International singer Celine Dion announced on Friday that she is canceling all her concerts scheduled until April 2024. The reason is a rare neurological disease he suffers from called “tough man syndrome“. “I’m fighting to get my strength back, but touring can be very difficult (…) It’s better if we cancel everything now until I’m really ready to get back on stage.”said the 55-year-old singer, who notes that she continues her treatment.
The pathology, which affects two in a million, makes it difficult for the Canadian singer to walk and use her vocal cords as she would like, she explained on social media in December. Can this disease be cured? What is causing this? An overview of the current medical knowledge on the problem.
THE: tough man syndrome, also called Moersch and Woltman syndrome after the scientists who discovered it in 1956, affects adults, men and women equally. Symptoms, mainly persistent contractions of certain muscles, develop insidiously and fluctuate over years, with peak incidence around age 45. Due to the lack of knowledge about this rare disease, which can appear potentially misleading to doctors, patients often experience medical wandering before diagnosis.
“Very Unusual Toughness”
The stiffness in the name originates from the continuous contraction of certain muscles at the root of the trunk and limbs, which prevent patients from walking or moving. “ These patients have a very unusual stiffness, says Dr. Carol Henry, neurologist and head of service at Saint-Denis Hospital Center. The muscles are not weak or congested, but they are constantly stimulated by the nerves». This differs from stiffness caused by osteoarthritis, damage to the brain or spine, or Parkinson’s disease. Contraction persists even when the patient attempts to rest, causing increasing disability. On the other hand, it mainly spares the face and “distal” muscles (legs, forearms) and stops during sleep.
The patient also suffers from muscle spasms caused by various stimuli (loud or sudden noises, emotions such as fear, etc.). These painful spasms can cause serious falls and fractures. “ The disease can be very disabling after several years of evolution. One of the few patients I have encountered in my career was unable to walk more than 2-3 steps at the time of diagnosis, and chest muscle spasms made it difficult for him to breathe, causing a temporary lack of oxygen. », says Dr. Henry. Sometimes the patient is affected only on one side of the body or one limb.
Imperfect treatment
We now know that stiff-man syndrome is an autoimmune disease. Most patients have high levels of anti-GAD autoantibodies, which reduce levels of GABA, a neurotransmitter involved in motor neuron control. Stiff-man syndrome is often associated with other autoimmune diseases (as is often the case with this family of diseases), such as type 1 diabetes. The syndrome can also occur “ autoimmunity caused by the body’s response to cancer This is called paraneoplastic syndrome “, explains Carol Henry.
Treatment is based on high doses of benzodiazepines (such as diazepam) or baclofen (a muscle relaxant). “ But this is not always perfectly effective. Our patients can remain in varying degrees of discomfort from stiffness or pain”, emphasizes Dr. Henry. The same observation for drugs aimed at blocking antibodies, such as immunoglobulins in infusions, or to prevent their production, biotherapies directed against B lymphocytes. “ The evolution is long, and if the aggravation can be stopped, the improvement is often incomplete “, summarized in 2013 in Neurological Practice by Dr. Thomas De Brucker from the same Saint-Denis Hospital Center. Dr. Henry, however, clarifies. “In the hospital, we see only the most serious cases. Undoubtedly, there are many less serious patients who are never diagnosed.
Source: Le Figaro
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