Rambam expert and “Mayo Clinic” researcher Shahar Sheli explains about Celine Dion’s rare disease
Celine Dion, one of the most famous singers in the world, announced earlier today (Thursday, 8.12.22), the cancellation of her tour of performances for the summer, which was supposed to include Israel, due to her health condition.
The singer stated today (Thursday 8/12/22) for the first time, that she suffers from a rare neurological disease called Stiff-Pearson syndrome, also known as “stiff man syndrome”. According to Dion’s announcement, the disease, which affects one in a million, affects the international singer’s ability to function on and off stage.
“It is a rare disease of the motor system, characterized by an involuntary contraction of muscles in the center of the body (axial muscles) in such a strong way that it causes intense muscle pain,” explains Dr. Shahar Sheli, head of neuromuscular diseases, in the neurological department of the medical center Rambam, “In extreme cases, the syndrome will cause a complete stagnation of that person without the ability to move.”
Dr. Shahar explains that among those with the “stiff man syndrome” (Moersch Woltman), 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 .
“The routine treatment used today is with sedatives (benzodiazepines),” notes Dr. Shahar, and adds: “There is a connection between this syndrome and the development of diabetes, epilepsy and a wide variety of autoimmune diseases.”
Studies recently published by Dr. Shelley, in collaboration with the neuroimmunology laboratories at the “Mayo Clinic” medical center in the USA, where he serves as a researcher and senior clinical lecturer, revealed mechanisms related to antibodies in patients with the syndrome and the link to malignant diseases.
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