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What is Stiff-person Syndrome, the incurable nerve condition iconic singer Celine Dion is suffering from?

What is Stiffperson Syndrome the incurable nerve condition iconic singer Celine Dion is suffering from
Celine Dion dropped a shocker on Thursday morning and left her fans stressed and emotional. The iconic singer, who crooned hit tracks like 'My Heart Will Go On', 'The Power Of Love' and 'I'm Alive', revealed in an emotional video that she's been diagnosed

Celine Dion dropped a shocker on Thursday morning and left her fans stressed and emotional. The iconic singer, who crooned hit tracks like 'My Heart Will Go On', 'The Power Of Love' and 'I'm Alive', revealed in an emotional video that she's been diagnosed with Stiff-person Syndrome, due to which she has cancelled and rescheduled some of her upcoming concert dates.

"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," the teary-eyed singer shared.

Like many of her fans, you might also not be aware of the rare and incurable neurological disease Celine spoke about. Here's a quick health guide to help you understand the condition better!

What exactly is Stiff-person Syndrome?

Stiff-person Syndrome, also known as SPS and Moersch-Woltman syndrome, is a rare autoimmune movement disorder, which usually occurs in about one in every one million people, and it affects their central nervous system. It is mainly diagnosed among middle-aged people like the 54-year-old singer.

The condition causes chronic pain and spasms, usually triggered by noise, emotional distress and light physical touch. The person dealing with this disorder also experiences stiffening of the muscles near the trunk and abdomen area, followed by the development of rigidity and stiffness around the legs and other muscles of the body.

Over time, the condition leads to an altered posture, and in severe cases, it can even limit your ability to walk or move. People suffering from this rare disorder need ongoing treatment to manage symptoms and maintain quality of life.

Stiff person syndrome is thought to be part of a wide range of similar diseases that involve one area of the body and then spread throughout the body.

Who is usually prone to Stiff-person Syndrome?

Stiff-person syndrome is, fortunately, an extremely rare condition and only about one out of every one million people have been diagnosed with this syndrome. And, it's twice as common in women than in men. Symptoms of the condition usually start developing around the age of 30 to 60. It is more likely seen in people with certain types of autoimmune disorders, such as diabetes, thyroiditis, vitiligo and pernicious anemia. People dealing with certain cancers, such as breast, lung, kidney, thyroid, colon and Hodgkin’s lymphoma, are also prone to the disease.

What causes the rare autoimmune movement disorder?

Researchers are unclear of the exact cause of this syndrome, however, it is believed that the syndrome is an autoimmune disorder, a condition where the immune system attacks healthy cells over time. People, dealing with this disorder, make antibodies that attack an enzyme called glutamic acid decarboxylase (GAD), which plays a pivotal role in making a neurotransmitter called gamma-aminobutyric acid (GABA). GABA helps control muscle movement.

Meanwhile, antibodies to another protein called amphiphysin are a less common finding in people suffering from this syndrome. This protein is involved in helping nerve cells communicate with each other and is found in nerve terminals.

But, do remember that the exact role that GAD plays in the development or worsening of this syndrome is not totally understood by experts and scientists. In fact, there have been cases where people with this syndrome do not have detectable antibodies to GAD.

Symptoms of Stiff-person Syndrome
  • Muscle stiffness near trunk and abdomen area
  • Intense pain
  • Discomfort
  • Hunched posture
  • Painful muscle spasms
  • Uncomfortable sleep

Symptoms for this disorder can take several months or years to develop. While some patients remain stable over the years, others' conditions worsen with time.

Diagnosis available for the condition

If you are experiencing one or more of the symptoms mentioned above, you must consult your healthcare provider. After a quick consultation, they will advise you to take a couple of tests. Here's a list!

Electromyography (EMG) - An EMG machine will be used to measure electrical activity in your muscles to detect continuous motor activity.

Blood test - A blood test will be done to check whether your body has antibodies to GAD or amphiphysin. It will also help detect signs that might indicate or rule out other diseases. About 60 and 80 per cent of people with stiff person syndrome have antibodies against GAD.

Lumbar puncture - For a lumbar puncture, a doctor uses a needle to draw fluid out of your spinal canal and checks for the presence of antibodies to GAD or for other signs.

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