What Is Stiff Person Syndrome? Celine Dion Announces Diagnosis of Rare Disease

Canadian pop star Celine Dion announced on Instagram this week the postponement of her upcoming tour due to a diagnosis of stiff person syndrome, a rare neurological disease that affects about one in a million people.  

“I’ve been dealing with problems with my health for a long time, and it’s been really difficult for me to face these challenges and to talk about everything that I’ve been going through,” Dion says in her video. “I always give 100 percent when I do my shows, but my condition is not allowing me to do that right now.”

So what exactly is stiff person syndrome?

It's an autoimmune and neurological disorder that causes rigidity and spasms in the torso and limbs, debilitating pain, and chronic anxiety, per the Stiff Person Syndrome Research Foundation. The spasms can occur randomly or in response to environmental stimuli like loud noises or emotional distress, and they can last anywhere from seconds to hours, Pavan Tankha, the medical director of comprehensive pain recovery at Cleveland Clinic, tells the New York Times’ Nicole Stock. They can even be so violent as to dislocate joints or break bones. 

“Just imagine having the worst Charley Horse you can have but it’s affecting a ton of muscles in your lower back and legs—and it’s constant,” Kunal Desai, a neurologist at Yale University, tells the Washington Post’s Lindsey Bever, Richard Sima and Annabelle Timsit. “It’s very painful.” 

Dion explains that the muscle 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.” 

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While the cause of the condition is unknown, research suggests it may be the result of an awry response in the brain and spinal cord, per CBS News’ Li Cohen. Some with the syndrome have higher levels of antibodies to an enzyme called glutamic acid decarboxylase, or GAD65. This enzyme facilitates the formation of a neurotransmitter called GABA that “helps to reduce nerve and muscle excitation,” per the Stiff Person Syndrome Research Foundation. 

The disease can affect anyone, but it occurs more in women and people aged 20 to 50, and very rarely in children. Currently, there is no cure, but medications such as sedatives, muscle relaxants and steroids can be used to relieve symptoms. 

Earlier this year, Dion canceled several shows on her North American tour and for her Las Vegas residency, citing health concerns. Now, eight of her shows scheduled for summer 2023 will be canceled, and her spring 2023 shows will be rescheduled to 2024.

"This is just such a severe diagnosis to have, especially if you’re an entertainer [on] the world-class type of stage," Simon Helfgott, a rheumatologist at Harvard Medical School, tells NBC News’ Aria Bendix. "It’s going to be very, very challenging to be able to continue." 

Stiff person syndrome itself is not fatal, but it can be debilitating, and complications from the disease can lead to a shortened life expectancy, per the Times. While it’s difficult to predict how the disease will progress, most people see a worsening of stiffness and spasms over time. "In some cases, the condition can level off and stay the way it is. I have people who are like that—they're no different now than they were 10 years ago," Helfgott tells NBC. "In others, it is a slow, subtle decline."

Though Dion’s video was solemn, she says she remains optimistic. “I have 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 says. “I have hope that I’m on the road to recovery. This is my focus, and I’m doing everything that I can to recuperate.”

“I love you guys so much, and I really hope I can see you again real soon,” she adds.

Margaret Osborne | | READ MORE

Margaret Osborne is a freelance journalist based in the southwestern U.S. Her work has appeared in the Sag Harbor Express and has aired on WSHU Public Radio.