What is Celine Dion’s stiff man syndrome?

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Celine Dion suffers from a very rare disease, Stiff Man Syndrome.

Health – by force, one might think it was cursed. After being forced to cancel her visit to the Vielles Charrues festival in 2020 due to Covid, Celine Dion has announced that she will also not be performing there in 2023. The reason? The star suffers from a neurological condition called stiff person syndrome (SHR), which is an extremely rare disorder, as she revealed in a video posted on Thursday, December 8th.

According to the Orphanet website, which was created by INSERM and specializes in rare diseases, this disorder affects about one in a million people worldwide. Two-thirds of the patients are women. Mainly causes Fluctuating stiffness of the trunk and extremities and painful muscle spasms. »

Orphanet described other symptoms as well, such as: A phobia related to the performance of certain tasks, and the tendency to jump in an exaggerated manner. And also an abnormal posture, hunched over or stiff, if the disease was poorly treated.

A very debilitating disease

Which corresponds to the evils mentioned by the Canadian star: “These spasms affect my daily life on many levels. Sometimes I find it very difficult to walk and I find it difficult to use my vocal cords to sing as well as I want to”she said in the video where she looked like she was about to cry.

So stiff man syndrome can be very disabling. “People with SHR are sometimes too unable to walk or move around, or afraid to leave their homes because street noise, such as the sound of a car horn, can trigger contractures and falls.”Reports of the US National Institute of Neurological Disorders.

But the cause of this disease is still not clear. Scientists assume that it is “The result of an aberrant autoimmune response in the brain and spinal cord.”continues the American Institute. But it is often confused with other diseases such as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, anxiety, or phobias because there is no specific lab test that can confirm its presence.

Difficult diagnosis

So how do we distinguish it correctly? Diagnosis is mainly based on observation of clinical signs and a blood test that measures the level of glutamic decarboxylase (GAD) antibodies in the blood – people with PSS have high levels of GAD.

It cannot currently be cured, but there is a treatment that can control the symptoms. The syndrome is mainly treated with benzodiazepines, drugs used against anxiety and convulsions. According to the American Institute, “Intravenous immunoglobulin (IVIg) therapy to reduce rigidity and reduce sensitivity to noise, touch, and stress in people with PSS.”

Celine Dion, has had to cancel a large part of her European tour scheduled for 2023. “I always give 100% in my concerts, but now I’m not in a position to do that”She regretted before concluding with a message of hope: to find you (his audience)I have no choice but to focus on my health and hope that I am on the right track. »

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