What is stiff-person syndrome as Canadian superstar Celine Dion reveals she’s been diagnosed with the rare debilitating condition?
In late 2022, the popular singer revealed she’d been diagnosed with the condition in an emotional post on Instagram.
The condition affects just one in a million people and ultimately leaves sufferers as human statues.
What is stiff-person syndrome?
Stiff-person syndrome is a rare neurological condition which causes muscles to tense uncontrollably.
The condition, which affects one in a million people, ultimately leaves sufferers as human statues as it progressively locks the body into rigid positions.
It leaves sufferers unable to walk or talk.
While there is no cure for stiff-person syndrome, there are treatments to slow down the progression.
The disorder makes the muscles in the torso and limbs alternate between spasming and being rigid.
Estimates suggest it affects around 70 people in the UK and 330 in the US.
The progressive disease sees patients’ stiffness increase over time and can lead to them needing to use a wheelchair.
There tend to be three types of the syndrome.
Classical person man syndrome is when rigidity and spasms are around the back and stomach, and occasionally thighs and neck. It can cause back curvature over time.
Stiff limb syndrome sees spasms especially affect the legs and feet, occasionally causing them to become fixed in place. Hands can also be affected.
Jerking stiff person syndrome is the rarest, most aggressive form. It includes symptoms from both the others, and also affects the head and eyes.
What causes stiff-person syndrome?
Experts do not know exactly what is behind the disease but they believe it may be caused by an autoimmune reaction.
This takes place when the body attacks its own nerve cells that control muscle movement.
In stiff-person syndrome, the immune system attacks a protein that helps make gamma-aminobutyric acid (GABA).
GABA regulates motor neurons — the nerves that control movement.
Low levels of GABA cause the neurons to continuously fire when they are not supposed to, resulting in the spasms and rigidity.
Spasms can be triggered by loud noises, with the condition also causing heightened sensitivity to sound.
Touch and emotional distress can also be felt more intensely as a result of the condition.
Stress and anxiety are also usually higher in those with the condition, particularly because of the unpredictability of spasms.
The lack of GABA — which regulates anxiety — also affects mental health.
It’s not clear which type of stiff-person syndrome Celine is suffering from.
View this post on Instagram
What has Celine Dion said about her diagnosis?
Celine told fans the diagnosis had forced her to cancel her upcoming European tour.
She said: “As you know I’ve always been an open book. I wasn’t ready to say anything before but im ready now.
These spasms affect every aspect of my daily life, sometimes causing difficulties when I walk.
“I’ve been dealing with problems with my health for a long time. It’s been really difficult for me to face my challenges and to talk about everything that I’ve been going through.
“Recently I’ve been diagnosed with a very rare neurological disorder called the stiff-person syndrome which affects 1 in a million people.
“While we’re still learning about this rare condition, we now know this is what’s been causing all the spasms I’ve been having.”
How is stiff-person syndrome affecting Celine?
She then revealed how the condition is taking its toll on her body.
“Unfortunately, these spasms affect every aspect of my daily life, sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to.
“It hurts me to tell you today that this means I won’t be ready to restart my tour in Europe in February.
“I have a great team of doctors working alongside me to help me get better and my precious children who are supporting me and giving me help.
“I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again. But I have to admit it’s been a struggle.
“All I know is singing is what I’ve done all my life and it’s what I love to do the most.
‘I miss you so much,” she said, adding: “I miss seeing all of you being on the stage performing for you.
“I always give 100% when I do my show but my condition is not allowing me to give you that right now.
“For me to reach you again I have no choice but to concentrate on my health at this moment and I have hope that I’m on the road to recovery.
“This is my focus and I’m doing everything I can to recuperate.
“Take care of yourselves. Be well. I love you guys so much and I really hope I can see you again real soon,” Celine concluded.
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