Celine Dion has stiff person syndrome: What is the rare neurological disorder?

My Heart Will Go On fame singer Celine Dion has revealed she has stiff person syndrome (SPS), a rare neurological disorder.

In an emotional video shared on her Instagram handle, Dion said, “Recently, I’ve been diagnosed with a very rare neurological disorder called the stiff-person syndrome, which affects something like one in a million people. While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having. 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.”

The Canadian singer said she had no choice but to postpone her “Courage” tour, which was to begin in February next year.

As per Associated Press (AP), the singer’s spring 2023 shows have been shifted to 2024 and her summer concerts next year have been called off.

What is stiff person syndrome affecting Celine Dion? What are its symptoms, cause and treatment? Is there a cure? Let’s take a closer look.

Stiff person syndrome

Stiff person syndrome, also known as Moersch-Woltman syndrome, is a progressive neurological disorder that affects one or two in a million people, says Johns Hopkins Medicine.

The condition causes muscle stiffness or rigidity and frequent painful muscle spasms.

“Muscular rigidity often fluctuates (i.e., grows worse and then improves) and usually occurs along with the muscle spasms,” says National Organization for Rare Disorders (NORD).

These spasms can occur randomly or be triggered by a sudden noise, light physical contact or even emotional stress.

“Most commonly, it affects the muscle skeletal system where people have really a significant amount of pain, spasms that can really affect any muscle within the body,” Dr Scott Newsome, a neurologist at the Johns Hopkins University School of Medicine told CTV News Channel.

The severity of the disease varies from one person to another.

As per Newsome, the disorder affects twice as many women as men, reported CTV News Channel.

Though it usually occurs between the age of 30-60 years, it has also been reported in children and older adults, as per NORD.

Symptoms of SPS

The symptoms of SPS include stiffening of muscles in the torso and limbs, as well as episodes of “violent” muscle spasms, as per Yale Medicine.

Those who have the disorder may experience “aching discomfort, stiffness, or pain, especially in the lower back or legs”, says NORD.

“We all have muscle spasms, but these are muscle spasms that are beyond your control to the point that muscles lock and are so rigid. There’s truncal dystonia,” Dr Robert Wilson, a neurologist told USA Today.

Elaborating further, Wilson said, “The limb can look distorted and contorted. I’ve seen people actually break a limb from it”.

This can affect walking, and individuals can also develop hunched or slouched posture or an arched back, says NORD.

The disorder hampers people’s daily lives and activities.

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Cause of stiff person syndrome

The exact cause of SPS is not known. But it has features of an autoimmune disorder, notes the National Institute of Neurological Disorders and Stroke.

“Autoimmune disorders are caused when the body’s natural defenses (e.g., antibodies) against ‘foreign’ or invading organisms begin to attack healthy tissue for unknown reasons,” says NORD.

The immune system targets glutamic acid decarboxylase (GAD), which helps to produce gamma-aminobutyric acid (GABA). This leads to a deficiency of GABA in the body whose job is to regulate muscle movement.

As per Yale Medicine, as many as 60-80 per cent of people with SPS have anti-GAD antibodies in their blood and cerebrospinal fluid.

It is also more common in people with cancer of the breast, lung, kidney, thyroid or colon, says Yale Medicine.

Treatment and cure

Newsome says a “multifaceted” approach should be adopted for the treatment of the disorder.

“The gold standard is treating people with muscle relaxers, but given that it’s an autoimmune condition, we do use immune-related therapies to help and then other non-pharmacologic therapies (like) occupational therapy” he was quoted as saying by CTV News Channel.

There is “no cure” for stiff person syndrome, says Yale Medicine website.

Doctors prescribe sedatives, muscle relaxants, and steroids to treat patients.

Conservative therapies like stretching, heat therapy, aqua therapy, massage therapy, and acupuncture can also help individuals with SPS, as per Barrow Neurological Institute.

Intravenous immunoglobulin and plasmapheresis are also prescribed for treating SPS patients, says Yale Medicine.

With inputs from agencies

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