Celine Dion premiered the documentary on Tuesday I am Celine Diona production in which the singer reveals how has been his life since, in 2022, he retired from the stage due to a rare neurological disease. In her images, you can see what day-to-day life is like since everything changed and the efforts, achievements and failures of a woman who went from being one of the most listened to voices in the world to someone forced to fight every day against his strange ailment: Rigid Person Syndrome (SPR).
Strange because it is not usually seen. And they manifest themselves in one or two cases per million inhabitants, causing serious consequences in their daily lives and even putting their lives at risk. MRS is a disease whose main symptom is muscle spasms. Shocks so strong – and painful – that they can even cause a bone fracture or a fall with much more serious consequences. Other common symptoms may be difficulty swallowing, tiredness or loss of strength and balance.
Her situation was so serious that the French singer did not even had appeared in the media again since 2022…Until May of this year. It was in that month when she gave an interview in Vogue France and assured to be working “with all my body and all my soul, from head to toe, with medical equipment.” “I want to be the best possible”, he assured at the end of the conversation.
Since it is such a rare disease, not much research has been done on it, which causes a fairly high level of ignorance about this ailment. It is believed, despite everything, that it is most likely that it is a autoimmune disease, that is, a disorder in the body that causes its own defenses to attack the tissues and organs of the body. In this specific case, very usuallye, to the low level of glutamic acid decarboxylase (GAD).
“Symptoms of stiff person syndrome can develop when the immune system mistakenly attacks the neurons that produce GAD”, they explain on the website of the Genetic and Rare Diseases Information Centre (GARD). “When GAD is not working properlythere is not enough gamma-aminobutyic acid, which is what helps control muscle movement”. The women have more than double the possibilities of suffering from it compared to men.
In this way, the low concentration of this type of antibodiesmeasurable in a blood testmay be one of the indicative most important to guide doctors in diagnosing this condition, although if the levels are normal you can continue suffering from the disease. It is because of that other tests They can also be very useful: electromyography oo even the lumbar puncture.
Although there is a wide range Of the symptoms that patients may present, the physical manifestations of the disease are what will cause the most damage to the patient. In this way, the muscle relaxants and anxiolytics are the most common, as well as anticonvulsantssometimes, and analgesics. A team of Colombian doctors, however, confirmed with a study published in 2019 in the journal Elsevier than these pills They did not always have the expected result. Instead, methods like intravenous immunoglobulin wave plasmapheresis They did result in an improvement for the patient.
Also the symptoms depend a lot on the quality of life of a person with the disease, since sometimes they do not even give it importance. This is because, while in some cases The disease is hereditary and affects to several parts of the body, in others it only affects to a certain part. In any case, well-being and autonomy of patients suffers noticeably and may end up leading to other problems such as anxiety wave depression.
