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What is Celine Dion's Stiff Person Syndrome?

What is Celine Dion's Stiff Person Syndrome?

(CNN) — Celine Dion shared in 2022 that she had been diagnosed with Stiff Person Syndrome (SPS), a rare neurological condition that can take years to diagnose.

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 very severe. These conditions can cause falls, severe pain and significant disability,” said Dr. Emile Sami Moukheiber from the center. Stiff Person Syndrome Center At Johns Hopkins Medicine. “Falls due to severe convulsions are very common. These convulsions can be triggered by stupor, intense emotions, and cold weather.

These spasms can be strong enough to break bones, and any fall can result in serious injuries.

Dionne said in a video that the spasms affect “every aspect” of her daily life, “sometimes causing difficulty when I walk and not allowing me to use my vocal cords to sing the way I used to.”

Symptoms of the syndrome can also cause anxiety.

“Many, if not all, patients experience anxiety inherent in the disease, and this anxiety actually feeds into the physical pathology of the disease that people may be experiencing,” Dr. Scott Newsom, director of the Center for Stiff Person Syndrome, said in a statement. In a video clip on the organization's website.

Who is affected by stiff person syndrome?

Mokheiber said that this condition affects about one million people, and most general neurologists will only see one or two cases in their lifetime.

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The first case of stiff man syndrome was reported in the 1950s, according to Newsom, and the disease was historically called “stiff man syndrome.”

It has since 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 characteristics of an autoimmune disease, according to 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 works 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 chronic pain care before receiving neurological care. On average, it takes about seven years for someone to be diagnosed with stiff person syndrome, Newsom says.

“sometimes, [los pacientes] “They are classified as crazy because on initial examination, there are no distinguishing features of stiff person syndrome,” he said.

How is stiff person syndrome treated?

There is no known cure for stiff person syndrome, but medications can relieve symptoms. Immunoglobulin medications can help reduce sensitivity to light or sound stimuli, which may help prevent falls or convulsions.

Analgesics, anxiolytics, and muscle relaxants can be part of the treatment for this disease. The Stiff Person Syndrome Center also uses botulinum toxin injections.

“If the disease is left untreated, it can cause serious impairment in daily life,” Mokheiber said.

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But with treatment, symptoms can be controlled.

Dion, who said he has been struggling with his health for a while, said he has a great team of medical professionals and supports his children.

“I work hard with my sports medicine therapist every day to regain my strength and 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.

Mokheiber said: “This is a very difficult disease and it can affect you if it is not treated aggressively, timely and appropriately.”

Editor's Note: This article was first published in December 2022 and was updated in March 2024