Celine Dion made a rare public appearance at the Grammys on Sunday night, presenting Taylor Swift with the award for Album of the Year. This marked one of the few times Dion has been seen in public since she announced her diagnosis of Stiff Person Syndrome in 2022.
In November, Dion, 55, was spotted at an NHL game with her three sons, as reported by Entertainment Tonight. However, her sister Claudette Dion revealed in a December interview that the condition has severely impacted Dion’s muscle control.
Stiff Person Syndrome, also known as Moersch-Woltman Syndrome, is a rare neurological disorder with autoimmune features. According to the National Institute of Neurological Disorders and Stroke, it causes the body to become rigid and more sensitive to noise, touch, and emotional stress. Patients often develop hunched postures and stiffened muscles.
Yale Medicine states that there is no cure for Stiff Person Syndrome. Treatments include therapies and medications such as sedatives, muscle relaxants, and steroids. Some doctors also recommend immune-based therapies, which may slow the progression of the disease, according to Johns Hopkins Medicine.
In severe cases, the condition can lead to significant disability, requiring patients to use walking aids or even wheelchairs. At the Grammys, Dion did not use any walking device but was escorted by her 23-year-old son, Rene Charles. “When I say that I’m happy to be here, I really mean it from my heart,” she said.
After postponing several tour dates, Dion decided to cancel her world tour in 2023 to focus on her health. Her sister Claudette mentioned that their dream is for Dion to return to the stage, but it remains uncertain. “Vocal cords are muscles, but so is the heart. That’s what gets to me. Because it’s a one-in-a-million case, scientists don’t have much research on the topic,” Claudette told the French news outlet 7 Jours.
Swift’s Grammy win for her album “Midnights” was her fourth in the Album of the Year category, the most for any artist. Some social media users speculated that Swift snubbed Dion by hugging other collaborators before taking the award from her. However, the two were seen smiling and embracing backstage after Swift’s acceptance speech.
In a statement posted on her social media, Dion explained that complications from Stiff Person Syndrome led to the cancellation of her “Courage World Tour” dates through 2024. “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,” Dion said.
Despite undergoing daily physical therapy, Dion is reportedly in a lot of pain. “I’m so sorry to disappoint all of you once again. I’m working really hard to build back my strength, but touring can be very difficult even when you’re 100%,” she said in her Instagram announcement.
Stiff Person Syndrome is characterized by muscle rigidity and spasms, heightened sensitivity to stimuli, and emotional distress that can cause muscle spasms. Over time, people with the condition can develop hunched postures, according to the National Institute of Neurological Disorders and Stroke.
The condition typically begins with muscle stiffness in the trunk and abdomen before advancing to the legs and other muscles, according to the Cleveland Clinic. The muscle spasms can be severe, causing falls, severe pain, and significant disability. “Falls from severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather,” said Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine.
These spasms can be strong enough to fracture bones, and any fall can lead to severe injury. Dion mentioned in her December Instagram video that spasms affect “every aspect” of her daily life, sometimes causing difficulties when walking and not allowing her to use her vocal cords to sing as she used to.
The syndrome can also cause anxiety. “Many patients, if not all, have an anxiety that is intrinsic to the disease and that anxiety actually feeds on the physical ailments of the disease,” said Dr. Scott Newsome, director of the Stiff Person Syndrome Center.
At times, people with Stiff Person Syndrome may be afraid to leave their homes because street noises, such as the sound of a car horn, can trigger spasms and falls, the NINDS noted.
Stiff Person Syndrome is very rare, affecting about 1 out of every 1 million people. Most general neurologists will see only one or two cases in their lifetimes, Moukheiber said. The first case was reported in the 1950s, and the disease was historically referred to as “stiff man syndrome.” Since then, it has been found to affect twice as many women as men, leading to the name change to avoid confusion.
The condition can develop at any age, but symptoms most often begin in a person’s 30s or 40s, according to the Cleveland Clinic. It is frequently associated with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo, and pernicious anemia.
Although the exact cause isn’t clear, research shows it may be due to an autoimmune response “gone awry” in the brain and spinal cord. People with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain. A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies.
Other tests include electromyography (EMG) and lumbar puncture. Because of the rarity of the disease and the ambiguity of its symptoms, people often seek care for chronic pain before getting neurological care. The condition can be misdiagnosed as anxiety, fibromyalgia, multiple sclerosis, Parkinson’s disease, psychosomatic illness, or even a phobia.
On average, it takes about seven years for someone to receive a diagnosis of Stiff Person Syndrome. “Sometimes, [patients] get labeled crazy because on exam early on, there aren’t the hallmark features of stiff person syndrome,” Newsome said.
There is no known cure for Stiff Person Syndrome, but medications can ease the symptoms. Immunoglobulin medications can help lower sensitivity to light or sound triggers, potentially helping head off falls or spasms. Pain relievers, anti-anxiety medications, and muscle relaxers can also be part of the treatment. The Stiff Person Syndrome Center uses botulinum toxin injections.
Additional benefits may come from acupuncture, physical therapy, heat, and water therapy. “If left untreated, the disease can cause severe impairment of daily living,” Moukheiber said.
Dion, who has struggled with her health for a while, said she has a great team of medical professionals and her children’s support. “I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again,” she said. “But I have to admit it’s been a struggle.”
For a performer like Dion, loud noises and bright lights could act as triggers of muscle spasms. “This is a very challenging illness that might take a toll on her if it is not treated aggressively, timely, and appropriately,” Moukheiber said.
