Celine Dion sang "Ode to Love" at the Paris Olympics. What kind of disease is her "stiff syndrome"?

in #tqg2 months ago

Rigid syndrome (SPS) is a rare neurological disease that may cause uncontrollable muscle tension and stiffness, affecting movement and singing. Moreover, there are currently no specific drugs for SPS, which can only rely on immunotherapy drugs and other symptoms.
On July 26 local time, the Paris Olympic Games were held romantically in the rain. At the opening ceremony, the famous Canadian singer Celine Dion sang "Ode to Love" at the finale. This is the first time she has performed in public since she announced her illness last year.
When Celine Dion appeared on the second balcony of the famous landmark Eiffel Tower in Paris, the scene was full of joy. People paid tribute to the legendary female singer who insisted on fighting against the disease while insisting on appearing on the stage.
In a speech last month, Celine Dion thanked neuroscientist Amanda Piquet to help find hope in the treatment of desperate rigid syndrome, and she began to feel that "a new day is coming".
Last December, Celine Dion announced that she had diagnosed "stiffness syndrome" (SPS), a rare neurological disease that may lead to uncontrollable muscle tension and stiffness, affecting movement and singing. Moreover, there are currently no specific drugs for SPS, which can only rely on immunotherapy drugs and other symptoms.
Professor Tu Jintai of the Department of Neurology of Huashan Hospital Affiliated to Fudan University told the First Financial Reporter: "There are many causes of zombie syndrome, which are not completely clear at present. It is mainly believed to be related to factors such as autoimmune, para-tumor and genetics."
He also said that although SPS does not yet have specific drugs, judging from several patients he has diagnosed and treated, the improvement of patient symptoms after immunization and symptomatic treatment is still very obvious.
According to reports, nearly half of SPS patients have autoimmune-endocrine diseases, such as diabetes, thyroid disease, vitiligo, malignant anemia and rheumatoid diseases. On the other hand, a variety of autoantibodies can be detected in patients' serum and cerebrospinal fluid, of which anti-GAD65 antibodies are the most common; in addition, there is a familial aggregation phenomenon.
Zombie syndrome does not have a specific age of onset. The median age of onset is between 35 and 40 years old, and women are 2-3 times more likely to develop the disease of men. This disease usually occurs intermittently or repeatedly.
The early diagnosis of SPS is also difficult. Due to the diversity of initial sites and atypical symptoms, patients will cause severe disabilities due to delays in treatment.
Complications mainly include fractures and joint dislocation, severe autonomic nerve disorders and muscle spasm pain, falls, eating imbalances, phobias, post-traumatic stress disorders, hypoxemia respiratory failure, esophageal obstruction caused by pharynx muscle spasm, etc.
Stiffness and spasm usually begin with leg and torso muscles, especially the waist and back, which affect the arms and even the face over time. There may also be other symptoms, such as unstable gait and unexplained falls.
Stimuli such as noise are the cause of spasm in SPS patients. Due to the lack of stress response, it is manifested in a unique way of falling, such as wood-like and statue-like fall. In the medium term, it is often manifested in the state of proximal limb involvement and frightening, and the limbs can have severe painful spasms to relieve retardation. At this time, the patient's quality of life has seriously declined, often accompanied by depressive symptoms. In the late stage, almost all muscle groups are seriously affected, and usually simple daily life is obviously limited. At this time, patients have significant pain symptoms and often rely on painkillers.

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