Celine Dion suffers from Stiff Man Syndrome (New York, March 8, 2020). Gotham / GC Pictures
INTERVIEW – Clemence Marois, a neurologist specializing in neuroresuscitation at the Pitié Salpêtrière Hospital, informs us about the extremely rare neurological disorder that the Quebec singer suffers from and the available treatments.
“We still don’t know everything about this rare disease, but now we know it’s the cause of the muscle spasms I suffer from,” Celine Dion said in a video posted on her Instagram page on December 8, 2022. on May 26, he announced in another post the cancellation of his concerts, which were scheduled until April 2024. The Quebec star has tough man syndrome. An extremely rare and still mysterious neurological disorder for the scientific community, which can affect one in a million people, according to Clemence Marois, a neurologist specializing in neuroresuscitation at the Pitié Salpêtrière Hospital. “During the last ten years, our service has encountered only ten to fifteen cases,” reports the doctor.
This syndrome is generally autoimmune in origin, meaning it is caused by the immune system producing an antibody called GAD. In other, less common cases, the cause is cancer. then we talk about paraneoplastic cases. Finally, other effects remain unexplained. Dr. Clemence Marois tells us more about the daily life of the victims, the treatments used and the ways of recovery.
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Miss Figaro. –What are the symptoms of stiff man syndrome?
Clemens Marois. – There are two main and typical ones. First, as the name suggests, patients suffer from general stiffness in their muscles. They cannot contract and release them, which hinders their movements. Then they are subjected to painful spasms, or rather sudden muscle contractions, which especially affect the trunk, legs and arms. Other symptoms such as neurological disorders (epilepsy, depression or other psychiatric disorders) may also be seen. It is because of the variety of symptoms that we speak of a syndrome rather than a disease. It is a set of clinical signs with multiple causes, not clearly established symptoms with a single explanation. And this same variety also makes diagnosis very difficult.
How do we live with this syndrome?
Spasms can affect the whole body at any time of the day and reach particularly sensitive areas such as the throat, eyes or even the stomach. On a daily basis, patients may then have more or less disabling consequences, such as difficulty breathing, walking or swallowing. Eventually, these chronic anxieties can lead to more serious disorders. Swallowing saliva repeatedly can lead to, for example, pneumonia. And the later the care is performed, the more complications increase.
Spasms can affect the whole body at any time of the day and reach particularly sensitive areas such as the throat, eyes or even the stomach.
Clemence Marois, neurologist
Can we cure it?
It’s hard to say: First of all, everything depends on when the diagnosis is made. The sooner, the greater the chance of recovery. Then the recovery depends on the causes of the syndrome. If the latter has an autoimmune origin, then very effective drugs are used that significantly limit spasms and stiffness or even completely eliminate them. These are so-called “immunomodulatory” treatments that target the source of the disorder and are associated with benzodiazepine drugs that limit symptoms. On the other hand, if the syndrome is caused by cancer, it is more difficult to treat it. It will all really depend on the management and treatment of the cancer. In very rare cases, when stiff man syndrome affects the brain, for example, the prognosis can even be life-threatening. Finally, when the origin of the syndrome is unknown, we can effectively reduce the symptoms, but a complete cure will not be provided, because by definition we cannot treat the cause.
Source: Le Figaro
