The famous female singer's condition worsens! She can no longer "control her muscles". What is Stiff-Person Syndrome?

It has been a year since the famous singer Celine Dion announced last year that she had stiff-person syndrome, a rare neurological disease that prevented her from singing with her angelic voice as she used to, and she canceled a large number of concerts.

Recently, her family said that Celine Dion's condition has worsened and she "can no longer control her muscles."

Celine Dion 'can't control her muscles'

In late October, she made a rare public appearance after a long absence and seemed to be in better condition. But last week, her sister Claudette Dion told the media that the Grammy winner might not be able to return to the stage for the time being because she "cannot control her muscles."

"What makes me sad is that she has always been so disciplined. She has always worked so hard." Celine Dion's sister said that Celine Dion had a desire to return to the stage. "Our mother always told her, 'You will do well, you will do well.'" But as the singer's condition worsened, it was difficult to maintain confidence.

Regarding Celine Dion's announcement in May that she would cancel her tour, Claudette believed that the decision was correct because she needed a break.

"To be honest, I think what she needs most is rest. She always goes above and beyond (in her performance) and always strives to do her best. (But) at some point, your heart, your body will try to tell you something, and it's important to listen to those voices," Claudette said.

Celine Dion's sister added that the vocal cords are muscles, the heart is also a muscle, and there is not much research on this rare disease because not many people have it. "We also hope that she can return to the stage. In what capacity? I don't know."

In August this year, Claudette Dion said in an interview with the media that her sister's condition was like a sudden leg cramp at night, except that it could occur in any muscle and at any time, and now almost no one can relieve her sister's pain.

Stiff-person syndrome is defined as an autoimmune disease of the nervous system that typically causes "progressive, severe muscle stiffness and spasms in the lower limbs and back" and "affects approximately one in a million people." In December 2022, the performer said in a video shared in English and French that he was diagnosed with the neurological disease.

Once appeared on the CCTV Spring Festival Gala stage

Celine Dion was born on March 30, 1968 in Quebec, Canada. She is a French singer and actress. In February 1998, her theme song "My Heart Will Go On" for the movie "Titanic" won the 41st Grammy Awards for "Song of the Year" and "Record of the Year", making her famous in China.

In 2013, Celine Dion took to the stage of the Spring Festival Gala in the Year of the Snake with "My Heart Will Go On", the theme song of the movie "Titanic" . Her wonderful performance not only made this classic song popular all over the country again, but also made it the most watched program of the Spring Festival Gala that year.

Since the onset of stiff-person syndrome, she has canceled several shows, including her "Courage" world tour, which was originally scheduled for May this year.

What is Stiff-Person Syndrome?

What kind of disease is "Stiff Person Syndrome"? A reporter from Guangzhou Daily and New Flower City interviewed Qiu Wei, chief physician of the Third Affiliated Hospital of Sun Yat-sen University.

"This disease is also called 'wooden man'. It is a very rare autoimmune and neurological disease. Celine Dion's symptoms are quite typical."

Chief Physician Qiu Wei pointed out that most of the patients are women. When they come to see the doctor, their waist and back are as stiff as a piece of wood, making it difficult for them to walk and they are prone to falling when walking. This has a great impact on the patients' body and mind and seriously affects their quality of life.

As an expert in neuroimmune diseases, Chief Physician Qiu Wei, together with Professor Hu Xueqiang, a famous neurologist in my country, has seen and treated many patients with "stiff person syndrome".

Stiff-person syndrome usually causes stiffness and spasms in the trunk and limbs. Patients may experience symptoms early in the disease, which eventually become a persistent condition. The stiffness and spasms usually begin in the legs and trunk muscles, especially in the lower back, and over time can affect the arms and even the face. Other symptoms may also occur, such as an unsteady gait and unexplained falls.

Since the symptom of stiff waist and back muscles is relatively common, this disease is easily misdiagnosed as the rheumatic immune disease "ankylosing spondylitis". The patient is treated according to the idea of ​​rheumatic immune disease, and when there is no improvement, he may be diagnosed with "hysteria and anxiety disorder", and finally be diagnosed after being referred to the neurology department.

Some people confuse stiff-person syndrome with amyotrophic lateral sclerosis (ALS), but the two are actually very different. The ultimate outcome of ALS, or amyotrophic lateral sclerosis, is death from respiratory muscle paralysis or lung infection. Stiff-person syndrome patients will not die from the disease, but they will suffer tremendous psychological pressure due to a serious decline in their quality of life.

My legs are so stiff that I can't walk

GAD65 antibody positive

Alan (pseudonym), 44, was diagnosed with "stiff person syndrome" in 2020.

Since 2015, she has experienced pain on the back of her left foot. Initially, it was a paroxysmal needle-like pain that would ease after a few seconds. Then, pain began to appear in the left groin area, which gradually worsened. She could not walk alone, and her legs felt stiff. She had a slight tightness in her waist, which was obvious when she was nervous. She could not even walk alone when her legs were stiff, but it would ease after about a few minutes.

However, five years later, these symptoms became more severe. Alan had difficulty walking, especially when she stood up, turned around, squatted, or went down the stairs. She was prone to falling. When Alan finally found Professor Hu Xueqiang and Chief Physician Qiu Wei after seeking medical treatment, she could no longer walk independently.

Finally, based on clinical symptoms and relevant examination results, Alan was diagnosed with this very rare autoimmune and neurological disease.

Reminder: If you experience these symptoms, please seek medical attention immediately

The medical community still does not know what causes patients with stiff-person syndrome to produce GAD65 antibodies.

"According to domestic and foreign literature reports, some patients, in addition to abnormalities caused by the nervous system, also have manifestations of other systems such as abnormally high blood sugar. Some patients also have tumors," Qiu Wei admitted that because this disease is so rare, there are few reported cases and insufficient follow-up, so the medical community's understanding of it needs to be deepened.

Some patients mistakenly believe that the disease is untreatable, and after being diagnosed, they become discouraged and hopeless and disappear into the vast sea of ​​people. In fact, the exploration of the neurology team of Zhongshan Third Hospital in recent years has yielded initial results.

Taking Alan as an example, Qiu Wei's team tried protein A immunoadsorption (IA) treatment, followed by immunosuppressant treatment.

Protein A immunoadsorption is also known as a type of "blood purification". It reduces the titer of GAD65 antibodies in the blood first, and then uses immunosuppressants to reduce the production of antibodies, thereby alleviating symptoms and improving quality of life.

Guangzhou Daily New Flower City reporter learned that Alan had received protein A immunoadsorption therapy 6 times. After the first immunoadsorption (IA) treatment, he walked much more smoothly than before the treatment, and his muscles were more relaxed. After the immunoadsorption treatment, the stiffness and movement disorders of both lower limbs were significantly improved. By December last year, Alan's life had basically returned to normal.

Qiu Wei called on people to go to the neurology department for relevant examinations and early treatment if they unfortunately experience stiffness in their legs and torso and are unable to walk, after excluding causes such as ankylosing spondylitis . At the same time, they should reduce the pressure on their minds and face the challenges of the disease with optimism.

Planning and production

Source: Guangzhou Daily, compiled from Metropolitan Express, Jimu News, Guangzhou Daily past issues, CCTV News

Editor: Yinuo