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In early December, Celine Dion shared the news of her diagnosis with Stiff-Person Syndrome (SPS), a progressive neurological condition. She revealed that due to this rare disorder, which affects only about 1 or 2 people per million, her 2023 European tour dates would be postponed to 2024.
For many, this announcement marked their first encounter with the existence of SPS. Here’s a breakdown of what you should know about this rare condition.
What is Stiff-Person Syndrome?
SPS is an autoimmune neurological disorder. Individuals with this condition experience severe muscle spasms and stiffness in their torso, arms, and legs. For Celine Dion, SPS has even affected her vocal cords, rendering her unable to sing. The condition can also lead to heightened sensitivity to sound, stress, and touch, with these triggers potentially causing the spasms. In some cases, the spasms become so intense that they impair mobility, leading to disability.
What Triggers Stiff-Person Syndrome?
Due to the rarity of SPS, the exact cause remains unclear, though researchers are closing in on a potential explanation. According to Yale Medicine, one possibility is that the immune system mistakenly attacks the body’s glutamic acid decarboxylase (GAD) protein. GAD is responsible for producing gamma-aminobutyric acid (GABA), which regulates motor neurons in the brain. A decrease in GABA results in overactive neurons, leading to spasms.
SPS occurs more frequently in women than men, with symptoms typically emerging between the ages of 30 and 60. It is often diagnosed alongside another autoimmune disorder, such as type 1 diabetes, and is more common in individuals with cancer.
Is SPS Treatable?
Sadly, there is no cure for SPS at the moment. However, it can be managed through medication, immunotherapies, and certain types of physical therapy. Medications like the anti-anxiety drug diazepam, the anticonvulsant gabapentin, and the muscle relaxer baclofen are used to alleviate the spasms and stiffness associated with SPS, according to the National Institute of Neurological Disorders and Stroke. Intravenous treatments can also reduce sensitivity to sound and touch, while aqua therapy may help ease muscle pain.
