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Céline Dion announced: Stiff Person Syndrome

Céline Dion announced that she suffers from a serious and incurable neurological disease called "Stiff Person Syndrome"

The popular singer had to cancel all her shows. “I have been with health problems for a long time,” acknowledged the artist, who will not resume her concerts in 2023… / Céline Dion announced: Stiff Person Syndrome

The popular singer Céline Dion, author and performer of unforgettable musical hits, announced Thursday that she will cancel all her shows due to serious health problems that she has been facing for a long time. The Canadian artist announced in a video of her that she suffers from “Rigid Person Syndrome” (SPR), a rare neurodegenerative disease that will prevent her from continuing her concerts and tours.

In a video that she made public this Thursday, Dion -54 years old- revealed the reasons that led her to make the painful decision to end her presentations for the moment. Hi everyone, sorry it took me so long to get in touch with all of you. I miss you so much and can’t wait to get on stage and talk to you in person. As you know, I have always been an open book and before I was not ready to say anything, but now I am, ”the singer began her message.

“I have had health problems for a long time and it has been very difficult for me to face them and talk about everything that has happened to me. I have recently been diagnosed with a very rare neurological disorder called Stiff Person Syndrome, which affects 1 in a million people. Although we are still learning about this rare disease, we now know what has been causing all the spasms I’ve been having,” Dion revealed.

PRS causes the muscles of sufferers to tense uncontrollably and involuntarily. The disease ends up leaving those affected as “human statues”, since it progressively blocks the body in rigid postures that end up preventing them from walking or speaking, which could inevitably end the career of the Canadian, one of the most popular artists in the world. .

During the video, Dion pointed out that she has a great team of professionals surrounding her and trying to keep the disease from progressing as fast as it could be, even though her progression is inevitable. “I have a great team of doctors working by my side to help me get better and my precious children, who support me and help me,” said the singer.

“I am working hard with my sports medicine therapist every day to get my strength back and my ability to perform again, but I have to admit it has been a struggle. The only thing I know is that singing is what I’ve done all my life and what I like to do the most. Miss them a lot. I miss seeing all of you on stage performing for you,” she stated.

Celine Dion Stiff Person Syndrome: First cancellations

Céline Dion had already canceled her “Courage” tour last January due to persistent health issues, though the reasons behind her suspensions were not fully disclosed at the time. “I had high hopes that I would be ready to go back on stage now, but I realize I have to be more patient,” the star lamented on her Twitter account later that month.

It wasn’t the first time Dion had canceled concerts. The first shows had to be postponed due to the pandemic, she had already suspended scheduled performances in Las Vegas, western United States, between November 2021 and February 2022.

On March 9, she was to resume her tour in Denver and until April 22, she was scheduled to perform in 15 other cities in the United States and also Canada.

These shows are now also canceled because the recovery of the health of the singer, who was known to suffer from “severe and persistent muscle spasms” that prevent her from performing on stage, “is taking longer than expected,” the firm said that represents it in a press release last January.

Now, Dion confirmed that these “severe and persistent muscle spasms” are the product of a neurological disease that affects one in a million patients worldwide: the incurable Stiff Person Syndrome.

What is Stiff Person Syndrome?

Stiff Man Syndrome (SMS) or gender-neutral Stiff Person Syndrome (SPS) is a rare neurological disease characterized by a generalized increase in muscle tone. It is an autoimmune disease that can occur spontaneously or as a paraneoplastic syndrome. According to estimates, the number of people suffering from SMS in Germany is around 300. Of these, around two thirds are women and one third are men.

Stiff Person Syndrome symptoms

A characteristic of SMS is an increase in muscle tone that increases over months to years; in addition, spontaneous or triggered spasms occur in the affected muscles. The back and hip muscles are usually affected symmetrically. While in some patients the muscle tone is only slightly increased, in others the back stiffness is so pronounced that it limits trunk flexion. An increase in lumbar lordosis is also a result of the increased tension in the back muscles. The symptoms can fluctuate, especially at the beginning, but the stiffness is often permanent over the course of the disease. The gait can be slowed down by the involuntarily increased tension of the hip and leg muscles and appear cautious and clumsy. The spasms can be triggered by external and internal stimuli (such as touch, movement, sudden loud noises). They typically begin with a brief, involuntary muscle contraction (myoclonus), which is then followed by the sustained (tonic) and painful contraction of the muscle. Emotion-related (joy, laughter, enthusiasm, anger, excitement) short-term loss of muscle tone (cataplexy) can also occur. Usually both halves of the body are affected.

Variants of stiff man syndrome

SMS forms a syndrome from a broader clinical spectrum, which also includes focal SMS (also called stiff-limb syndrome or stiff-leg syndrome (SLS)), progressive encephalomyelitis with rigidity and myoclonus (PERM), and paraneoplastic are attributable to SMS. The variants of the SMS differ mainly with regard to the affected muscles and the presence of other pathological neurological findings. Over time, an SLS can turn into an SMS, which in turn can develop into a PERM even years later.

Diagnosis

The diagnosis is based on the anamnesis, the clinical-neurological examination, the electromyographic examination and the laboratory evidence of antibodies against endogenous proteins (autoantibodies).

Electromyography shows continuous motor unit activity. It is the reason for the increased muscle tone and is also detectable when the patient tries to relax completely. On the other hand, the continuous activity during sleep, during spinal anesthesia and during anesthesia is less pronounced.

In laboratory medicine, antibodies against the enzyme glutamic acid decarboxylase (GAD) are found serologically in 60–90% of patients with SMS. This enzyme is necessary for the synthesis of the neurotransmitter γ-aminobutyric acid (GABA) in nerve cells. However, autoantibodies against GAD are not only found in SMS, but also in other diseases such as type 1 diabetes mediated by the immune system. Antibodies against the protein amphiphysin can be found in patients with paraneoplastic SMS. Amphiphysin antibodies are also found in other paraneoplastic diseases such as paraneoplastic encephalomyelitis (PEM). The usefulness of determining antibody titers in serum and CSF as a marker for disease progression is questionable.

Therapy

Therapy based on the disease mechanism essentially has two starting points. On the one hand, attempts are being made to increase the GABA-dependent inhibition in the central nervous system. Benzodiazepines and baclofen can be used for this. On the other hand, the suspected autoimmune process is to be weakened by suppressing the immune system (immunosuppression). Therapeutic measures of this type include administration of intravenous immunoglobulins, plasmapheresis, administration of methylprednisolone and therapy with rituximab. If antibodies against amphiphysin proteins are found in the serum and CSF, a causal underlying tumor disease must be sought (usually a breast carcinoma or a bronchial carcinoma is present). If a tumor is found, it should be treated according to oncological guidelines.

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