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Explainer: What is Stiff-Person Syndrome which singer Celine Dion has been diagnosed with?

NEW YORK — Pop superstar Celine Dion announced Thursday (Dec 8) that she is canceling and rescheduling her planned 2023 tour dates after being diagnosed with stiff-person syndrome, a rare neurological condition.

Canada's Celine Dion performs on stage at AccorHotels Arena concert hall in Paris on June 24, 2016.

Canada's Celine Dion performs on stage at AccorHotels Arena concert hall in Paris on June 24, 2016.

NEW YORK — Pop superstar Celine Dion announced Thursday (Dec 8) that she is canceling and rescheduling her planned 2023 tour dates after being diagnosed with stiff-person syndrome, a rare neurological condition.

The syndrome, which causes progressive stiffness in the body and severe muscle spasms, is “exquisitely rare” and affects perhaps one in a million people, according to Dr Pavan Tankha, the medical director of comprehensive pain recovery at Cleveland Clinic.

Here’s what to know about the diagnosis, symptoms and treatment of the condition.

WHAT IS STIFF-PERSON SYNDROME?

Stiff-person syndrome is a rare autoimmune neurological condition that affects the central nervous system and can cause rigidity throughout the body and painful muscle spasms. It was first coined in the 1920s (as “stiff man syndrome”) after doctors described patients falling over like “a wooden man.”

The exact cause of the condition is not clear, but “the immune system is involved,” said Dr Scott Newsome, the director of the Stiff Person Syndrome Center at Johns Hopkins Medicine. The syndrome is difficult to diagnose, Dr Newsome said, and may be underrecognized.

The syndrome itself is not deadly, but it can significantly affect a patient’s quality of life. And like many chronic conditions, the associated complications can lead to shortened life expectancy.

WHO IS AT RISK OF STIFF-PERSON SYNDROME?

Anyone at any age can get the condition, said Dr Richard Nowak, an assistant professor of neurology at Yale School of Medicine, but it’s most typical for people in middle adulthood, between the ages of 30 and 60, and can be associated with highly stressful events. Like many other immune conditions, it’s more common in women than men, Dr Newsome said.

Certain autoimmune conditions and cancers have been tied to a slight increase in risk for developing the syndrome, Dr Tankha said, including diabetes, thyroiditis, vitiligo, breast cancer, thyroid cancer, lung cancer and colon cancer. However, the risk remains low.

WHAT ARE THE SYMPTOMS OF STIFF-PERSON SYNDROME?

Stiff-person syndrome often begins with stiffness in the torso and abdomen, which can then spread to the legs, arms and face, Dr Tankha said.

At first, this decrease in mobility may be infrequent, but over time it can become constant, cause people to walk slightly hunched over or lose the ability to walk altogether. People also can experience painful muscle spasms or continuous aching.

The duration of the spasms can be anywhere from seconds to hours, Dr Tankha said, and they can be so severe that they break bones or cause patients to fall down.

The muscle spasms can be triggered by a variety of environmental factors, such as loud noises, cold temperatures and emotional stress, Dr Newsome said.

The presence of chronic pain can also lead some patients to develop anxiety, depression and phobias of going outside or trying new activities.

Symptoms and their severity vary person to person, according to Dr Newsome. “It’s an individual disease,” he said.

HOW IS STIFF-PERSON SYNDROME TREATED?

There is no cure for stiff-person syndrome, so doctors focus on symptom and pain management. The treatment for the condition is often a patchwork of medication and non-medication interventions, Dr Newsome said.

The rigidity and spasming can be treated with muscle relaxers and Botox injections. More severe symptoms are treated with immunotherapy and immunosuppressants, Dr Newsome said.

Tapering down the body’s immune response can help alleviate symptoms, said Dr Senda Ajroud-Driss, an associate professor of neurology at Northwestern Feinberg School of Medicine.

For the non-pharmacological treatments, patients may undergo cognitive behavioural therapy and other psychotherapy to help them mitigate the emotional triggers that prompt muscle spasms, as well as to develop skills for living with a chronic condition.

Other types of treatments, such as physical therapy, aqua therapy, heat therapy and acupuncture can provide relief for some patients.

HOW IS STIFF-PERSON SYNDROME DIAGNOSED?

Diagnosing the condition takes a combination of tools, Dr Nowak said. Because of how rare it is, stiff-person syndrome is typically diagnosed by first ruling out other, more common conditions.

Healthcare providers will then perform a physical and neurological exam, do blood work, evaluate the stiffness of muscles, take a magnetic resonance imaging (MRI) scan and other imaging tests and administer a spinal tap.

It’s important to remember that while there are common symptoms that can be associated with stiff person syndrome, the condition is very rare, Dr Nowak said.

“Every ache and pain is not necessarily stiff person,” he said.

This article originally appeared in The New York Times.

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Health Stiff-Person Syndrome

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