Current:Home > ScamsCeline Dion is battling stiff person syndrome, a rare neurological disorder. What is it? -VisionFunds
Celine Dion is battling stiff person syndrome, a rare neurological disorder. What is it?
View
Date:2025-04-12 12:47:00
Celine Dion received a standing ovation at Sunday's Grammy Awards when she made a surprise appearance on stage to present the award for album of the year, which went to Taylor Swift.
"Those who have been blessed enough to be here, the Grammy Awards, must never take for granted the tremendous love and joy that music bring to our lives and to people all around the world," Dion told the supportive crowd.
The 55-year-old music powerhouse, who has five Grammys of her own, is battling stiff person syndrome, 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.
Here's what you need to know about stiff person syndrome, the symptoms of the disease and more.
What is stiff person syndrome?
Stiff person syndrome, or SPS, is a rare "neurological disorder with features of an autoimmune disease," the National Institute of Neurological Disorders and Stroke notes.
"It's a disease that's characterized by progressive muscle stiffness, muscle spasms, rigidity – typically in the muscles of the back, specifically the lower back, as well as the upper legs," Dr. Kunal Desai, a Yale Medicine neurologist and assistant professor of neurology who specializes in neuromuscular disease, told USA TODAY.
"It can affect other muscles, including muscles in the arms as well as speaking and swallowing muscles," Desai said. "But that's less common."
With extreme muscle spasms, "the muscles can feel tight as a rock. So, it can be very, very uncomfortable and painful," Desai said, adding that other stimuli that can provoke these spasms sometimes include touching the muscles, "certain subtle movements" and stress.
What are the symptoms of SPS?
Prominent SPS symptoms include muscle stiffening and painful muscle spasms that can be provoked by emotional stress or, again, outside stimuli. It's possible for these spasms to be "so severe that they cause the person to fall down," Yale Medicine says.
"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 and the director of the Autonomic Center at the Cleveland Clinic, told USA TODAY, expanding on some severe cases he's seen. "The limb can look distorted and contorted. I've seen people actually break a limb from it."
These symptoms can lead to difficulty walking, among other disabilities. People with SPS are also more likely to experience anxiety and depression, Yale Medicine notes – pointing to how unpredictable the disease can be and because patients "have lower levels of the neurotransmitter GABA, which regulates anxiety."
NINDS adds that many people with SPS "are afraid to leave the house" due to the environmental stimuli, such as loud noises, that can trigger episodes.
Are the symptoms same for each person?
Of course, SPS symptoms can range significantly and every patient's experience is unique. "It's a spectrum of severity," Desai says.
"Some people have mild spasms. Some people can have the full dystonia where a part of the body becomes almost like rigid to a board or like a statue. ... And some people can be constant, with 24/7 locking, or some people could just (have) episodes," Wilson said. "There's a variability within each patient."
In rare cases, both Desai and Wilson note, stiff person syndrome has also been associated with cancer.
How rare is SPS?
SPS is considered to be very rare because the disease is expected to affect one or two people per million, according to Johns Hopkins Medicine.
Desai adds that people who are diagnosed with stiff person Syndrome are usually in their 20s to 50s, although the disease has been found in people who are younger and older as well. The disease is also "two to three times more common in women," he said.
How is SPS diagnosed?
Diagnosing the syndrome can be difficult, especially because the disease is so rare, Desai and Wilson note.
A comprehensive review of a patient's medical history, a neurological exam and tests including blood tests, spinal fluid analysis and an EMG can be used to confirm the diagnosis.
Especially if a patient's symptoms were left unexplained or misdiagnosed in the past, Wilson says, diagnosis is crucial.
"Validation is such an important thing for a person with a neurologic disorder, because they can look good at some level and yet still suffer internally," he said. "Once you feel the validation, we can help them out."
What causes the disease?
The exact cause remains unknown. However, research suggests that SPS results from "an autoimmune response gone awry in the brain and spinal cord," NINDS writes.
Is SPS curable? Is it terminal?
There is no cure for SPS, Yale Medicine notes. But there are treatments to help relieve symptoms – including various medications and immunotherapies prescribed by doctors and different types of physical therapy.
SPS can be terminal in rare cases, Desai explained.
"It's a rare disease to begin with. And there are cases of death being reported, but ... it's rare for a rare disease," Desai said.
veryGood! (1)
Related
- Kylie Jenner Shows Off Sweet Notes From Nieces Dream Kardashian & Chicago West
- Some Seattle cancer center patients are receiving threatening emails after last month’s data breach
- Where the Republican presidential candidates stand on Israel and Ukraine funding
- Hong Kong holds first council elections under new rules that shut out pro-democracy candidates
- Buckingham Palace staff under investigation for 'bar brawl'
- 'Wait Wait' for December 9, 2023: With Not My Job guest Fred Schneider
- We Ranked All of Meg Ryan's Rom-Coms and We'll Still Have What She's Having
- Third victim ID'd in UNLV shooting as college professors decry 'national menace'
- Federal appeals court upholds $14.25 million fine against Exxon for pollution in Texas
- Greyhound bus service returns to Mississippi’s capital city
Ranking
- B.A. Parker is learning the banjo
- How Felicity Huffman Is Rebuilding Her Life After the College Admissions Scandal
- Eagles security guard DiSandro banned from sideline for Sunday Night Football vs. Cowboys
- A gigantic new ICBM will take US nuclear missiles out of the Cold War-era but add 21st-century risks
- Could your smelly farts help science?
- Zimbabwe holds special elections after court rules to remove 9 opposition lawmakers from Parliament
- How Kyle Richards, Teresa Giudice and More Bravo Stars Are Celebrating the 2023 Holidays
- Brazil’s Lula takes heat on oil plans at UN climate talks, a turnaround after hero status last year
Recommendation
Warm inflation data keep S&P 500, Dow, Nasdaq under wraps before Fed meeting next week
Regulators’ recommendation would mean 3% lower electric rates for New Mexico residential customers
Coco Austin Reveals How She Helped Her and Ice-T's Daughter Chanel Deal With a School Bully
Why Shohei Ohtani will be worth every penny of $700 million contract for Los Angeles Dodgers
Travis Hunter, the 2
At UN climate talks, cameras are everywhere. Many belong to Emirati company with a murky history
Bangladesh opposition party holds protest as it boycotts Jan. 7 national election amid violence
Teen gunman sentenced to life for Oxford High School massacre in Michigan