Last week, due to a neurological disease known as stiff person syndrome (SPS), Celine Dion was forced to make the difficult decision to cancel and reschedule concerts that were supposed to take place from 2023 to 2024.
“I always give 100% when I do my shows, but my condition is not allowing me to give you that right now,” she explained in her Instagram video.
Stiff Person Syndrome
Stiff person syndrome (SPS) is a rare neurological disease that affects around twice as many women than men. While there is no known cause, it is likely due to an immune system attack on a protein (glutamic acid decarboxylase – GAD) responsible for the production of the gamma-aminobutyric acid (GABA) neurotransmitter.
Since GABA is an inhibitory chemical, its role is to reduce nerve signalling. What this means is that GABA decreases neuronal stimulation, leading to healthy nervous system modulation, rather than constant excitement.
For individuals living with SPS, like Ms. Dion, without nervous system regularity, debilitating symptoms can arise such as muscle spasms, muscle rigidity, chronic pain, and elevated anxiety, which can then lead to hunched postures, decreased mobility, muscle stiffness, irregular walking gaits, and respiratory difficulties. It can be violent, leading to joint dislocation and broken bones. It can also trigger heightened sensitivity to noise, touch, and emotional distress, even resulting in the individual being wheelchair bound or bedridden for life.
“People with SPS may be afraid to leave the house because street noises, such as the sound of a car horn, can trigger spasms and falls,” the US National Institute of Neurological Disorders and Stroke (NINDS) says.
Living with SPS
Because of the rarity of SPS, affecting only around one million people worldwide, it is often misdiagnosed as Parkinson’s disease, multiple sclerosis (MS), fibromyalgia, and psychosomatic illness. And with no cure in sight, treatments such as limiting pain, decreasing muscle contractions, suppressing autoimmune responses, and medicating will be the road Ms. Dion will be travelling for the time being.
“I am working hard with my sports medicine therapist every day,” Dion said. “[I will] build back my strength and my ability to perform again.”
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