What is Stiff Person Syndrome?

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

Singer Celine Dion announced Instagram In the video, Thursday, she is canceling some shows because she has been diagnosed.

“While we are still learning about this rare condition, we now know that it was the cause of all the cramps I was having,” Dion said.

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 that can be very severe. “This can cause a fall, severe pain and significant disability,” said Dr. Emile Sami Mukheiber. Stiff Person Syndrome Center At Johns Hopkins Medicine. “Falling from severe spasms is very common. These spasms can cause extreme agitation, stupor, and cold weather.”

These spasms can be strong enough to break bones and any fall can lead to serious injury.

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

The symptoms of the syndromes can also cause anxiety.

Scott Newsom, director of the Stiff Person Syndrome Center, said in a video on the organization’s website.

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

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

Since then, it has been found to affect twice as many women as men, and the name has been changed to Stiff Person Syndrome to avoid confusion.

Stiff person syndrome is thought to have features of an autoimmune disease, according to the National Institute of Neurological Disorders and Stroke.

Although the exact cause isn’t clear, 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 care for chronic pain before they get neurological care. On average, it takes about seven years for someone to receive a diagnosis of stiff person syndrome, Newsom says.

“sometimes, [patients] He said, “Because in the early exam, there are no hallmarks of stiff person syndrome.”

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 avoid falls or convulsions.

Pain relievers, anti-anxiety medications, and muscle relaxants can all be part of the treatment for this disease. The Stiff Person Syndrome Center also uses botulinum toxin injections.

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

But with treatment, symptoms can be controlled.

Dion, who said she struggled with her health for a while, said she had an amazing team of medical professionals and support for her children.

“I work very hard with my sports medicine therapist every day to rebuild my strength and ability to perform again,” she said. “But I have to admit it was a struggle.”

For an artist like Dion, loud noises and bright lights can be triggers for muscle spasms.

“This is a very difficult disease that may have a negative impact on her if it is not treated aggressively, in a timely and appropriate manner,” Mukheiber said.