(CNN) — Celine Dion shared in 2022 that she was diagnosed with Difficult Person Syndrome (SPS), a rare neurological condition that can take years to diagnose.
Rigid 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's often associated with muscle spasms, which can be very severe. These can cause falls, severe pain and significant disability,” said Dr. Emily Sammy Maukeiber. Center for Difficult Person Syndrome In Johns Hopkins Medicine. “Falls due to severe cramps are very common. These cramps can be triggered by startling, strong emotions, cold weather.
These grips can be strong enough to break a bone, and any fall can lead to serious injuries.
Dion said in a video that the spasms affect “every aspect” of her daily life, “sometimes making it difficult for me to walk and not allowing me to use my vocal cords to sing the way I'm used to.”
Symptoms of the syndrome can also cause anxiety.
“Most, if not all, patients have an inherent anxiety about the disease, and that anxiety actually feeds into the physical toll the disease can have on people,” Dr. Scott Newsom said in a statement. Director of the Stiff Person Syndrome Center. In a video on the organization's website.
Who Suffers from Difficult Person Syndrome?
The condition affects about a million people and most general neurologists will see only one or two cases in their lifetime, Mokhaibar said.
According to Newsom, the first case of Stiff-Man syndrome was reported in the 1950s, and the disease was historically called “Stiff-Man syndrome.”
It affects twice as many women as men and has been renamed Stiff Person Syndrome to avoid confusion.
What Causes Difficult Person Syndrome?
Acute Person syndrome is thought to have characteristics of an autoimmune disease National Institute of Neurological Disorders and Stroke.
Although the exact cause is unclear, according to the institute, “People with SPS have elevated levels of GAD, an antibody that acts against an enzyme involved in the synthesis of an important neurotransmitter in the brain.”
Because of the rarity of the disease and the vagueness of its symptoms, people often seek care for chronic pain before seeking neurological care. On average, it takes about seven years for someone to be diagnosed with difficult-person syndrome, Newsom says.
“some times, [los pacientes] They are labeled as crazy,” he said, adding, “On initial examination, there are no distinctive features of difficult person syndrome.”
How is difficult person syndrome treated?
There is no known cure for difficult person syndrome, but medications can relieve symptoms. Immunoglobulin medications can help reduce sensitivity to light or sound stimuli, which can help prevent falls or seizures.
Analgesics, anxiolytics, and muscle relaxants may be part of the treatment for this condition. The Stiff Person Syndrome Center also uses botulinum toxin injections.
“If left untreated, this disease can have a serious impact on daily life,” Maukeiber said.
But with treatment, symptoms can be controlled.
Dion, who said she has struggled with her health for some time, said she has a great team of medical professionals and the support of her children.
“I work hard every day with my sports medicine therapist to regain my strength and ability to function again,” he said. “But I have to admit it was a struggle.”
For an artist like Dion, loud noises and bright lights can trigger muscle spasms.
“It's a very challenging disease that, if not treated aggressively, timely and appropriately, can take a toll on you,” Moukheiber said.
Editor's note: This article was originally published in December 2022 and was updated in March 2024
