What is Stiff Person Syndrome? Celine Dion's incurable illness explained

21 June 2023, 14:22

Fans across the world were in shock when the Canadian singer, Celine Dion, announced she had an incurable illness in December 2022.

When Celine Dion gave a statement to say she was suffering from an illness called Stiff Person Syndrome, she shed light on an illness many people had never heard of.

The Canadian songstress is suffering from an illness so rare that only 'one in one million people' in the world suffer from the condition.

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So just what is Stiff Person Syndrome, its symptoms and its prognosis? Here is everything you need to know about the rare disease:

What is Celine Dion's illness, Stiff Person Syndrome?

December 2022 saw Celine Dion make a statement to fans across the world confirming she had a rare illness.

The 54-year-old singer said on Instagram that she had SPS, which leaves people as "human statues", locking the body into rigid positions.

What is stiff-person syndrome?

According to John Hopkins Medicine, Stiff Person Syndrome is "a rare autoimmune neurological disorder that most commonly causes muscle stiffness and painful spasms that come and go and can worsen over time.

"SPS most commonly develops in people ages 40 to 50, but in rare cases, it occurs in children and older adults. SPS is thought to affect one to two people in a million."

Celine told fans that because of her deteriorating health, she was forced to cancel her European tour.

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"Hello everyone, I'm sorry it's taken me so long to reach out to you. I miss you all so much and can’t wait to be on stage talking to you in person.

"As you know I’ve always been an open book and I wasn’t ready to say anything before but I'm ready now.

"While we’re still learning about this rare condition, we now know this is what’s been causing all the spasms I’ve been having," she added.

"Unfortunately, these spasms affect every aspect of my daily life sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I'm used to."

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She explained that she has a "great team of doctors working alongside her" and is being supported by her "precious children".

What are the symptoms of Stiff Person Syndrome?

The symptoms of Stiff Person Syndrome are wide-ranging and depend on the type of SPS the patient is diagnosed with, but according to John Hopkins Medicine, there are some common symptoms that occur.

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"Stiff person syndrome most often causes painful muscle contractions and spasms that often begin in the legs and back," says a statement from the research facility.

"The spasms can occur in episodes, especially when a person with SPS is surprised or startled, or moves suddenly. Cold temperatures and emotional stress can also set off a muscle spasm. In some people, the spasms are brought on by certain exercises or touch.

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"The areas where muscle contractions occur can be stiff and board-like," John Hopkins Medicine says, before adding a list of symptoms including: Difficulty walking, unsteadiness and falling because of sudden spasms, shortness of breath if SPS affects muscles in the chest and chronic pain.

How is Stiff Person Syndrome diagnosed and treated?

Often misdiagnosed as Parkinson's disease, multiple sclerosis, fibromyalgia or anxiety, according to the National Institute of Neurological Disorders and Stroke (NIHDS), a definitive diagnosis can be made with a blood test that measures the level of GAD antibodies.

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A post shared by Céline Dion (@celinedion)

NIHDS states: "Scientists don't yet understand what causes SPS, but research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord.

The institute goes on to say: "With appropriate treatment, SPS symptoms may be kept under control. 

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"Several symptoms improve with oral diazepam (an anti-anxiety and muscle relaxant drug) or with drugs that alleviate muscle spasms, such as baclofen or gabapentin."

John Hopkins Medicine confirms there is no cure for SPS, but the symptoms can be managed.

"Although there is no treatment that cures stiff person syndrome, working with a specialist and maintaining symptom control can make it easier to live with the condition."

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What is Celine Dion's life like with Stiff Person Syndrome?

Living with SPS can cause some sufferers to be afraid to leave the house and become agoraphobic because a variety of common sudden noises can bring on symptoms.

"People with SPS may be afraid to leave the house because street noises, such as the sound of a car horn, can trigger spasms and falls," the NIHDS explains.

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"Over time people with SPS may develop hunched over postures. Some people may be too disabled to walk or move. Many fall frequently because they do not have the normal reflexes to catch themselves. This can lead to serious injuries."

Celine announced on May 26 that she was cancelling her entire world tour as her Stiff Person Syndrome condition worsened.

She said it was with "tremendous disappointment" she was unable to perform on her world tour, and that she's "working really hard to build back her strength."

Living with Stiff Person Syndrome

A source close to Celine Dion recently said the singer can now 'barely move' since her diagnosis that the singer is in a 'lot of pain'.

"She has the best medical team money can buy, but things aren’t looking good," they told Radar.

However, Celine Dion's sister, Claudette, is determined the singer will stay positive throughout her ordeal.

"I am confident that life will give her back what she gave because she is an extremely intelligent woman, so very generous and talented and in love with life as well," she said in February 2023.

"Rather than crying over her fate, I try to send her positive vibes in the hope that one day she will go back on stage," she added.

"I truly believe in the power of love. I wish her the best of luck, that's for sure. She is surrounded by specialists as she continues to recover."