Stiff person syndrome, Celine Dion has lost control of her muscles: what now?

A few days ago Claudette Dion, interviewed by the magazine 7 dayss stated that the sister Celine Dion he “lost control of his muscles”. The singer's last public appearance, which in a Instagram video from 2022 had declared that he had the stiff person syndromehad been documented on the same social network by the journalist Chantal Machabée after the ice hockey match between Vegas Golden Knights And Montreal Canadienslast November.

On that occasion Celine attended the match together with her children, then greeted the players and management of the Canadiens team: a moment of serenity that seemed to herald a recovery. Her sister's statement, however, makes us think that this is not the case.

«The stiff person syndrome, or rather the Stiff Person Syndrome (SPS) – tells us the doctor Maria Claudia Vigliani ofMolinette Hospital City of Health of Turin Department of Neurosciences – is actually the name umbrella of a group of diseases that have similar symptoms, but they are probably different pathologies: so this already complicates the picture and being able to define exactly what type of disease Celine Dion has. We can certainly say that it is a chronic disease that does not proceed in stages, very painful and disabling, due to antibodies that practically work against the central nervous system, whose main symptom is muscle rigidity: the muscles contract, no longer able to relax and therefore have difficulty walking to the point of total disability. It is therefore an extremely i diseasedisabling and very painful. It is chronic and does not proceed in stages: «It can begin in all its violence after which it is possible to recover with therapies and have periods of remission, then have new relapses. So let's say that there is no succession in pejorative order.” The problem, underlines Vigliani «is that it is one very rare diseasewe are talking about one person in 1 million, therefore precisely for a reason of numerical scarcity of patients it is not possible to compose groups of trials and understand to the end what can work, for which people and what doesn't.”

Is this a fatal syndrome? Can it attack the heart?
«Not the heart, because this organ is a myocardial muscle with mechanisms completely different from other muscles. It is true, however, that there are also more serious cases (we speak of stiff person syndrome plus) which can lead to alterations in heart rhythm. The disease, however, can have outcomes that can lead to death in case of blockage of the respiratory muscles».

What are the known therapies at the moment?
«Stiff person syndrome is, as mentioned before, a chronic disease for which there is no definitive cure, but there are some therapies that can work very effectively for the containment of the disease same e the alleviation of symptoms (very painful muscle contractions, often with spasms). I am Three the main therapeutic pathsall aimed at limit the production of antibodies which attack the nervous system and cause the disease: of the immunosuppressive type, for which blood washing is practically carried out with a technique called plasmapheresis; or the “bad” antibodies are counteracted by injecting immunoglobulins from donors into a vein or, a very effective therapy, the use of monoclonal drugs to block the production of antibodies. The therapies can be applied one at a time: in some cases one is already effective and therefore we stop, or all three therapies are used in the life of a patient.”

On a pharmacological level, however, what prospects are there?
«There are some specific drugs to alleviate the symptoms which are essentially gods muscle relaxants (they relax the muscle). One in particular is the baclofen. There have been more serious and complicated cases for which we inserted a pump into the spinal cord that continuously released the drug, so as to allow the muscle to relax. Unfortunately, not all patients respond equally to therapies, furthermore every time a relapse occurs, the body is affected and the muscle and the entire nervous system are no longer what they were five, ten or twenty years before. An aggravating factor is that the physical symptoms of this syndrome are very often accompanied by anxiety, depression and cognitive difficulties with concentration disorders».

How is the onset of the disease recognized?
«In reality, the other problem that adds up is precisely the diagnosisi: it is often emitted late because at the beginning the symptoms are vague and non-specific and can be attributed to various other pathologies, such as fibromyalgia, or mistaken for psychosomatic conditions such as anxiety and phobias. Very often the diagnosis is then issued when the person reports having had several falls or being unable to get out of bed, through a simple blood test.”