What is stiff person syndrome and how does the disease progress?

  • Canadian singer Celine Dion suffers from Stiff Person Syndrome, a neurological pathology that has no cure and affects the muscles of the body.

Stiff person syndrome is characterized by “episodes of stiffness and spasms in the muscles of the trunk and arms and legs,” which are often caused by increased sensitivity to noise, touch, and as a startle response, they point out. the US National Institutes of Health (NIH).

In December 2022, through a publication on her social networks, the international artist explained to her fans: “I have had health problems for a long time, it has been very difficult for me to face them and talk about everything that has happened to me. Although we are still learning about this rare disease, we now know what has been causing all the spasms.” Months later, in May of this year, she had to completely cancel her “Courage World Tour,” with no return date.

Claudette Dion, sister of the singer of hits like “My Heart Will Go On,” said in an interview that the artist lost control of her muscles and must indefinitely postpone her return to the stage. In an emotional report, Ella Claudette revealed that, despite Celine Dion’s efforts and discipline, the muscle spasms she suffers from due to her condition are uncontrollable.

What is stiff person syndrome?
“Stiff person syndrome is an autoimmune disease that affects approximately one person in every million and two-thirds of cases are women. The age of onset is usually between 40 and 60 years.

Experts agree that little is known about the disease, which is characterized as an autoimmune pathology in which the immune system attacks its own central nervous system (brain and spinal cord). In addition, it is frequently associated with other autoimmune diseases such as diabetes, thyroiditis, vitiligo and pernicious anemia, according to the NIH in the United States.
Symptoms may include:

  • Abnormal postures, such as hunching over
  • Episodes of muscle stiffness and spasms that occur due to increased sensitivity to noise, touch, and as a startle response
  • Falls due to muscle spasms and stiffness suffered by the patient while walking or standing
  • Cerebral palsy and/or epilepsy
  • What are the symptoms of stiff person syndrome?
    Dr. López Rosetti, who is president of the Stress Section of the World Federation for Mental Health (WFMH), explained that this neurological disorder manifests itself with progressive muscle rigidity and is characterized by the presence of antibodies that attack the necessary components. for the production of the neurotransmitter gamma aminobutyric acid, causing muscle spasms that usually begin in the lower extremities and then spread.
    “Muscles become stiffer and spasms may occur. In some cases, the voice may be affected, causing dysphonia, or there may be difficulty swallowing,” added the cardiologist.
    What are the treatments for stiff person syndrome?
    Specialists from the United States National Institutes of Health (NIH) emphasize that treatment depends on the specific manifestations of each case and includes the use of drugs such as benzodiazepines, diazepam or baclofen, in addition to anticonvulsant medications and for pain.
    Intravenous immunoglobulin (IVIG) therapies and plasmapheresis (which involves drawing and purifying blood to remove harmful antibodies) have shown promising results, as have autologous stem cell transplants. Physical and occupational therapy can also be helpful in slowing the progression of the disease.
    How does the progression of the disease affect the patient?
    “We can’t find any medicine that works, but having hope is important. She is listening to the best researchers in the field of this rare disease and she will do that as much as possible,” Celine Dion’s sister said in an interview in August.
    For patients in advanced stages of the pathology, it is very difficult to fall asleep at night, and they may also have difficulties walking or speaking.
    NIH experts note that in some patients, symptoms resolve with treatment, or the after-effects of the neurological disorder only affect a particular area of ​​the body.