Celine Dion’s heartbreaking announcement that she was forced to pull out of her tour due to “a very rare neurological disorder” has sparked an outpouring of sympathy, shock—and flat-out confusion over what the hell stiff person syndrome is.
While many may have initially found themselves wondering if the diagnosis was real, experts say the disease is no joke.
Affecting between one and two people in a million, the “very rare disease” leaves the afflicted suffering progressive muscle stiffness and painful spasms, according to Johns Hopkins. “These symptoms can lead to difficulty walking and, over time, even greater disability,” Yale Medicine notes.
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In severe cases, patients can be left incapacitated. Only a handful of health centers around the world specialize in the disease. There is no known cure, but patients can find at least some relief with medications, including anti-convulsants.
Dr. Scott Newsome, an expert who established the Stiff Person Syndrome Center at Johns Hopkins, said the disease is a “really challenging disability” that can be so hard to diagnose some patients are wrongly considered “psychiatrically unwell” at first.
“SPS is an autoimmune, neurological condition where, even though we don’t know the primary cause, the immune system gets angry and is misdirected to attack the nervous system in a way that causes signs and symptoms we see in patients: horrific spasms, generalized pain, eye movement issues, problems walking, there’s a full spectrum people can experience with this condition,” he said in an interview with the Stiff Person Syndrome Research Foundation.
In revealing her health struggles, Dion rightly noted that “we’re still learning about this rare condition.”
Experts still don’t know what causes it, but numerous studies are underway seeking new kinds of treatment, according to the U.S. National Institute of Neurological Disorders and Stroke.
For those suffering from the disease, that may not be much consolation.
The NINDS reports that some patients are literally “afraid to leave the house” because ordinary street noises “such as the sound of a horn” can trigger painful spasms or cause them to fall over.
The disease affects twice as many women as men, and frustratingly, it is often misdiagnosed as other autoimmune and neurological disorders.
Gay, a patient whose struggle with the disease was documented by Johns Hopkins, said she was “barely functioning” after first experiencing muscle stiffness and pain and weakness in her legs.
Her doctor initially didn’t even think there was a chance she had SPS because it’s so rare, she said.
Dion seemed to hint at a similar struggle, revealing on Instagram that her health struggles had been going on “for a long time” and “we now know this is what’s been causing all the spasms I’ve been having.”
