- Celine Dion continues to battle a rare condition called stiff person syndrome (SPS) and has reportedly lost control over her muscles.
- Despite her symptoms, Dion presented an award at the 2024 Grammys and sang backstage with R&B star Sonyae Elise.
- SPS is a progressive neurological disorder that affects the brain and spinal cord and may cause stiff muscles in the torso, arms, and legs.
It’s been over a year since the French Canadian singer Celine Dion revealed on Instagram she has stiff person syndrome (SPS). This rare neurological disorder causes muscle stiffness and painful muscle spasms.
But Dion’s ongoing battle with this debilitating health condition did not stop her from appearing at the 2024 Grammys on February 4 and presenting the final award for album of the year, which went to Taylor Swift for “Midnights.”
“Thank you all, I love you right back,” Dion said. “When I say, I’m happy to be here, I really mean it from my heart.”
The 55-year-old pop also icon sang an impromptu duet backstage with R&B star Sonyae Elise, who shared a video on Instagram on February 6. “Highlights of an ICONIC night,” Elise wrote in the post.
Stiff person syndrome is an extremely rare progressive neurological disorder that affects the brain and spinal cord, according to the
SPS occurs in an estimated 1 in 1 million people, with the condition affecting women twice as much as men.
“It’s quite rare. During their career, many neurologists will not have seen more than one or two patients with a diagnosis of stiff-person syndrome,” said Dr. Stacey Clardy, a neurologist at the University of Utah Health in Salt Lake City, specializing in autoimmune neurology.
“At the Autoimmune Neurology clinic at the University of Utah, we longitudinally care for over 50 patients with stiff-person syndrome,” she told Healthline.
“And we’re a large, dedicated national referral center — so hopefully that gives perspective on the relative rarity of this condition.”
In a recent interview with 7 Jours, Celine’s sister Claudette Dion shared the singer no longer has “control over her muscles” due to the condition.
“There are some who have lost hope because it is a disease that is not [very well] known,” Claudette said in the interview.
According to the NINDS, the symptoms of stiff person syndrome may include stiff muscles in the torso, arms, and legs. People with SPS may also have greater sensitivity to noise, touch, and emotional distress.
“Changes in temperature (extreme cold or heat) can trigger [muscle spasms in] some patients, as well as large crowds, emotional stress, and generally any setting where there is a lot of simultaneous sensory input,” Clardy said.
People with stiff person syndrome may eventually develop abnormal, hunched-over postures or be too disabled to walk or move, according to NINDS.
In addition, falling is common because the condition can affect people’s ability to catch themselves. Serious injuries can result from these falls.
People with stiff person syndrome may also avoid leaving the house because loud noises or stress can trigger spasms, leading to a fall.
For most people, stiff person syndrome is a slowly progressive disease.
“As with any medical condition, the severity of symptoms fall along a spectrum — some people have very mild symptoms, others have quite severe symptoms,” said Clardy. “But in general, for most SPS patients, symptoms slowly progress over time.”
The cause of stiff person syndrome is unknown, but it is considered an autoimmune disorder.
Stiff person syndrome is associated with more common systemic autoimmune conditions.
“It often occurs in an individual who has inherited a predisposition to certain types of more common systemic autoimmune conditions, such as type 1 (insulin-dependent) diabetes, autoimmune thyroiditis, pernicious anemia and Addison’s disease,” said Clardy.
“It very rarely would occur in someone who doesn’t have some sort of family or personal history of systemic autoimmune conditions,” she said.
“And even then, most people with a history of these more common autoimmune conditions will never have a family member who is diagnosed with stiff person syndrome,” she added.
A blood test that measures the levels of glutamic acid decarboxylase (GAD) antibodies can diagnose stiff person syndrome. Most people with SPS have higher levels of GAD antibodies.
However, it may take some time before people receive an accurate diagnosis.
Because stiff person syndrome is so rare, people with this condition may visit several doctors before seeing a neurologist specializing in the condition.
Since there are more common causes of muscle spasms and cramps, people may have been, appropriately, treated with muscle relaxant or pain medications, said Clardy.
“This makes achieving the diagnosis even more challenging, ultimately, because the muscle relaxants or painkillers can interfere with our ability to diagnose SPS,” she said.
Another challenging aspect of diagnosis is the episodic nature of the symptoms.
“You can have a person with stiff-person syndrome in the exam room, and if they’re early in their condition, and having a good day, they can appear essentially normal at that one point in time when you are examining them,” said Clardy.
“But after they leave the clinic, they can have a very severe unprovoked muscle spasm,” she said.
In addition, existing systemic biases in medical practice related to gender may lead to delays in SPS diagnosis, she added, in particular for women.
Clardy said treatment for stiff person syndrome involves a two-pronged approach, with treatments targeting symptom management and the immune system.
“Symptomatic treatments lessen the muscle cramps and spasms. For that, we generally give a benzodiazepine such as diazepam (trade name Valium),” she said.
“The other half of our treatment approach involves dampening down the immune system attack on the body. That’s where IVIg [intravenous immune globulin therapy] falls. We also use some other immunosuppressive medications off-label,” she said.
Dr. Marinos Dalakas, a neuromuscular medicine specialist at Thomas Jefferson University in Philadelphia, ran the clinical trial that initially showed that high dose IVIg significantly improved stiffness, spasms, and gait over a three-month period in people with SPS.
Longer-term results from that trial were published this year in the journal Neurology Neuroimmunology & Neuroinflammation.
Dalakas told Clardy on a podcast of the American Academy of Neurology that his recent study showed that IVIg benefits patients with stiff person syndrome over the long term. The average follow-up period in the study was 3.3 years.
“We found that 67% of the patients improved,” he said. “The improvement was related to improved gait, better balance, reduced spasms and stiffness, reduced startle response and better ability to walk and appear in public spaces.”
However, for about 30% of people who initially responded to IVIG, the benefits diminished over time.
“This [diminishment] is related to disease progression,” he said, “and highlights the need [for] better therapies [and] more effective therapies over the long term.”
Clardy said more awareness of this disease is needed in the medical community, as are large, high-quality multicenter clinical trials. These trials will require additional funding.
“Those of us in autoimmune neurology who treat patients have been trying — and will continue trying — to attract funding agencies to work with us to study SPS,” she said.
Celine Dion made an appearance at the 2024 Grammys amid her ongoing battle with stiff person syndrome. The French Canadian singer Celine Dion reportedly lost control of her muscles.
Stiff person syndrome is an extremely rare neurological disorder affecting the brain and spinal cord.
Although the condition is not fully understood, some treatments are available.
More research and funding to improve treatments for stiff person syndrome are still needed