What mysterious illness is afflicting Céline Dion after the singer bravely released a film about an agonizing seizure… and who is at risk?

Canadian singer Céline Dion has been praised for bravely sharing harrowing footage of what it’s like to live with stiff person syndrome (SPS).

In the terrifying clip, 56-year-old Dion is seen gasping for air, screaming in pain and unable to control her own body.

Paramedics are seen desperately trying to ease her excruciating cramps while a film crew silently documents the incident.

Dion insisted that the entire scene be included in her new Amazon Prime documentary, “I Am: Céline Dion,” a heartbreaking look into the star’s life with SPS.

Immediately afterwards, she added: “Every time something like this happens, I am so embarrassed.”

“I don’t know how to express it, you know, not having control over yourself.”

Dion announced her SPS diagnosis in 2022, but had been living with the disease in secret for 17 years.

SPS is an autoimmune disease, meaning the symptoms are caused by the immune system, which normally protects the body by attacking pathogens and mistakenly damaging healthy tissue.

In SPS patients, this manifests itself in the blocking of an enzyme that regulates the proper functioning of the body’s nervous system and thus controls our ability to control our movements and respond to stimuli.

The result can be extreme muscle stiffness, which severely impairs mobility and can turn those affected into “human statues” so that they can hardly walk or speak.

As Dion shows in her documentary, seizures can also occur because the misfiring of nerve cells is so great that it can potentially cause bone fractures.

Other signs of the disease include increased sensitivity to noise, sudden movements and emotional stress – as natural reactions fail.

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This can mean that something as simple as a knock on the door traps a patient in their own body.

Here MailOnline explains everything we know about this extremely rare disease:

What is stiff person syndrome?

Stiff person syndrome (SPS) is an extremely rare condition in which the muscles in the trunk and limbs alternately spasm and stiffness.

It is also known as the “human statue disease.” The spasms it causes can be so severe that they dislocate joints and break bones.

It is estimated that around 70 people are affected in the UK, compared to just 330 in the US. Women and men are affected about twice as often.

It is probably so rare that it affects only one or two people in a million.

SPS typically occurs in people between 40 and 50 years of age, but in rare cases it also occurs in younger or older people.

Women are also more susceptible to developing SPS overall.

Over time, the disease worsens and can lead to physical disabilities, requiring patients to use a walker or wheelchair.

There are three main types of the syndrome:

• Classic stiff-person syndrome: When stiffness and cramps occur in the back and abdomen, and occasionally in the thighs and neck. This can lead to a curvature of the back over time.
• Stiff limb syndrome: The spasms mainly affect the legs and feet and sometimes cause them to become rigid in position. The hands can also be affected.
• Twitching stiff-person syndrome: The rarest and most aggressive form, which includes symptoms of both forms and also affects the head and eyes.

What causes it?

The cause of the disease is not yet known to experts.

However, they believe the cause could be an autoimmune reaction in which the body attacks its own nerve cells that control muscle movement.

About 40 percent of those affected also have type 1 diabetes, another autoimmune disease. Type 1 diabetes is particularly associated with the classic person syndrome.

Other autoimmune diseases such as vitiligo, which causes white patches on the skin, and pernicious anemia, a deficiency of red blood cells, are also associated with it.

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The disease also occurs more frequently in people with breast, lung, kidney, thyroid or colon cancer, as well as lymphomas. However, researchers do not yet know the cause.

In stiff person syndrome, the immune system attacks a protein involved in the production of gamma-aminobutyric acid (GABA), which in turn regulates motor neurons – the nerves that control movement.

Low GABA levels cause neurons to fire continuously when they shouldn’t, leading to spasms and rigidity.

What are the symptoms?

The main symptoms of stiff person syndrome are cramps and stiffness of the trunk and limbs.

Loud noises can trigger cramps. It can also lead to increased sensitivity to noise.

Touch and emotional stress can also be felt more intensely due to the illness.

The spasms can be so severe that they can cause falls or lead to difficulty walking or other disabilities.

Stress and anxiety are also typically higher among those affected, particularly due to the unpredictability of the cramps.

The lack of GABA – which regulates anxiety – in their system also affects mental health.

How is it diagnosed?

Because of its rarity and mysterious symptoms – the syndrome is often confused with Parkinson’s disease or multiple sclerosis (MS) – diagnosis can take a long time.

However, if doctors suspect stiff person syndrome, they can confirm it through two tests.

The first looks for antibodies in the blood against the previously mentioned protein, the so-called glutamic acid decarboxylase (GAD).

High levels of GAD antibodies may indicate stiff person syndrome, although levels are also elevated in people with type 1 diabetes.

The second test is an electromyogram (EMG), which assesses the health of the muscles and motor neurons.

Doctors insert a needle directly into the affected muscles and record the electrical activity there.

Is there a cure?

No. However, treatments can be done to keep symptoms under control.

Medications such as diazepam and baclofen – both spasm control agents – can help regulate seizures and reduce stiffness.

Some patients with more severe symptoms also receive therapies to manipulate their immune system and increase GABA levels.

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In some cases, immunoglobulin transfusions may be given to influence antibody levels in the blood.

Medications such as sedatives and steroids may also be prescribed to relieve symptoms.

Patients are now often prescribed physical and aquatic therapy to improve muscle function.

Can it be fatal?

Yes, but this is usually not a direct result of the disease itself.

Rather, deaths from SPS are usually due to injuries or subsequent infections caused by the spasms.

Blood clots have also been reported in people who have been immobilized due to this condition.

In extreme cases, SPS spasms in the chest muscles can also dangerously impair a person’s breathing.