Since 2022, the Canadian singer had virtually abandoned the stage, with the exception of an appearance at the opening ceremony of the Paris 2024 Olympic Games. The reason was a rare neurological disease: stiff person syndrome, also known as Moersch-Woltman.
The international star's situation has drawn attention to this condition, previously unknown to the general public. Most estimates suggest one case per million people. Some specialists in the disease, such as the American neurologist Amanda Piquet who treats the singer, estimate its frequency at one or two cases per 100,000, due to probable underdiagnosis.
"We don't have reliable statistical data."
"We don't have reliable statistical data: in these ultra-rare diseases, we're always dealing with rather rough estimates." admits Pierre-François Pradat, neurologist at the Parisian hospital of Pitié-Salpêtrière (AP-HP).
In any case, this syndrome, which mainly affects women, remains a rare disease that often takes years to diagnose. Its symptoms resemble more common conditions such as multiple sclerosiswhich complicates its identification. "Because it's painful, it can also be confused with rheumatological problems." Mr. Pradat adds.
The disease is characterized by progressive muscle stiffness and repeated episodes of painful muscle spasms. These can occur randomly or be triggered by a variety of different events, including a sudden noise or slight physical contact.
"It's like someone is strangling you."Celine Dion testified in June 2024 that the pain could spread throughout her body and that the spasms sometimes broke her ribs. A clip from a documentary broadcast shortly afterward showed the star in the midst of an attack, completely immobile and unable to speak.
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An immune system disorder?
"The age of onset is around 45 years, and the symptoms develop over several months or years." explains Orphanet, a French-language portal on rare diseases, emphasizing that the prognosis is "Highly variable."
The cause is not known for certain, but the most likely explanation is a dysfunction of the immune system. Most patients have abnormally high levels of certain antibodies, which supports this hypothesis.
Like many neurological conditions, there is no proven cure for stiff-person syndrome. However, it is crucial to offer patients ongoing support to manage their symptoms. If left untreated, the syndrome can significantly impact a person's ability to perform routine daily tasks.
"Many patients lose their ability to walk independently and stiffness becomes generalized, leading to a general decline in functional status and quality of life," Orphanet points out that there is currently no single protocol among doctors to deal with the symptoms, as a consensus is difficult to establish given the rarity of cases.
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"The earlier it's diagnosed, the better."
However, the approach generally involves combining certain medications, particularly muscle relaxants such as benzodiazepines, with non-drug approaches like physical activity. "The earlier it's diagnosed, the better." Mr. Pradat points out. "Then there is variability linked to the characteristics of the disease, which will be different from one person to another."
In Céline Dion's case, her doctor, Professor Piquet, detailed to several media outlets a protocol combining conventional medication, immunotherapy, physiotherapy, and vocal rehabilitation. Ultimately, the media attention surrounding the singer "has allowed us to better understand the disease and for even doctors to better understand it," He greeted Mr. Pradat, expressing his satisfaction. "that patients feel less isolated in these very rare diseases."
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