An expert analyzes Celine Dion’s condition

About two years ago, the legendary singer Celine Dion bravely revealed that she was dealing with the “stiff person syndrome”, a rare and incurable neurological syndrome. Since then, the 56-year-old singer shared in several interviews about the difficulties and the progress of the disease. In a hard and revealing documentary from the documentary I Am Celine Dion that is available to watch starting yesterday (Tuesday) on Amazon Prime, Dion was recorded while she was having a seizure and convulsions, and writhing in pain. We checked with an expert how common these attacks are among those who suffer from the syndrome, and which treatments are discussed in order to deal with them.Dr. Shahar Sheli, Director of the Department of Neurology at Rambam, explains that Stiff-Person Syndrome is a relatively rare neurological disease characterized by muscle stiffness and sudden uncontrollable contractions. “What is unique is that patients have a hypersensitivity to external stimuli such as: sudden noises, touch or mental stress, which can cause muscle contractions,” he explains. According to him, muscle stiffness appears mainly in the muscles of the back and limbs, but it can also appear in the muscles of the face and neck. He also notes that the disease is also characterized by unexpected muscle contraction attacks that may cause falls.

 

Dr. Sheli emphasizes that the disease occurs in one person out of a million, and most of the patients are actually women. According to him, this is a syndrome that develops gradually, and as you can see in the video – it can lead to limitations in movement and severe pain. “You can see that the singer is going through an attack of muscle stiffness, It is a very painful attack, which causes the feeling that all the muscles in the body are seized at the same time.” He points out that according to the documentation it is possible to conclude about the progression of the syndrome, as well as that the disease is not under control.

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In the video in question you can see how Dion receives a kind of spray into her nose. Dr. Shelly explains that this is indeed the recognized treatment for her condition. “The spray is called intranasal diazepam And intensive treatment with it contributes to a significant reduction in the frequency and intensity of attacks. It contains benzodiazepines, substances that increase the effect of a neurotransmitter called GABA. The purpose of these substances is actually to calm down, and ease the stiffness and contractions.” He adds that in rare situations, the syndrome appears as a secondary syndrome to an autoimmune disease, so the patients receive immunological treatments to stimulate the immune system.

Due to the illness, Dion had to cancel the last tour in Europe. Recently, she said in an interview with the NBC network : “I experienced muscle contractions so strong that I broke ribs.”