Stiff-person syndrome: Here's what to know about the disorder affecting Celine Dion

Popular Canadian singer, Celine Dion, has revealed the reason for her long-time silence, saying she's battling a rare neurological disorder called Stiff-Person syndrome.

She said, "I've been dealing with problems with my health for a long time, and it's been really difficult for me to face these challenges and to talk about everything that I've been going through...It hurts me to tell you that I won't be ready to restart my tour in Europe in February."

Because of her viral video, viral video, we would be shedding more light on the nature of the disease.

What is stiff-person syndrome?

Stiff-person syndrome (SPS) is a rare, chronic neurological disorder that 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.

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, and although it can affect a person at any age, it is most often diagnosed among middle-aged people.

What are the symptoms?

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.

The condition typically only affects skeletal muscles that we can voluntarily control. It does not seem to affect cognition but may be associated with anxiety.

What is the life expectancy for someone with stiff-person syndrome?

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

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

Treatment

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, anti-convulsants and pain medications for those experiencing pain.