why Celine Dion had to cancel concerts

Celine Dion has shared that she suffers from a very rare disease called stiff person syndrome. Discover all about it here.

Latest update: April 12, 2023

Celine Dion, 54 years old, has announced that she is suffering from the effects of a very rare disease called stiff person syndrome. This will force her to cancel concerts to focus on treatment.

In fact has she already rescheduled many dates of her European tour of 2023. Some were moved to 2024, but others have been cancelled.

In a statement released on social networks, Celine Dion stated that she suffers from stiff person syndrome. After acknowledging that she had strange symptoms that prevented her from living a normal life, she finally found out where they came from.

“Unfortunately, these cramps affect all aspects of my daily life, sometimes causing me difficulty walking. They don’t allow me to use my vocal cords to sing like I used to.”

-Celine Dion, in the video where she explains her illness-

What is Stiff Person Syndrome?

The feature of it stiff person syndrome his spasms. In addition, as the name implies, the person tends to have stiff muscles. (Spanish link)

It is considered an autoimmune disease (Spanish link). This means that the body itself produces antibodies directed against the central nervous systemcausing the classic symptoms of the condition.

According to the statistics (English link) it is more common in women, especially between the ages of 30 and 60. And while the possibility of a genetic predisposition is unclear, cases have been reported to occur in familial clusters.

It is common for patients to also have one of the following associated syndromes:

  • Pernicious anemia
  • Diabetes
  • Epilepsy
  • Thyroid inflammation
  • Vitiligo

Also read this article:
Symptoms of neuromuscular disorders

Why do the symptoms occur?

In stiff person syndrome, the antibodies would attack the enzymes responsible for making GABA.

Céline Dion’s disease has neurological symptoms. As the singer described in her video, she suffers from spasms that can occur in different places in the body.

It has been reported that muscle stiffness and contractions often respond to external stimuli. If a patient with stiff person syndrome is exposed to loud noises or even touch, the symptoms are triggered more intensely.

Because this is a degenerative condition, which tends to progress, abnormal postures can develop over months and years. Patients become bent over and fall more oftenbecause they cannot easily mobilize their limbs.

Research (English link) is not clear about the specific target of the antibodies. The strongest evidence suggests that the immune system blocks an enzyme responsible for the production of GABA. Thus, with less GABA available, the neurons become inefficient in controlling muscle movement.

How was Celine Dion’s disease diagnosed?

While the Canadian singer didn’t provide details about the diagnosis process, we know it wasn’t easy. According to her statements they are still figuring out what the problem is and had to go through a painstaking process to certify the origin of the spasms.

The symptoms may initially confuse doctors to take. Misdiagnoses are likely to be misdiagnosed, thinking of more common syndromes in the population, such as the following:

  • Fibromyalgia.
  • Multiple sclerosis.
  • Parkinson’s disease.
  • Anxiety crises or panic attacks.

Currently, the de rigueur test for diagnosis is a blood test that involves searching to antibodies against the enzyme that makes GABA. Its presence points to the stiffness syndrome, as up to 80% of patients test positive for it.

The electromyogram complements the tests. Besides being a method that every neurologist will ask in the presence of muscle spasms, it usually gives specific results here. Patients with Celine Dion’s disease have constant electrical motor activity, as if there was never any relaxation in the muscles of their extremities.

Finally can a lumbar puncture may also be used. By studying the characteristics of the cerebrospinal fluid, other pathologies that could cause the same symptoms are ruled out.

You may also be interested in this article:
What Are the Different Forms of Cerebral Palsy?

Diagnostic criteria for stiff person syndrome

Recurrent spasms of the syndrome interfere with daily activities.

Clinical guidelines have established a set of criteria that must be met in order for someone to be diagnosed with the syndrome. These are as follows:

  • Lack of facial expressionwith stiffness in the face.
  • Stiffness of the abdominal muscleswith a tendency to arch the back and difficulty breathing.
  • Muscle spasms that are triggered by specific stimuli, such as sound or touch. The duration of the spasm is several minutes.
  • Continuous electrical activity in an electromyogram.
  • Lack of diagnostic clues for other neurological pathologies.
  • Increased levels of antibodies against the GABA enzyme in the blood.
  • Improvement of patients’ symptoms after starting treatment with benzodiazepines.

Does Celine Dion’s Rare Disease Have a Treatment?

There is no cure for stiff person syndrome. It problem is addressed with drugs and physical rehabilitation therapybut there is still no protocol to reverse the progression of the condition.

Physiotherapy and kinesiotherapy are essential. They are aimed at improving the quality of everyday life.

I am working hard with my sports medicine therapist to regain strength and ability to perform.
-Celine Dion, in the video about her illness-

As for medicines is the first choice benzodiazepines. Many patients improve in the short term and slow progression with a regimen of these drugs.

Promising therapies are currently being investigated to slow the progression of stiff person syndrome. On the one hand, plasmapheresis with intravenous immunoglobulin could block the antibodies that attack the GABA enzyme. On the other hand, recent results confirm that stem cell transplantation is a viable alternative.

All I know how to do is sing. That’s what I’ve done all my life. And it’s what I love to do most.

It is very difficult to establish a specific prognosis. Each case develops differently and while there are patients with marked improvement, there are also those who do not respond to drugs and therapy.

Celine Dion’s illness thus dealt a serious blow to her career. Suspending concerts is the logical decision she had to make to focus on her treatment. Perhaps also interesting for you

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