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Stiff-person syndrome (SPS) is a autoimmune and neurological disorder that causes rigidity in the torso and limbs.
Symptoms can occur at random but are often triggered by environmental stimuli (like loud noises), touch, or emotional stress.
Muscle spasms can be so severe they can dislocate joints and even break bones, in some cases causing the person to fall down. The muscles gradually relax after the stimulus is gone.
SPS occurs in about one in a million people and is most commonly found in middle-aged people.
Over time, these symptoms can lead to difficulty in walking and eventually greater immobility.
As the disease progresses, so does the emotional toll exerted on the patient. Symptoms of depression and anxiety are commonplace.
Levels of nervousness and apprehension are heightened by the fact that sufferers of SPS have lower levels of the neurotransmitter gamma-aminobutyric acid (GABA), which regulates anxiety.
The immune system appears to attack a protein called glutamic acid decarboxylase (GAD), which helps make the aforementioned substance called GABA.
The cause of SPS remains clouded in mystery. Research suggests that its onset may be caused by an autoimmune reaction.
GABA helps regulate motor neurons by decreasing their activity. But lower than normal levels of GABA can cause those neurons to fire continuously even when they’re not supposed to. This impairment probably causes the stiffness and spasms that characterize SPS.
SPS patients commonly have another autoimmune disorder, typically type 1 diabetes, vitiligo, or pernicious anemia.
Those stricken with SPS also tend to be cancer patients, specifically those suffering from breast cancer, lung cancer, kidney cancer, thyroid cancer, colon cancer, and lymphomas. The reason for this link is unknown.
Stiff-person syndrome was first described in 1956 by neurologists Moersch and Woltman (the disease is sometimes referred to as Moersch-Woltman syndrome). However, it was only in 1989 that criteria for an SPS diagnosis were adopted.
As the condition worsens, the shoulders, neck, and hips may also be affected.
Progression of SPS is characterized by stiffness of the leg muscles and a slow, rigid manner of walking.
As stiffness increases, affected individuals may develop a hunched or slouched posture due to outward curving of the upper spine, a condition known as kyphosis. SPS can also cause hyperlordosis, where there is an excessive spine curvature in the lower back.
The severity of advanced SPS can impact an individual's ability to perform daily activities and routines. Some patients may end up requiring the use of a wheelchair.
There is no cure for stiff-person syndrome. A diagnosis of SPS is made based upon identification of characteristic symptoms, a detailed patient history, and a thorough clinical evaluation.
Treatment for the condition typically involves relieving symptoms with medications such as sedatives, muscle relaxants, and steroids.
In some cases, immunotherapy may be prescribed, procedures such as intravenous immunoglobulin and plasmapheresis.
When treating SPS, emphasis is also placed on regular activity. Physical, occupational, and aqua therapy are vital for patients with SPS.
Diagnosis of SPS can be tricky. Symptoms can mimic those of Parkinson’s disease, multiple sclerosis, fibromyalgia, or anxiety, and it often takes time to identify the exact cause.
By its nature, SPS is difficult to treat, more so than other autoimmune disorders like rheumatoid arthritis or Crohn's disease.
Symptoms of SPS and their severity levels vary. It's difficult therefore to predict whether a patient's condition will get worse over time.
Early recognition and neurological treatment of SPS can limit its progression. But even with rapid intervention, quality of life generally declines as stiffness precludes many activities. Ultimately, about 65% of SPS patients are unable to function independently. About 10% of those will require intensive care at some point.
Sources: (National Organization for Rare Disorders) (NBC News) (The Stiff Person Syndrome Research Foundation)
See also: Stars who suffer from rare diseases
People with the syndrome can expect chronic pain, impaired mobility, and lumbar hyperlordosis (excessive spine curvature in the lower back).
Stiff-person syndrome inhibits the normal pathways of communication between the brain and the muscles.
Generally, SPS begins slowly over several months or a few years. Affected individuals may initially experience aching discomfort, stiffness, or pain, especially in the lower back or legs.
Besides stiffening across the body, symptoms of this rare condition also include episodes of severe muscle spasms.
Inevitably, sufferers of SPS will develop muscle spasms. The legs are often involved. But spasms can also involve the muscles of the chest and respiratory system, potentially requiring emergency medical treatment with ventilatory support.
In December 2023, one year after Dion went public with her diagnosis, her sister Claudette (pictured) gave a worrying update on the singer's condition in an interview with Canada’s 7 Jours. She revealed that Dion had “lost control of her muscles" as the symptoms worsen. However, she was still determined to return to the stage. “In what capacity? I don’t know," said Claudette, given the seriousness of the illness.
The singer revealed her diagnosis in December 2022, when she announced that she would have to postpone several shows on her Courage World Tour. On May 26, 2023, Dion announced that she was canceling the remaining 42 dates of the tour. “I’m so sorry to disappoint all of you once again,” she said with regret. Dion shared that stiff-person syndrome was affecting both her mobility and her ability to sing. “The 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,” she wrote on Instagram.
A couple of months later, Dion did an interview with Vogue, opening up about the details of her condition at length for the first time. She revealed that she trains like an athlete daily to recover her body: "I work on my toes as well as my knees, calves, fingers, singing, voice…" She also stated that she won't give up until she's back on the stage performing for her fans, although she doesn't know when that will be.
However, Dion made a few surprise appearances in early 2024, showing the world that she's still here and still fighting. She delighted fans with an unexpected appearance on stage at the Grammys in February, and was spotted attending a football game with her sons in March.
After having made a triumphant return to perform as a final act at the opening ceremony of the Paris Olympics this summer, Celine Dion once again managed to vow an audience, stealing the show as she sang at the 1001 Seasons of Elie Saab event in Riyadh, Saudi Arabia. For the past two years, she has been struggling with stiff person syndrome, which she opened up about in the ‘I Am: Celine Dion’ documentary that was released earlier this year.
Dion sang two songs: 'The Power of Love,' a cover of the 1984 song by Jennifer Rush, and an Arabic remix of 'I’m Alive,' a popular single from her album 'A New Day Has Come.' According to some news sources, even the biggest celebrities in the room lost themselves in the moment. Once again, she has proven to the world her exceptional talent, delivering a performance that was truly one-of-a-kind.
Celine Dion’s battle with her illness was not an easy one. To learn more about her journey and the disease she suffers from, click through this gallery.
Celine Dion delivers unforgettable performance in Saudi Arabia
Many attendees were moved by her incredible energy
HEALTH Sps
After having made a triumphant return to perform as a final act at the opening ceremony of the Paris Olympics this summer, Celine Dion once again managed to vow an audience, stealing the show as she sang at the 1001 Seasons of Elie Saab event in Riyadh, Saudi Arabia. For the past two years, she has been struggling with stiff person syndrome, which she opened up about in the ‘I Am: Celine Dion’ documentary that was released earlier this year.
Dion sang two songs: 'The Power of Love,' a cover of the 1984 song by Jennifer Rush, and an Arabic remix of 'I’m Alive,' a popular single from her album 'A New Day Has Come.' According to some news sources, even the biggest celebrities in the room lost themselves in the moment. Once again, she has proven to the world her exceptional talent, delivering a performance that was truly one-of-a-kind.
Celine Dion’s battle with her illness was not an easy one. To learn more about her journey and the disease she suffers from, click through this gallery.