Movies

What is stiff person syndrome? Celine Dion reveals a rare diagnosis

December 10, 2022

Celine Dion revealed she was diagnosed with stiff person syndrome on Thursday.
Stiff-person syndrome is a very rare neurological disorder that causes progressive muscle stiffness and painful spasms.

In a video posted Thursday about the future of her world tour, Celine Dion shared that she had been diagnosed with stiff person syndrome.

The Canadian singer said the rare neurological disorder is what caused her severe muscle spasms, affecting her ability to walk and sing. The diagnosis means she will have to postpone much of her tour, as well as cancel some shows.

“The spasms affect all aspects of my daily life, sometimes causing difficulty when walking and not allowing me to use my vocal cords to sing as I normally do,” Dion said in the video posted on Instagram. “I have to admit it was a struggle. All I know is sing, that’s what I’ve done all my life.”

Dion said she now had to focus on her health and was working with “an excellent team of doctors”. She said she hopes to perform again in the future.

Stiff-person syndrome is a rare neurological disorder that affects one or two in a million people, according to Johns Hopkins Medicine. The disease notably causes progressive muscle stiffness and painful spasms. Treatments focus on relieving symptoms.

Celine Dion’s announcement:Singer says she has stiff person syndrome, cancels and postpones 2023 tour dates

“It’s a condition characterized by progressive muscle stiffness, muscle spasms, rigidity – usually in the muscles of the back, especially the lower back, as well as the upper legs,” said Dr Kunal Desai , a neurologist at Yale Medicine and an assistant professor of neurology specializing in neuromuscular diseases, told USA TODAY.

“It can affect other muscles, including arm muscles as well as speech and swallowing muscles,” he said. “But it’s less common.”

Here’s what you need to know.

What is stiff person syndrome?

Stiff person syndrome, or SPS, is a “rare neurological disorder with features of an autoimmune disease,” notes the National Institute of Neurological Disorders and Stroke.

The condition causes “progressive muscle stiffness and painful spasms” that can be triggered by environmental factors such as “sudden movement, cold temperature, or unexpected loud noises,” Johns Hopkins Medicine said.

With extreme muscle spasms, “the muscles can be tense like a rock. So it can be very, very uncomfortable and painful,” Desai said, adding that other stimuli that can cause these spasms sometimes include touching the muscles, ” certain subtle movements” and stress.

Do you suffer from lower back pain? Maybe it’s time to get out of the medicine cabinet

What are the symptoms of PSS?

The main symptoms of PSS include muscle stiffness and painful muscle spasms which can be brought on by emotional stress or, again, by outside stimuli. It’s possible that these spasms are “so severe that they cause the person to fall,” says Yale Medicine.

“We all have muscle spasms, but these are muscle spasms that are beyond your control to the point where the muscles lock up and are so stiff. There’s trunk dystonia,” says Dr. Robert Wilson, neurologist and director of the Cleveland Autonomous Center. Clinic, told USA TODAY, expanding on some serious cases he has seen. “The limb may look deformed and twisted. I’ve seen people break a limb.”

These symptoms can lead to difficulty walking, among other disabilities. People with PSS are also more likely to suffer from anxiety and depression, Yale Medicine notes – noting how unpredictable the disease can be and because patients “have lower levels of the neurotransmitter GABA, which regulates anxiety. “.

“I wish I could live a normal life”:What your friend with an anxiety disorder would like you to know

The NINDS adds that many people with PSS “fear to leave the house” due to environmental stimuli, such as loud noises, which can trigger episodes.

Are the symptoms the same for everyone?

Of course, the symptoms of PSS can vary widely and each patient’s experience is unique. “It’s a gravity spectrum,” Desai says.

“Some people have mild spasms. Some people can have full dystonia where one part of the body becomes almost board-like or statue-like. … And some people can be constant, with a 24/7 lockdown , or other people might just (have) episodes,” Wilson said. “There is variability in every patient.”

In rare cases, Desai and Wilson note, stiff-person syndrome has also been linked to cancer.

How rare is SPS?

SPS is considered very rare because the disease is expected to affect one or two people in a million, according to Johns Hopkins Medicine.

Desai adds that people diagnosed with stiff-person syndrome are usually between the ages of 20 and 50, although the condition has also been found in younger and older people. The disease is also “two to three times more common in women”, he said.

Interview:Celine Dion Talks Returning to Las Vegas, How Whitney Houston Inspired That ‘Surprise’ High Rating on ‘All By Myself’

How is PSS diagnosed?

Diagnosing the syndrome can be difficult, especially because the disease is so rare, note Desai and Wilson.

A comprehensive review of a patient’s medical history, neurological examination, and tests including blood work, cerebrospinal fluid analysis, and EMG can be used to confirm the diagnosis.

Especially if a patient’s symptoms have been left unexplained or misdiagnosed in the past, says Wilson, diagnosis is crucial.

“Validation is such an important thing for someone with a neurological disorder, because they can look good on some level and yet be in pain on the inside,” he said. “Once you feel the validation, we can help them.”

What causes disease?

The exact cause remains unknown. However, research suggests that PSS results from “an autoimmune response gone awry in the brain and spinal cord,” writes the NINDS.

Is PSS curable? Is it terminal?

There is no cure for SPS, notes Yale Medicine. But there are treatments to help relieve symptoms, including various medications and immunotherapies prescribed by doctors, and different types of physical therapy.

PSS can be terminal in rare cases, Desai explained.

“It’s a rare disease to begin with. And there are reported fatalities, but…it’s rare for a rare disease,” Desai said.

What is everyone talking about? Subscribe to our trendy newsletter to receive the latest news of the day

Contributor: Elise Brisco, USA TODAY.