Stiff person syndrome explained: What is it, symptoms, causes and treatment

Due to stiff-person syndrome, Dion gets muscle spasms and after knowing about her reason for her muscle pain and difficulties with mobility, the renowned Canadian singer postponed several performances in December, reported CNN.

“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,” CNN had quoted Dion as saying at the time.

Though undergoing daily physical therapy, ‘she is in a lot of pain’.

ALSO READ: Celine Dion cancels European stretch of her world tour, cites medical condition

Citing the reason, Dion to Instagram and announced the tour’s cancellation, saying, “I’m so sorry to disappoint all of you once again. I’m working really hard to build back my strength, but touring can be very difficult even when you’re 100%.

“It’s not fair to you to keep postponing the shows, and even though it breaks my heart, it’s best that we cancel everything now until I’m really ready to be back on stage again. I want you all to know, I’m not giving up… and I can’t wait to see you again!”

Stiff person syndrome, what is it?

According to the National Institute of Neurological Disorders and Stroke, SPS is characterised by muscle rigidity and spasms, heightened sensitivity to stimuli such as sound and lights, and emotional distress that can cause muscle spasms.

It begins with muscle stiffness in the middle part of the body, then covers trunk and abdomen, before advancing to stiffness and spasms in the legs and other muscles, says the Cleveland Clinic.

Experts say, the muscle spasms can be ‘quite severe, can cause falls, severe pain and significant disability’. Dr Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine, said, “Falls from severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather.”

“These spasms can be strong enough to fracture bone, and any fall can lead to severe injury.”

While Dr. Scott Newsome, director of the Stiff Person Syndrome Center said the syndrome can also cause anxiety. “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.”

Who is at risk of developing SPS?

According to Dr Moukheiber about 1 out of every 1 million people have the syndrome. First reported in 1950s, as per Newsome, it was historically referred to as ‘stiff man syndrome’.

Cleveland Clinic claims the SPS can develop at any age, but symptoms most often begin in a person’s 30s or 40s.

What causes SPS?

The National Institute of Neurological Disorders and Stroke say that SPS is thought to have features of an autoimmune disease. “It is frequently associated with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo, and pernicious anemia.”

Though the exact cause of the syndrome is yet to be ascertained, research says it may be due to an autoimmune response “gone awry” in the brain and spinal cord.

“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,” the institute noted on its website. “A definitive diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies.”

Treatment for SPS?

To be straight, till now there is no known cure for stiff person syndrome, but medications can ease the symptoms. Also, immunoglobulin medications can help lower sensitivity to light or sound triggers, potentially helping head off falls or spasms, reported CNN.

Meanwhile, the Stiff Person Syndrome Center also uses botulinum toxin injections.

Updated: 27 May 2023, 08:05 PM IST