Text/Li Jian, Contributing Writer of CC Intelligence Bureau
Key Takeaways:
1. Celine Dion, the singer of the well-known theme song "My Heart is Eternal" in the movie "Titanic" and an internationally renowned singer, is suffering from the rare disease "stiff person syndrome", which makes it difficult for her to sing smoothly and control her muscles. According to media reports on December 19, she has still not been able to return to the stage and can only cancel the concert altogether.
2. "Zombie syndrome" (SPS), also known as zombie disease, is an autoimmune disease that causes muscle stiffness in patients, and the incidence rate in women is twice that of men, with more than 1,000 cases in China. Due to its rarity, early diagnosis of the disease is difficult, and it took Celine Dion nearly 5 years to be diagnosed. It is a rare disease that cannot be cured or prevented, and there are only partial treatments to improve symptoms. May Celine Dion and other SPS patients recover soon.
Celine Dion suffered from "stiffness", and after a year, her condition worsened and she was unable to control her muscles
Celine Dion, the singer of the theme song of the movie "Titanic", is suffering the biggest pain of her life: her condition has deteriorated and she can no longer control her muscles!
On December 19, the sister of 55-year-old internationally renowned singer Celine Dion revealed in an interview with Canadian media 7 Jours that her famous sister "has deteriorated and is unable to control her laryngeal muscles." What breaks my heart is that she has always followed the doctor's advice and she has been working very hard. Now we don't seem to see hope. There is no cure for the disease, and she still struggles with a rare neurological disorder. ”
Celine Dion, a music diva who has become popular for many classic songs such as the theme song of "Titanic" "My heart will go on" (My heart will go on), has a high reputation and singing status in the world, and is a five-time Grammy Award winner. She is familiar to the domestic public for her popular "My heart will go on", and in February 2013, she appeared on the CCTV Spring Festival Gala and sang the song "Jasmine" with Song Zuying.
But since November last year, Celine Dion has repeatedly rumored that she suddenly canceled her 21 concert tours because of health problems, and it was rumored that she was almost paralyzed, which made fans very worried about her physical condition.
On December 8, 2022, she posted a video through Instagram, confirming to fans that she suffers from an incurable rare neurological disease "Stiff person syndrome" (Stiff person syndrome), which is the cause of her constant cramps, in addition to causing mobility problems, but also making her unable to sing smoothly with her vocal cords, so she canceled the 2023 concert completely.
Celine Dion said in a video recorded to fans that although there is a medical team around her to treat and improve her disease, she admits that the disease is very difficult to treat, there is no specific drug, and all the current treatments are only to improve her symptoms, everything is very difficult, and emphasized that she is trying to fight the disease, "I miss you very much, and I also want to perform on stage, I am always 100% committed to myself, but now the situation does not allow me to do this, I must focus on my condition first" He promised to actively cooperate with the treatment provided by the doctor to improve his condition.
After Celine Dion was diagnosed last year, she was carefully cared for by her 74-year-old sister, found many top medical teams around the world, and tried a variety of drugs, but unfortunately it still could not delay Celine Dion's condition and alleviate her pain.
In October this year, my sister revealed the good news, saying that Celine Dion does not need a wheelchair to walk, and still plans to return to the entertainment industry. In early November, she was also watching an NHL hockey game in Las Vegas with her son, and she looked in good spirits. I also took a group photo with the players in the lounge. The match was her first public appearance in more than two years. At that time, she seemed to be in good spirits, and once the outside world was full of expectations, thinking that her health had made great progress, and she might soon be able to complete the performance she had stopped before.
But it all seemed to have suddenly spiraled out of control in less than a month. Stiff person syndrome can make the patient's trunk and proximal limbs stiff and tight, and if Celine wants to continue singing, her voice will definitely be affected if she can't control her muscles.
Sister Claudette Dion said: "Whether it's our dream or hers, the goal is to get back on stage. I don't know, but as I understand it, the vocal cords are muscles, and the heart is a muscle. But now she can't control them. And this is a fatal blow to the singer. The most heartbreaking thing for me is that she is always very disciplined and always so hardworking. To be honest, our dream and her are that we want her to be back on stage, but how much can it achieve? I really don't know. We pray that researchers will find a cure for this terrible disease. ”
What is stiff syndrome, why is it called an incurable disease, and how is it different from ALS?
▎Stiff person syndrome (SPS) is an incurable disease that eventually leads to the patient becoming a "statue man" because it gradually locks the body into a stiff position, making it impossible for people to walk or speak.
What is stiff-person syndrome, why is it called zombie disease? There are about 1,000 cases of patients in China
Stiff person syndrome, also known as zombie syndrome or stiff man syndrome, is a rare and disabling autoimmune disease of unknown etiology, in which patients gradually begin to become stiff and rigid. It can cause some patients to develop a hunched back and be unable to walk unaided.
Stiff man syndrome was first described in 1956 as "stiff man syndrome", in which the main clinical manifestations of patients are gradual stiffness of the neck, shoulders and upper back, as well as paroxysmal painful muscle spasms and difficulty walking. Subsequent observations found that the disease was both male and female, so it was renamed stiff person syndrome (SPS). Because many of these patients have other autoimmune diseases, the immune mechanism of this disease has gradually been emphasized.
The disease usually presents with acute or subacute onset and is characterized by muscle tonics and spasms induced by sound, light, touch stimuli, and emotional agitation in the trunk or limb muscles, which last from a few seconds to a few minutes at a time. Muscle spasms can occur at any time, and in severe cases, they can even cause the patient to fall to the ground suddenly and cause fractures. Approximately 1 in 1 million people is affected, and the incidence in women is twice as high as in men. It is understood that more than 1,000 cases have been admitted to the country.
At present, there is no specific drug for stiff person syndrome, and there is no cure or prevention.
Because stiff person syndrome is rare and difficult to diagnose early, many people will confuse stiff person syndrome with ALS, and there is a clear difference between the two: ALS is a motor neuron disease, which is a neurodegenerative disease, while stiff person syndrome is related to genetics, viral infections and autoimmunity, and is an autoimmune disease.
In addition, early diagnosis should rule out ankylosing spondylitis, rheumatic arthropathy, paraneoplastic syndromes, etc. May be distinguished from the stiff spasms of tetanus and rabies. It has also been reported that some people with stiff person syndrome will also have tumors.
From onset to diagnosis, Celine Dion took nearly 5 years.
In 2016, after her husband Rene died of cancer, Celine Dion suffered from muscle spasms, hearing impairment, retinopathy and other diseases due to excessive grief and fatigue. At the time, doctors thought she was just ordinary muscle spasms, and it wasn't until her condition worsened that she was diagnosed with stiff-person syndrome in December last year.
Zombie syndrome mostly occurs in adulthood, 6 to 80% of patients, blood and cerebrospinal fluid will appear anti-glutamic acid decarboxylase antibody, this enzyme is required for the synthesis of a central nervous system inhibitory conduction substance GABA, if it is damaged by antibodies, it may lead to the deficiency of central nervous system inhibitory function, so that the muscles produce excessive activity, and then become stiff.
There is currently no effective cure for stiff-person syndrome. Medication also only improves symptoms. Current benzodiazepines (such as diazepam and clonazepam) or baclofen can improve muscle stiffness and spasticity, and antiepileptic drugs can reduce pain. In some cases, anti-inflammatory drugs and corticosteroids may be used to improve pain episodes. However, because the disease is a mechanism of autoimmune dysregulation, the disease can be improved with immunotherapy drugs, plasmapheresis, and immunoglobulin injections.
There have been reports in China that a case of stiff person syndrome has been "cured". In 2021, Guangdong Keyuan People's Hospital reported that a 44-year-old female patient with stiff person syndrome was admitted to the hospital, and returned to normal after treatment. No recurrence has been reported in this case.
It is understood that some patients with SPS respond well to the drug, but about 10% of patients have autonomic dysfunction due to repeated spasms or abrupt discontinuation, which eventually leads to sudden death.
May Celine Dion recover soon.