Celine Dion suffers from “stiff man syndrome”, a very rare neurological disease

HEALTH – Celine Dion back in the news. The singer will answer Anne-Claire Coudray’s questions in an interview broadcast on TF1’s 20 Heures, this Sunday June 16. This first interview given to a French audiovisual media in many months is part of the promotion of the documentary I am: Celine Dionwhich will be released on June 25 on Prime Video.

In this film, Céline Dion recounts her daily life with the disease. As she revealed in a video published in December 2022, she suffers from a neurological disease, the so-called “stiff man” syndrome (SHR) (stiff person syndromein English), a disorder which is extremely rare.

According to the Orphanet website, created by Inserm and specializing in rare diseases, this disorder affects around one in a million people worldwide. Two thirds of patients are women. It mainly causes “fluctuating rigidity of the trunk and limbs and painful muscle spasms. »

Other symptoms are also described by Orphanet, such as “ a phobia associated with carrying out certain tasks, a tendency to startle in an exaggerated way”, as well as an abnormal posture, curved or stiffened, if the disease is poorly treated.

A very disabling disease

Which corresponds to the ills mentioned by the Canadian star: “These spasms affect my everyday life on several levels. I sometimes have a lot of trouble walking and I have trouble using my vocal cords to sing the way I want to.”she declared in a video where, forced to cancel part of her tour, she seemed on the verge of tears.

Stiff man syndrome can therefore be highly disabling. “People with HRS are sometimes too disabled to walk or move around, or are afraid to leave their homes because street noises, such as the sound of a horn, can trigger spasms and falls.”reports the American National Institute of Neurological Disorders.

But the cause of this disease is still quite unclear. Scientists assume that it is “the result of an autoimmune response gone wrong in the brain and spinal cord”, continues the American institute. But it is often confused with other pathologies such as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, anxiety or phobia because no specific laboratory test can confirm its presence.

A difficult diagnosis

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

It is currently not possible to cure it, but there is treatment capable of controlling the symptoms. The syndrome is mainly treated with benzodiazepines, drugs used against anxiety and spasms. According to the American institute, “treatment with intravenous immunoglobulin (IVIg) to reduce rigidity and reduce sensitivity to noise, touch and stress in people with SPS. »

Celine Dion has not gone on stage since a concert in Newark, in the United States, in March 2020, during a tour interrupted by Covid. Her state of health has prevented her from meeting her audience since. In a press release released in May, she regretted: “As much as it breaks my heart, it’s best to cancel everything now, until I’m truly ready to go back on stage.” I want you to know that I’m not giving up… and above all, I can’t wait to see you again! »

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