What is Stiff Person Syndrome?Diagnosis, Symptoms, Treatment




CNN

Singer Celine Dion said in a statement posted to her social media accounts on Friday that her ‘Courage’ was scheduled until 2024 due to complications from stiff-person syndrome, a rare neurological disorder that causes spasms and muscle stiffness. World Tour has been cancelled.

Dion postponed several shows in December after learning the reasons for muscle pain and mobility difficulties. “We’re still researching this rare condition, but it turned out to be the cause of all the seizures I’ve had,” Dion said at the time.

“She’s in a lot of pain,” a source close to Dion told CNN, despite receiving physical therapy every day.

In announcing the tour cancellation on Instagram, Dion reportedly said: I’m working hard to get back in shape, but even at 100%, tours are very difficult.

“It’s unfair and heartbreaking to keep postponing shows, but until we’re really ready to get back on stage, it’s best to cancel everything now. But I haven’t given up…and I can’t wait to see you again!”

According to the National Institute of Neurological Disorders and Stroke, stiff-person syndrome is characterized by muscle stiffness and spasms, hypersensitivity to stimuli such as sound and light, and emotional distress that can lead to muscle spasms. NINDS says people with the condition may develop a “slouched posture” over time.

According to the Cleveland Clinic, the symptoms usually begin with muscle stiffness in the mid-body, trunk, and abdomen before progressing to stiffness and spasms in the legs and other muscles.

Muscle spasms can be “very severe.” These can cause falls, severe pain, and significant disability,” said Emile Sami Mookaiber, Ph.D., of the Center for Stiff Person Syndrome at Johns Hopkins University Hospital. “Severe convulsive falls are very common.

These spasms can be severe enough to break bones, and a fall can seriously injure you.

In a December Instagram video, Dion said the spasms affect “every aspect” of her daily life, adding that she “has occasional difficulty walking and can’t use her vocal cords to sing as usual. ‘ said. It pains me to say this today. ”

This syndrome can also cause anxiety.

“Many, if not all, patients have anxieties inherent in the disease, and those anxieties actually contribute to the physical ailments people can get,” says Stiff Person Syndrome. Dr. Scott Newsom, director of the center, said. Video on the organization’s website.

People with stiff-person syndrome may be afraid to leave their homes because “street noises, such as the sound of car horns, can trigger cramps and falls,” NINDS notes.

Stiff-person syndrome is extremely rare. About one in a million people have the syndrome, Mukhaiber said, and most general neurologists see only one or two cases in their lifetime.

Newsom said the first cases of stiff-man syndrome were reported in the 1950s, and the disorder was historically called “stiff-man syndrome.”

It has since been found to affect twice as many women as men, and the name was changed to stiff-person syndrome to avoid confusion.

The condition can develop at any age, but symptoms most often start in the 30s or 40s, according to the Cleveland Clinic.

According to the National Institute of Neurological Disorders and Stroke, stiff-person syndrome is thought to have features of an autoimmune disease and “is associated with other autoimmune diseases such as type I diabetes, thyroiditis, vitiligo, and pernicious anemia.” There are many things we do,” he said.

The exact cause is unknown, but research suggests it may be due to an “abnormal” autoimmune response in the brain and spinal cord, according to the institute.

“Patients with SPS have elevated levels of GAD, an antibody that acts against enzymes involved in the synthesis of key neurotransmitters in the brain,” the institute noted on its website. “The definitive diagnosis can be made with a blood test that measures levels of glutamic acid decarboxylase (GAD) antibodies.”

Other tests include electromyography (EMG), which measures the electrical activity of muscles, and lumbar puncture, commonly called spinal tap.

Because of the disease’s rarity and symptom ambiguity, people often seek treatment for chronic pain before undergoing neurological treatment. The condition can be misdiagnosed as anxiety, fibromyalgia, multiple sclerosis, Parkinson’s disease, psychosomatic disease, or even phobias, notes NINDS.

It takes an average of about seven years to receive a diagnosis of stiff-person syndrome, Newsom said.

“sometimes, [patients] “I get labeled as insane,” he said, “because early tests show no features of stiff-person syndrome.”

There is no known cure for stiff-person syndrome, but drugs can relieve symptoms. Immunoglobulin drugs help reduce sensitivity to light and sound triggers and may prevent falls and seizures.

Analgesics, anti-anxiety drugs, and muscle relaxants may be part of treatment for this disorder. The Stiff Person Syndrome Center also offers botulinum toxin injections.

The Cleveland Clinic noted that using acupuncture, physical therapy, heat therapy, and hydrotherapy may provide additional benefits.

“If left untreated, the disease can seriously disrupt daily life,” Mukaibur said.

Dion previously said she had struggled with her health for some time, but said she had a wonderful team of medical professionals and support for her children.

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

For performers like Dion, loud noises and bright lights can cause muscle spasms.

“This is a very difficult disease that can wreak havoc on her if not treated aggressively, timely and appropriately,” Dr. Mukaibur said.





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