Celine Dion: what is the “stiff person syndrome”, the rare disease suffered by the singer

• Writing
• BBC News World

3 hours

image source, Getty Images

In an emotional video posted on her Instagram account, Canadian singer Celine Dion announced that she suffers from “stiff person syndrome”, a rare autoimmune disease that affects the nervous system and affects “one in a million people”.

“As you know, I’ve always been an open book, and I wasn’t ready to say anything before, but now I am,” the “My Heart Will Go On” singer said, trying to hold back tears.

“Although we are still learning regarding this rare disease, we now know what has been causing the spasms I’ve been having,” confessed the 54-year-old singer, who has been suffering from health problems for some time.

But what symptoms exactly does this disease cause and how is it treated?

fluctuating stiffness

The “stiff person syndrome” is characterized by a fluctuating muscle stiffness in the torso and extremities, and a greater sensitivity to stimuli such as noise, touch, and emotional distress, which can cause muscle spasmsas defined by the US National Institute of Neurological Disorders and Stroke.

“Abnormal postures, often hunched over and rigid, are characteristic of the disorder,” the institute also indicates.

image source, Getty Images

People with this condition, which affects the twice as many women as menthey may have difficulty walking or moving, and often avoid leaving the house because street noises can trigger spasms and falls.

Most people with SPS – as the syndrome is known by its acronym in English – have frequent falls, and because they lack normal defensive reflexes, injuries caused by falls can be severe.

Causes

The scientists still do not understand what causes this syndromebut it is believed to be an autoimmune disease, that is, it is the person’s immune system that attacks their own central nervous system (brain and spinal cord).

The American institute has also associated it with other autoimmune diseases such as diabetes, thyroiditis, vitiligo and pernicious anemia.

The disease is so rare that it is often misdiagnose such as Parkinson’s, multiple sclerosis, fibromyalgia, psychosomatic illness or anxiety and phobia.

But to make a definitive diagnosis, a blood test must be done to measure the level of antibodies once morest glutamic acid decarboxylase. (GAD)since most people with SPS have elevated levels of these antibodies.

While this is a disease that currently has no cure, medications such as muscle relaxants can help improve symptoms.

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