Main points of the article:
SPS– stiff person syndrome – And in Hebrew, “Stiff Man Syndrome”, is a rare neurological disease, in which the sufferers gradually turn into a human statue, unable to move. This musculoskeletal disease causes spasms so severe that bones can break.
The rare syndrome is autoimmune – That is, the body produces it by itself. It is not caused by a virus or bacteria, and the hypothesis is that it erupts as the body’s response to distress such as stress.
There is no medicine that cures the disease – But those with it receive sedatives, which calm the contractions, and also drugs against diabetes, which often goes hand in hand with the rare syndrome.
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Celine Dion, the beloved 54-year-old Canadian singer, published a video this week in which she said that she is dealing with many medical problems, which are forcing her to cancel her upcoming concert tour, which, among other things, was also expected to take place in Israel. The highlight of the video, which caused shock and pain to her fans, was when she said that she suffers from SPS – stiff person syndrome – “stiff person syndrome”, which currently manifests itself mainly in very painful muscle contractions.
This is such a rare, severe and incurable disease, and in the video in which the singer appeared, she said in tears: “It hurts me to tell you that I won’t be able to start my European tour in February. I miss seeing you all, being on stage and performing for you. I always give 100% of myself during every performance, but I can’t give it to you at this time. If I want to come back and perform for you, I have to concentrate on my health, and I hope I’m on the road to recovery.”
Celine Dion. announced the cancellation of the tour next year. Photo: Shutterstock
What is stiff man syndrome?
What is this syndrome that most people have never heard of (although it was the subject of an episode of Grey’s Anatomy in 2007)? Is there a cure for it and does it in its final stage really cause complete paralysis?
Stiff person syndrome affects the nervous system, especially the brain and spinal cord. The symptoms at the beginning of the disease are manifested by muscle stiffness and painful spasms, which interfere with mobility. The convulsions are so intense that they can cause bones to break.
How is the disease caused?
The rarest disease occurs in one in a million. It is believed to be an autoimmune disease, meaning a condition where the immune system attacks the body and is triggered by mental stress. The disease is often associated with diabetes, extreme anemia and other autoimmune diseases involving the thyroid gland.
How is stiff person syndrome diagnosed?
The diagnosis of the syndrome is made after various tests, including blood tests that test acid antibodies, lumbar puncture and electromyography. People suffering from the disease feel the stiffness of the muscles, painful muscle contractions and experience great difficulty in conducting themselves normally, even a simple walk. Some patients suffer from convulsions, convulsions and high sensitivity to noise or strong lights.
The disease causes intense muscle pain and in extreme cases to complete stagnation without being able to move. Photo illustration: Shutterstock
How is the disease treated?
Currently, there is no known drug that can cure the disease, but there are several drugs that can alleviate the symptoms: sedatives, anticonvulsants, anti-anxiety drugs, muscle relaxants and pain relievers.
Who might suffer from this rare neurological syndrome?
As mentioned, this is a very rare disease that occurs in one in a million people. It mainly attacks middle-aged women, and indeed Celine Dion meets this criterion.
What do the experts say?
Dr. Shahar Sheli, head of neuromuscular diseases in the neurology department at the Rambam Medical Center in Haifa, a researcher at the leading Mayo Clinic medical center in the USA, explains about Celine Dion’s rare disease:
“This is a rare disease of the motor system, characterized by an involuntary contraction of muscles in the center of the body (axial) in such a strong way that it causes intense muscle pain,” explains Dr. Sheli. “In extreme cases, the syndrome will cause the person to freeze completely without You could move.”
Dr. Shelley explains that among those with stiff man syndrome (Moersch Woltman) – another name for the disease) these muscle contractions are sometimes stimulated by loud noise or mental stress, two triggers that are very characteristic of the lifestyle of Dion, who leads a demanding international music career .
According to him, “the routine treatment used today is with sedatives (benzodiazepines). There is a connection between this syndrome and the development of diabetes, epilepsy and a wide variety of autoimmune diseases.”