Celine Dion has seizure in new documentary. What is stiff-person ...

Dion, now 56, announced in 2022 that she was diagnosed with stiff-person syndrome, a rare, chronic neurological disorder that causes muscle stiffness and sometimes intense spasms. She has discussed her health challenges in the past, and the documentary’s director, Irene Taylor, said Dion had asked that the footage of her seizure be kept in. Here’s what to know about the disorder.

Stiff-person syndrome (SPS) causes muscle stiffness and sometimes intense muscle spasms in the trunk and limbs, affecting posture, balance and the ability to use certain muscles. It usually has an autoimmune component and, in some cases, it can be progressive and painful, experts say.

Andrew McKeon, professor of neurology at the Mayo Clinic, said SPS affects nerves in the spinal cord and neurons in the brain that regulate movement. In other words, when the nervous system becomes overly excited, it can send too many signals to the muscles, causing them to stiffen or spasm.

A person’s “whole body can seize up when startled or in other situations,” putting them at risk for falls and injuries, he said.

The syndrome affects women at twice the rate of men, experts say. The condition can affect a person at any age, though it is most often diagnosed among middle-aged people.

SPS causes muscle stiffness, muscle aches and muscle spasms, often in the lower back and legs, which can make it difficult for some patients to walk. Those who have symptoms that are not well-controlled may need to use a walker or wheelchair to keep from falling or injuring themselves.

The muscle spasms are what neurologists call “stimulus sensitive” and can be provoked by a sudden noise, light touch or even emotional distress. One form of the condition can affect muscles that control the eyes, speech or singing or swallowing.

Chi-Ying “Roy” Lin, a neurology professor specializing in movement disorders at Baylor College of Medicine, said in the cases he has seen, patients “were very, extremely uncomfortable, and it’s usually very painful.”

“And when the pain occurs it’s very debilitating no matter what position,” he added. “There’s basically no comfortable position for them to stay, either sitting or laying.”

The condition typically only affects skeletal muscles that we can control, and not smooth muscles or those found in the heart. It does not seem to affect cognition, but may be associated with anxiety.

Months after revealing her diagnosis, Dion canceled dozens of show dates on her Courage world tour due to the illness, telling fans that she was “so sorry … I’m working really hard to build back my strength, but touring can be very difficult even when you’re 100%.” She added that she was “not giving up.”

While discussing in the documentary how the seizures make her feel, Dion covered her face and said: “Every time something like this happens it makes you feel so embarrassed.”

“You don’t like to not have control of yourself, you know?” she added.

Writing on social media earlier this year about her decision to take part in the documentary, Dion said she “wanted to document this part of my life, to try to raise awareness of this little-known condition, to help others who share this diagnosis.”

In an interview with Variety, Irene Taylor, the documentary’s director, said Dion told her not to remove the dramatic scene: “She said, ‘It’s OK. I told you I don’t want you to cut it. That’s what I go through. That’s what this feels like.’”

In a recent interview with NBC’s “Today Show,” Dion said she once suffered a spasm so “severe” it gave her broken ribs. She called her experience with the illness “very difficult, very painful, challenging” and “scary.”

SPS is thought to be an autoimmune disorder where the immune system is overstimulated and generates antibodies that target neurons that inhibit activity in the brain. There is no clear mechanism for why these antibodies are generated, Lin said.

Although it’s a rare disease, when it is diagnosed, it’s common to see it alongside other conditions, including diabetes, thyroid disorders and pernicious anemia.

“The world will benefit from having more understanding about this disease because it’s just so rare,” Lin said.

Although SPS is rare and not completely understood, experts say the syndrome does not typically have an effect on longevity, except in very rare circumstances in which muscles used for breathing or swallowing are compromised.

When symptoms are well-controlled, patients can live a relatively normal life. However, when the symptoms are not able to be managed, they can suffer significant impairments, experts say.

Lin said the main effect is on quality of life. “I don’t think for the cases I saw, their life quality ever comes back to like normal baseline,” Lin said.

SPS is diagnosed based on symptoms, through neurological and neuromuscular exams, including using electromyography, or EMG, to test muscle and nerve function. Diagnosis also often relies on a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies. Most people with SPS have higher levels of GAD antibodies, the National Institutes of Health says.

Because the symptoms can share similarities with multiple sclerosis and various muscular dystrophies, it “can be a challenging diagnosis to make, and it does require some expertise,” McKeon said.

SPS is not curable, but it can be managed, experts say.

Treatment is aimed at targeting the nervous system directly to restore balance, as well as targeting the immune system to stop it from attacking the nervous system, McKeon said.

For patients who have an autoimmune cause, treatment may include intravenous immune globulin (IVIG), a treatment that uses antibodies to blunt the immune response. Other treatments involve using muscle relaxants, anticonvulsants and pain medications for those experiencing pain.

In addition, Pavan Tankha, medical director of Comprehensive Pain Recovery at the Cleveland Clinic, said he refers patients to pain psychologists, physical therapists and other health professionals “to try to improve their overall quality of life and decrease the suffering associated with pain.”

“Pain simply isn’t what you feel; it’s also what it does to you,” he said.