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Canadian singer Céline Dion has been praised for bravely sharing heartbreaking images of what it’s like to live with stiff person syndrome (SPS).
In the terrifying clip, Dion, 56, is seen gasping for air and moaning in pain unable to control her own body.
Doctors are seen desperately easing his agonizing spasms as a film crew silently documents the episode.
Dion insisted the entire scene be included in her new documentary I Am: Céline Dion on Amazon Prime, a heartbreaking look at how the star lives with SPS.
Speaking directly afterwards, she added: “Every time something like this happens, it makes me feel so embarrassed.”
“I don’t know how to put it, you know, not having control over yourself.”
Dion revealed her SPS diagnosis in 2022, but had secretly lived with the disease for 17 years.
SPS is an autoimmune condition, meaning the symptoms are caused by the immune system, which normally protects the body by attacking pathogens, mistakenly damaging healthy tissue.
For SPS sufferers, this involves blocking an enzyme that regulates the body’s nervous system, which governs our ability to control our movements and react to stimuli, from functioning properly.
This can cause extreme muscle stiffness that severely affects mobility and can turn those affected into “human statues”, leaving them with difficulty walking or talking.
As Dion demonstrated in his documentary, it can also cause spasms when nerve cells fail and have enough force to potentially fracture bone.
Other signs of this condition include increased sensitivity to noise, sudden movements, and emotional distress as natural reactions fail.
This can mean that something as simple as knocking on the door can lock a patient inside their own body.
Here MailOnline explains everything we know about this extremely rare condition:
What is stiff person syndrome?
Stiff person syndrome (SPS) is an extremely rare disorder that causes the muscles of the torso and extremities to alternate between spasms and stiffness.
It has been called the “human statue disease.” The spasms it causes can be so severe that they dislocate joints and break bones.
Estimates suggest it affects around 70 people in the UK and only 330 in the US. Approximately twice as many women as men suffer from it.
It is believed to be so rare that it only affects one or two people in every million.
SPS usually develops among people aged 40 to 50, but rarely affects younger or older people as well.
Women are also generally more likely to develop SPS.
The disease becomes more severe over time and can paralyze patients, forcing them to use a walker or wheelchair.
Céline Dion first revealed that she suffered from one-in-a-million stiff-person syndrome in December 2022. She photographed the singer in 2016 during a performance in Paris.
There are three main types of syndrome:
- Classic stiff person syndrome: When stiffness and spasms occur around the back and stomach, and occasionally in the thighs and neck. It can cause curvature of the back over time.
- Stiff limb syndrome: Spasms especially affect the legs and feet, sometimes causing them to stay fixed in one place. The hands may also be affected.
- Stiff and shaken person syndrome: The rarest and most aggressive form, which includes symptoms of the others and also affects the head and eyes.
What causes it?
Experts still don’t know what causes the disease.
But they believe it may be caused by an autoimmune reaction when the body attacks its own nerve cells that control muscle movement.
About 40 percent of sufferers also have type 1 diabetes, another autoimmune disease. Type 1 diabetes is particularly associated with classic person syndrome.
Other autoimmune diseases are also associated, such as vitiligo, which causes white spots on the skin, and pernicious anemia, a deficiency of red blood cells.
The condition is also more common in people with breast, lung, kidney, thyroid or colon cancer, as well as lymphomas, but researchers still don’t know why.
In stiff person syndrome, the immune system attacks a protein that helps produce gamma-aminobutyric acid (GABA), which regulates motor neurons, the nerves that control movement.
Low levels of GABA cause neurons to fire continuously when they shouldn’t, causing twitching and stiffness.
What are your symptoms?
The main symptoms of stiff person syndrome are spasms and stiffness of the torso and extremities.
Spasms can be triggered by loud noises, and the condition also causes increased sensitivity to sound.
Touch and emotional distress may also be felt more intensely as a result of this condition.
The spasms can be so severe that they can cause people to fall or lead to difficulty walking and other disabilities.
Stress and anxiety also tend to be higher in those who suffer from this condition, especially due to the unpredictability of the spasms.
Lack of GABA (which regulates anxiety) in your system also affects mental health.
In a trailer for the film, which will be released on June 25, Céline broke down in tears as she talked about the illness and the effects it has had on her.
How is it diagnosed?
Because of its rarity and puzzling symptoms, which are often confused with Parkinson’s disease or multiple sclerosis (MS), diagnosing the syndrome can be time-consuming.
But if doctors suspect stiff-person syndrome, they can confirm it with two tests.
The first looks for antibodies in the blood against the protein mentioned above, called glutamic acid decarboxylase (GAD).
Elevated levels of GAD antibodies indicate that stiff person syndrome may be occurring, although levels are also elevated in people with type 1 diabetes.
The second test is an electromyogram (EMG), which evaluates the health of your muscles and motor neurons.
Doctors insert a needle directly into the affected muscles and record the electrical activity in them.
Is there a cure?
No. However, treatments can be given to help control symptoms.
Medications such as diazepam and baclofen, which control spasms, can help regulate episodes and reduce stiffness.
Some patients with more severe symptoms are also given therapies to manipulate their immune system and increase GABA levels.
In some cases, immunoglobin transfusions may be given to affect antibody levels in the blood.
Medications such as sedatives and steroids may also be prescribed, again to relieve symptoms.
Meanwhile, patients are often prescribed physical therapy and aquatic therapy to improve the functioning of their muscles.
Can it be fatal?
Yes, but this is usually not caused directly by the condition itself.
Instead, deaths from SPS generally result from subsequent injuries or infections sustained from the spasms.
Blood clots have also been reported in people who became immobile due to this condition.
In extreme cases, SPS spasms in the chest muscles can also dangerously inhibit a person’s ability to breathe.