Stiff-person syndrome: What is the rare condition Celine Dion has? - explainer

Stiff-person syndrome is a rare neurological disorder, occurring in about one in a million people, causing chronic pain, spasms and impaired mobility.

Celine Dion (photo credit: Courtesy)
Celine Dion
(photo credit: Courtesy)

Canadian singer and pop music icon Celine Dion announced on Thursday, December 8, 2022 that she has been diagnosed with stiff-person syndrome, thus necessitating the postponement of her 2023 tour.

And she isn't alone in suffering from this disease either.

Also known as SPS, stiff-person syndrome is a rather rare neurological disorder, occurring in about one in every one million people, mainly those who are middle-aged – like the 54-year-old Celine Dion. The condition itself is somewhat debilitating, impairing mobility and causing chronic pain, but also causing spasms. 

But what causes stiff-person syndrome? Is it fatal? Can it be treated? What can aggravate it?

Here is everything you need to know.

Singer Celine Dion arrives to attend the Haute Couture Fall/Winter 2019/20 collection show by designer Alexandre Vauthier in Paris, France, July 2, 2019 (credit: REUTERS/REGIS DUVIGNAU)Singer Celine Dion arrives to attend the Haute Couture Fall/Winter 2019/20 collection show by designer Alexandre Vauthier in Paris, France, July 2, 2019 (credit: REUTERS/REGIS DUVIGNAU)

What is stiff-person syndrome?

Stiff-person syndrome is a rare disorder that causes parts of the body, specifically the truncal muscles in the torso, to become stiff and rigid.

This stiffness also can cause spasms, severely impact one's posture and causes chronic pain.

The stiffness may come and go at first, but it then it starts lasting longer, consistently impacting the body and impairing mobility. Patients may start to struggle to bend or walk. Chronic pain usually sets in and gets worse as time goes on, and acute pain can manifest as well.

The spasm attacks can be severe, lasting several minutes.

What triggers stiff-person syndrome?

Some things like fast movements, stress, sudden sounds or touches can trigger these spasms, but they can also be entirely unpredictable.

Stiff-person syndrome patients can also become incredibly sensitive to sounds and touch, which can trigger symptoms.

Can stiff-person syndrome get worse?

All symptoms can increase and worsen due to a number of factors, such as cold weather and stress. However, going to sleep can help calm symptoms down.

The symptoms can cause stiff-person syndrome patients to frequently fall down and suffer severe injuries.

There are other types of stiff-person syndrome that can manifest as well. For example, paraneoplastic stiff-person syndrome impacts the neck and arms more severely and the overall condition progresses far more quickly.

Then there is stiff-limb syndrome, which can turn into full SPS as time goes on. This starts impacting just one limb and stays more severe there, but it can continue to get worse and can impact both the brain and the sphincter. However, the spasms and stiffness usually stays limited to the legs.

Can stiff-person syndrome stop you from working and living a normal life?

Absolutely.

Stiff-person syndrome's spasms alone can trigger severe ramifications in one's daily life. It is typically associated with increased feelings of fear, anxiety, depression and numerous types of phobias. 

Patients can also not be able to work and may need assistance in their daily lives.

The fact that stiff-person syndrome can make it difficult to move or handle being around loud sounds can only make it even harder to properly function. 

This latter point in particular could further explain why Celine Dion had to cancel her summer 2023 tour.

What makes it worse, though, is that the condition is very difficult to diagnose, and doctors will often think symptoms are psychosomatic. It can take years before stiff-person syndrome is properly diagnosed.

What causes stiff-person syndrome?

Stiff-person syndrome is somewhat mysterious, and scientists aren't sure what causes it. There are some theories, and there is some agreement that glutamic acid decarboxylase (GAD) antibodies play a key role. But we don't know how. After all, while GAD antibodies are only in 1% of the population but in almost all SPS patients, most people with GAD antibodies don't have SPS.

There is also a theory that stiff-person syndrome may be genetic, and a significant percentage of SPS patients also have type 1 diabetes, but these links are also unclear.

Is stiff-person syndrome fatal?

It can be.

Eventually, stiff-person syndrome can continue to worsen, even with all efforts made to limit its progression. 

Eventually, stiff-person syndrome patients can die from sudden death, such as from blood acidity issues. However, this doesn't happen with everyone.

Can stiff-person syndrome be cured?

No, but its symptoms can be treated.

Technically, there are no agreed-upon treatment methods for stiff-person syndrome. However, there are some drugs that have been used.

The most commonly used treatment is an anti-anxiety drug known as benzodiazepines, also known as benzos, which are known to be useful in treating anxiety, panic disorders and muscle spasms.

However, there are issues with this, and others, such as the US National Institutes of Health, have advocated for the use of intravenous immunoglobulin (IVIg) treatment to reduce stiffness and lower sensitivity.