won’t want to miss her impassioned and honest autobiography, My Story, My Dream, which tracks her early life to becoming the “international singing sensation” beloved the world over. This touching memoir takes readers backstage, behind the scenes and her early career breakthroughs, as well as her storybook romance and eventual marriage to Rene Angelil, her manager and soul mate. This is an intimate, funny at times and ultimately heartfelt look at an unparalleled talent in the industry, in her own words. See why critics called Celine Dion’s My Story, My Dream “a remarkable” reflection of a pop star’s life and career.
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