What Is Stiff Person Syndrome? Diagnosis, Symptoms And Treatment

What Is Stiff Person Syndrome? Diagnosis, Symptoms And Treatment

Stiff person syndrome is a rare neurological condition that can take years to diagnose and treat.

Celine Dion announced in an Instagram video on Thursday that she is canceling some performances due to the condition.

“While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having,” Dion said.

Read Also Céline Dion Battles Rare Incurable Disease (VIDEO)

What is stiff person syndrome?

According to the National Institute of Neurological Disorders and Stroke, stiff person syndrome is characterized by muscle rigidity and spasms, increased sensitivity to stimuli such as sound and light, and emotional distress that can cause muscle spasms.

“It frequently is associated with muscle spasms, which could be quite severe. These can cause falls, severe pain and significant disability,” said Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine. “Falls from severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather.”

These spasms can be powerful enough to fracture bones, and any fall can result in serious injury.

In her video, Dion stated that her spasms affect “every aspect” of her daily life, including “sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to.”

Anxiety can be caused by the syndrome’s symptoms.

“Many patients, if not all, have an anxiety that is intrinsic to the disease and that anxiety actually feeds on the physical ailments of the disease that people can have,” Dr. Scott Newsome, director of the Stiff Person Syndrome Center, said in a video on the organization’s website.

Who is affected by stiff person syndrome?

According to Moukheiber, the condition affects approximately one million people, and most general neurologists will only see one or two cases in their lifetimes.

According to Newsome, the first case of stiff person syndrome was reported in the 1950s, and the disease was previously known as “stiff man syndrome.”

To avoid confusion, the name was changed to stiff person syndrome after it was discovered to affect twice as many women as men.

What causes stiff person syndrome?

According to the National Institute of Neurological Disorders and Stroke, stiff person syndrome is thought to be an autoimmune disease.

Although the exact cause is unknown, “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,” according to the institute.

Because of the disease’s rarity and the ambiguity of its symptoms, people frequently seek care for chronic pain before seeking neurological care. According to Newsome, it takes an average of seven years for someone to be diagnosed with stiff person syndrome.

“Sometimes, [patients] get labeled crazy,” he said, “because on exam early on, there aren’t the hallmark features of stiff person syndrome.”

How is stiff person syndrome treated?

Although there is no known cure for stiff person syndrome, medications can help to alleviate the symptoms. Immunoglobulin medications can help reduce sensitivity to light or sound triggers, possibly preventing falls or spasms.

This disease can be treated with pain relievers, anti-anxiety medications, and muscle relaxants. Botulinum toxin injections are also used at the Stiff Person Syndrome Center.

“If left untreated, the disease can cause severe impairment of daily living,” Moukheiber said.

However, symptoms can be managed with treatment.

Dion, who has struggled with her health for some time, says she has a great team of medical professionals and the support of her children.

“I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again,” she said. “But I have to admit it’s been a struggle.”

Loud noises and bright lights could cause muscle spasms in a performer like Celine Dion.

“This is a very challenging illness that might take a toll on her if it is not treated aggressively, timely and appropriately,” Moukheiber said.

Credit: CNN

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