(CNN) — Stiff Person Syndrome (SPS) is a rare neurological condition that can take years to diagnose and tailor treatment.
Singer Celine Dion announced in December 2022 in a video of instagram the cancellation of some performances because he was diagnosed with this condition. Months later, in May 2023, the singer completely canceled her “Courage World Tour” due to health complications.
“”I’m so sorry to disappoint you all once again,” reads an announcement on the singer’s social media. “I am working very hard to get my strength back, but touring can be very difficult even when you are 100%,” Dion said in canceling her 2023-2024 tour.
What is stiff person syndrome?
Stiff person syndrome is characterized by muscle stiffness and spasms, increased sensitivity to stimuli such as sound and lights, and emotional distress that can cause muscle spasms, according to the National Institute of Neurological Disorders and Stroke.
“It is often associated with muscle spasms, which can be quite severe. These can cause falls, severe pain and significant disability,” said Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine. “Falls due to severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather.
These spasms can be strong enough to fracture a bone, and any fall can cause serious injury.
Dion said in a video that the spasms affect “every aspect” of her daily life, “sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing in the way I’m used to.”
The symptoms of the syndrome can also cause anxiety.
“Many, if not all, patients have anxiety that is intrinsic to the disease, and that anxiety is actually fueled by the physical ailments of the disease that people may have,” Dr. Scott Newsome said in a statement. director of the Stiff Person Syndrome Center in a video on the organization’s website.
Who is affected by stiff person syndrome?
The condition affects around a million people and most general neurologists will see only one or two cases in their lifetime, Moukheiber said.
The first case of stiff person syndrome was reported in the 1950s, according to Newsome, and the condition was historically called “stiff man syndrome.”
Since then, it has been found to affect twice as many women as men, and the name was changed to Stiff Person Syndrome to avoid confusion.
What Causes Stiff Person Syndrome?
Stiff person syndrome is thought to have features of an autoimmune disease, according to the National Institute of Neurological Disorders and Cerebrovascular Accidents.
Although the exact cause is unclear, according to the institute, “People with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain.”
Due to the rarity of the disease and the ambiguity of its symptoms, people often seek care for chronic pain before receiving neurological care. On average, it takes about seven years for someone to be diagnosed with stiff person syndrome, Newsome says.
“Sometimes, [los pacientes] they’re labeled crazy,” he said, “because on initial examination, there aren’t the hallmarks of stiff person syndrome.”
How is stiff person syndrome treated?
There is no known cure for stiff person syndrome, but medications can relieve symptoms. Immunoglobulin medicines can help reduce sensitivity to light or sound triggers, which could help prevent falls or spasms.
Analgesics, anxiolytics and muscle relaxants can be part of the treatment of this disease. The Stiff Person Syndrome Center also uses botulinum toxin injections.
“If left untreated, the disease can cause serious impairment to daily life,” Moukheiber said.
But with treatment, the symptoms can be controlled.
Dion, who said she has been struggling with her health for a while, said she has a great team of medical professionals and the support of her children.
“I am working hard with my sports medicine therapist every day to get my strength back and my ability to perform again,” he said. “But I have to admit, it’s been a struggle.”
For an artist like Dion, loud noises and bright lights can trigger muscle spasms.
“This is a very challenging disease that could affect her if not treated aggressively, promptly and appropriately,” Moukheiber said.
Editor’s Note: This article was first published in December 2022 and updated in May 2023.
Source: CNN Espanol