Canadian-born singing superstar Celine Dion shocked her fans this week by announcing she has a rare neurological disorder – one that most people had probably never heard of before her news.
In an emotional Instagram video, the 54-year-old chanteuse discloses she’s been diagnosed with a condition known as Stiff Person Syndrome. She recounts how it is having an impact “on every aspect of my daily life.” That includes her ability to walk, move and even use her vocal cords to sing. The illness has forced her to reschedule some of her tour dates and undergo regular therapy. “It’s been really difficult for me to face these challenges,” she says in the video.
Katie Beadon is a neurologist at St. Paul’s Hospital and a co-director of the St. Paul’s Immunotherapy in Neurology (SPIN) Clinic, where she treats patients with autoimmune neuromuscular disorders, including some with SPS.
She explains more about this exceedingly rare condition, which affects about one in a million people.
What is SPS?
Stiff Person Syndrome is characterized by progressive muscle stiffness, rigidity and spasms. It affects major muscles of the trunk, such as the back and abdomen. Imagine the pain of a charley horse, but to the nth degree. Painful muscle spasms can occur suddenly without warning, causing falls and limiting a person’s ability to walk. I know of one person whose back muscle spasms were so powerful they fractured vertebrae. Symptoms generally progress slowly in most patients. They are not particular to a specific age, affecting people from their 20s to 50s.
What causes it?
There seems to be an autoimmune component, as it is commonly linked to autoimmune disorders such as Type 1 diabetes and autoimmune thyroid disease. One patient I know of with SPS also had five different autoimmune disorders.
Is it genetic?
Not that we know of, although autoimmune diseases run in families. It seems to affect women more than men, which may be because women are more prone to autoimmune disorders.
Is it curable?
No, but it can be managed to the point that some patients can enter into remission with aggressive therapy.
How is it treated?
The first line of therapy includes anti-spasm medications such as benzodiazepines like Clonazepam (an anti-seizure medication). We also treat patients with IV immunoglobulin therapy. (Immunoglobulins carry antibodies to fight germs or disease as well as modulate the immune system.) Some patients may need stronger immune therapy like chemotherapy that suppresses the immune system.
Plasmapherisis, or plasma exchange, is another therapy. It is like dialysis, in which antibodies are removed from the blood. I have not used this with patients, but it is in the research literature.
What’s the impact of this condition on a person’s quality of life?
It is massive. These patients cannot work, they’re in significant pain, and their movement is severely affected. Spasms are unpredictable. They can come on at any time. One patient had a spasm crossing the street and fell in the middle of that street. We can see the impact on Celine Dion.