What is Stiff Person Syndrome? The 'human statue' condition afflicting Celine Dion

Celine Dion announced she had been diagnosed with Stiff Person Syndrome in 2022. Credit: AP

Stiff Person Syndrome (SPS) is a rare neurological condition that can take years to diagnose and requires tailored treatment.

In 2022, singer Celine Dion revealed she had been diagnosed with SPS, which causes spasms that affect her ability to walk and sing.

She has remained largely out of the spotlight since and was forced to cancel her Courage World Tour in 2023 after experiencing difficulties living with the condition.

But on Saturday, she said she is “deeply grateful” for the support from her family and fans as she opened up about her experience of SPS.

She wrote on Instagram: “Trying to overcome this autoimmune disorder has been one of the hardest experiences of my life, but I remain determined to one day get back onto the stage and to live as normal of a life as possible."

What is Stiff Person Syndrome?

SPS is a rare, progressive neurological disorder of the central nervous system and affects around one in a million people, according to the National Organization for Rare Disorders (NORD).

If left untreated it can lead to permanent disability or death.

The symptoms usually develop slowly over several months or years, often beginning with muscle stiffness and discomfort which can come and go - but the stiffness gradually becomes fixed.

Some develop a "statue-like" appearance due to the rigidity.

Many also experience painful muscle spasms that can last a few minutes to several hours and may affect the whole body or certain muscles. The spasms can be triggered by unexpected loud noises, minor physical contact, cold environments, stress, or situations that cause heightened emotions, says the NORD.

Spasms in the chest and respiratory muscles can be serious and require ventilation or emergency treatment, while spasms in the legs often lead to falls.

Experts still do not know the exact cause of SPS and it is often underdiagnosed.

Who is affected by Stiff Person Syndrome?

The condition is twice as likely to affect females than males, according to the National Institute of Neurological Disorders and Stroke.

It is frequently associated with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo, and pernicious anemia.

The condition only occurs in about one in every million people.How is Stiff Person Syndrome treated?

There is no cure for SPS but treatment, such as anti-seizure drugs, painkillers and anti-anxiety medication, can help lessen symptoms.

The condition is thought to have features of an autoimmune disease, according to the National Institute of Neurological Disorders and Stroke.

Several symptoms improve with anti-anxiety and muscle relaxant drugs, or with drugs that alleviate muscle spasms, such as baclofen or gabapentin.

For a singer like Dion, bright lights and loud noises that could occur while performing could trigger muscle spasms.

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