Stiff Person Syndrome
Celine Dion announced, Thursday, December 8, 2022, the cancellation of part of her tour due to her health problems. The singer says she suffers from a very rare neurological disorder, the Stiff Person syndrome. Here is what we know about this disease.
Stiff-Person syndrome, or stiff-person syndrome (SPS) from which singer Céline Dion suffers, is a rare neurological condition characterized by progressive muscle stiffness and repeated episodes of painful muscle spasms.
Symptoms that change over time
This syndrome is an extremely rare disease. According to the North American organization, which aims to provide support for people with rare diseases, the incidence would be around one person in a million, as Celine Dion claims in her video.
According to Orphanet, the portal for rare diseases and orphan drugs, the peak incidence is around age 45 and symptoms evolve over months or years. Muscle stiffness often fluctuates, i.e. it gets worse then better, and usually occurs at the same time as muscle spasms.
These spasms can occur randomly or be triggered by a variety of different events, including sudden noise or light physical contact. If left untreated, SPS can potentially progress to difficulty walking and significantly impact a person’s ability to perform simple daily tasks.
An autoimmune disease?
Although the exact cause of this syndrome is unknown, it is believed to be an autoimmune disease and sometimes occurs with other autoimmune diseases.
The diagnosis is essentially based on clinical observation and is confirmed by the demonstration of certain characteristic antibodies and electromyographic abnormalities (an essential examination of neurological diseases, editor’s note). A CT scan of the spinal cord can help rule out mechanical causes such as the presence of a herniated disc or a cyst in the spinal cord.
Causes still unknown
Researchers do not yet understand the causes of this very debilitating and very rare syndrome, but research indicates that it is the result of an autoimmune response in the brain and spinal cord.
This disorder is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, anxiety or phobia.
A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) in the blood. Affected individuals have elevated levels of GAD, an antibody that acts against an enzyme involved in the synthesis of an important neurotransmitter in the brain.
Most sufferers are women
The first cases were documented in 1956. Stiff person syndrome has been described in the medical literature under many different names. Originally described as stiff-man syndrome, the name has been changed because the disorder can affect people of any age and both genders. In fact, most people with the disease are women.
A treatment mainly to relieve symptoms
Treatment is aimed at relieving the specific symptoms that occur in each individual, which often requires non-drug approaches (stretching, heat therapy, aqua therapy, massage therapy, acupuncture, etc.) Some medications can also be used to treat muscle stiffness and pain. episodic spasms.
Celine Dion trains “every day” with her therapist
“I have an excellent team of doctors by my side who treat me so that I get better”, detailed Celine Dion. “I train every day with my sports medicine therapist to regain strength and endurance, she continues in her video, “to allow me to perform again. It’s a continuous fight”, she breathes in the image, before thanking her fans for their messages of encouragement and their support.
Information: Cleverly Smart is not a substitute for a doctor. Always consult a doctor to treat your health condition.
Sources: PinterPandai, NBC News, The New York Times, The Sun
Photo credit: FerminDion (CC BY-SA 4.0) via Wikimedia Commons
Photo description: Céline Dion live concert at Hyde Park, London. July 2019.
