Stiff person syndrome: the rare disease that will drive Céline Dion away from the stage

Céline Dion, 54, has announced that she is suffering from a very rare disease. This will force her to distance herself from shows and concerts to focus on treatment.

Indeed, has rescheduled many 2023 tour dates across Europe. Some have passed for 2024, but there are others canceled.

In a statement released through the social networks, Céline Dion said she suffers from stiff person syndrome. After recognizing that her strange symptoms didn’t allow her life to develop normally, she was finally able to find out where they came from.

Unfortunately, these spasms affect all aspects of my daily life, sometimes causing difficulty when walking. They don’t let me use my vocal cords to sing like I’m used to.

Céline Dion, in the video explaining her illness

What is stiff person syndrome?

The hallmark of stiff person syndrome is spasms. Also, as the name indicates, there is a tendency for the person to experience stiffness in the muscles.

It is considered a autoimmune disease. This means that it is the same organism that produces antibodies that target the central nervous systemproducing the classic symptoms of the disorder.

Second statisticsIt 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 that occur in family groups.

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

  • Pernicious anemia.
  • Diabetes.
  • Epilepsy.
  • Thyroiditis.
  • Vitiligo.

Why do symptoms occur?

Céline Dion’s disease has neurological symptoms. As the singer described in her video, there are spasms that can occur in different areas of the body.

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

Being a degenerative disorder, which tends to progress, abnormal postures may appear over the months and years. Patients bend over and have multiple fallssince they cannot mobilize members easily.

In the investigations in this regard it has not been clear what the specific point of attachment of the antibodies is. The strongest evidence points to it the immune system blocks an enzyme responsible for the production of the substance GABA. Therefore, with less GABA available, neurons become inefficient at controlling muscle movement.

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

How did Céline Dion’s disease get diagnosed?

While the Canadian singer didn’t provide details on the diagnostic process, we know it wasn’t easy. According to his statements, I’m still learning what the problem is and had to go through a tedious route to certify the origin of the spasms.

Symptoms can initially confuse doctors. Misdiagnoses are likely to be made, thinking of more frequent pathologies in the population, such as the following:

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

Currently, the standard test for diagnosis is a blood test in which Antibodies are sought against the enzyme that produces GABA. Its presence is indicative of stiff person syndrome, as up to 80% of patients are positive for it.

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

Finally, lumbar puncture can also be used. Through the study of the characteristics of the cerebrospinal fluid, other pathologies that could cause the same symptoms are excluded.

Diagnostic criteria for stiff person syndrome

THE clinical guidelines have established a number of criteria that must be met for a person to be diagnosed with the syndrome. Are the following:

  • Lack of expression on the facewith facial stiffness.
  • Stiffness of the abdominal muscleswith a tendency to hunch the back and difficulty breathing.
  • Muscle spasms activated by specific stimuli, such as noise or touch. The duration of the spasms is several minutes.
  • Continuous electrical activity on the electromyogram.
  • Lack of diagnostic evidence of other neurological disorders.
  • Increased levels of antibodies against the GABA enzyme in the blood.
  • Improvement of patients’ symptoms after initiation of benzodiazepine treatment.
Hand spasms due to Celine Dion's disease.
The recurring spasms of the syndrome interfere with daily activities.

Does Celine Dion’s rare disease have a cure?

there is no cure for stiff person syndrome. The problem is addressed with medications and rehabilitative physical therapybut there is still no protocol that reverses the progression of the condition.

Physiotherapy and kinesiotherapy jobs are essential. They aim to improve the quality of daily life.

I am working hard with my Sports Medicine Therapist to regain my strength and ability to perform.

Céline Dion, in the video about her illness

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

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

It is very difficult to establish a concrete forecast. Each case evolves differently and, while there are patients with marked improvement, there are also those who don’t respond to medication or therapy.

Céline Dion’s illness meant a serious blow to her career. Suspension of the concerts is the logical decision she had to make in order to focus on treating her.

All I know how to do is sing. It’s what I’ve done all my life. And that’s what I like to do the most.

Celine Dion

Post Stiff person syndrome: the rare disease that will drive Céline Dion away from the scene appeared for the first time in research-school.



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