Celine Dion Battles With Stiff Person Syndrome, A Rare Disease That Affects More Women Than Men

Claudette Dion has reported that her sister Celine Dion's health condition, Stiff-Person Syndrome, is regrettably progressing.

In an article published by the Canadian website 7Jours on Tuesday, December 12, 2023, Claudette said that the Grammy award winner is now encountering difficulties in controlling certain body movements.

Translated from French to English, her statement read, "She doesn't have control over her muscles.

"What breaks my heart is that she's always been disciplined. She's always worked hard. Our mother always told her, 'You're going to do it well, you're going to do it properly.'

"It's true that in both our dreams and hers, the goal is to return to the stage. In what capacity? I don't know."

The family's biggest worry is that the rare illness may also impact Celine's vocal cords and heart-both of which are muscles.

And unfortunately, there isn't much that can be done because this autoimmune movement disorder, according to National Library of Medicine's website, is "incompletely understood."

Claudette continued, "That's what's coming to get me. Since it's one in millions, scientists didn't do that much research, because it didn't affect that many people."

In December 2022, Céline sadly revealed the diagnosis in an Instagram post, mentioning that she and her teams of doctors and specialist s have been actively helping her keep the symptoms under control. 

On May 27, 2023, Celine announced the cancellation of her Courage World Tour concert due to the rare neurological disorder causing painful muscle spasms and stiffness.

Part of her post read, "I'm so sorry to disappoint all of you once again. I'm working really hard to build back my strength, but touring can be very difficult even when you're 100%.

"It's not fair to you to keep postponing the shows, and even though it breaks my heart, it's best that we cancel everything now until I'm really ready to be back on stage again."

In August 2023, Claudette told Le Journal de Montreal that Celine has been residing with their sister Linda in the Las Vegas area, where she is receiving care from doctors specializing in Stiff Person Syndrome.

On October 30, 2023, she attended a hockey game with her 22-year-old son René-Charles and her 13-year-old twins, Nelson and Eddy.

Her husband René Angélil died in 2016.

What is Stiff Person Syndrome?

According to Johns Hopkins Medicine, Stiff Person Syndrome (SPS) is a rare autoimmune neurological disorder characterized by muscle stiffness and painful spasms that can fluctuate in intensity and may be accompanied by symptoms such as an unsteady gait, double vision, or slurred speech.

The condition, which is believed to exist on a spectrum, primarily manifests in individuals aged 40 to 50 but can occur in children and older adults.

Although there is no cure for SPS, symptom management through collaboration with specialists can improve the quality of life for affected individuals.

SPS is considered rare, affecting an estimated one to two people per million, but recent perspectives suggest it may be more common than initially believed.

What are the symptoms of Stiff Person Syndrome?

Stiff person syndrome (SPS) primarily involves painful muscle contractions and spasms, typically initiating in the legs and back and potentially affecting other areas such as the abdomen, upper trunk, arms, neck, and face.

Spasms often occur episodically, triggered by surprises, sudden movements, cold temperatures, emotional stress, specific exercises, or touch.

Affected muscle areas can become stiff and board-like, leading to difficulties in walking, a rigid posture, unsteadiness, falls, shortness of breath, chronic pain, hyperlordosis (exaggerated lower back curve), and potential spinal cord compression.

Additional symptoms may include eye movement problems causing double vision, speech issues, lack of coordination, anxiety, and agoraphobia.

Symptom onset varies, with some experiencing leg cramps or stiffness that worsen gradually, while others may encounter different symptoms preceding muscle spasms.

What causes Stiff Person Syndrome?

Stiff Person Syndrome (SPS) is believed to be an autoimmune disorder, with the immune system attacking healthy tissues, and it predominantly affects women. Individuals with SPS often have unique anti-GAD65 antibodies in their blood, which block the GAD enzyme responsible for producing the neurotransmitter GABA. Insufficient GABA leads to hyperexcitability in the nervous system, causing physical symptoms like muscle spasms and psychological symptoms such as anxiety.

Triggers for SPS symptoms, like being startled, are thought to be linked to disruptions in the GABA pathway. SPS is commonly associated with other autoimmune disorders like type 1 diabetes, thyroid disorders, pernicious anemia, and occasionally vitiligo. Cancer, specifically paraneoplastic SPS, is rarely linked to SPS cases, with breast or lung cancer being the most common types identified within a few years of SPS symptom onset.

Blood tests, imaging studies, electromyography, and lumbar puncture can help confirm an SPS diagnosis.

Is Stiff Person Syndrome fatal?

While death directly from stiff person syndrome (SPS) is uncommon, it is usually caused by complications arising from the condition, such as blood clots or infections from immobility-related wounds. In rare instances, severe spasms in the chest muscles can impact breathing.

This story originally appeared on PEP.ph. Minor edits have been made by the Smartparenting.com.ph editors.