Singer Celine Dion has postponed tour dates planned for 2023 due to stiff person syndrome diagnosis. What is this rare neurological disorder affecting only one or two people in a million?
Dion announced the news in a video posted on Instagram. She said she had been dealing with health problems for a long time and was recently diagnosed with stiff person syndrome.
"While we are still learning about this rare condition, we now know this is what's been causing all of the spasms I've been having," she said.
Dion is now working with her sports medicine therapist every day to build strength and ability to perform again, but admits "it's been a struggle."
What is stiff person syndrome?
Stiff-person syndrome (SPS) is a rare neurological disorder affecting only about one or two in a million people. Women develop SPS twice as many as men. The disease typically affects people who are aged between 40 to 60.
The cause of SPS is still unknown, but scientists think it may have something to do with the immune system attacking a protein called glutamic acid decarboxylase (GAD).
GAD helps produce a substance called gamma-aminobutyric acid (GABA), which plays a role in regulating motor neurons by decreasing their activity. Low levels of GABA can cause those neurons to fire continuously even when they're not supposed to.
What are the symptoms?
The SPS is characterized by muscle stiffening in the torso, limbs, and even the face, which eventually remains constant. As a result, patients may develop a hunched posture, and in severe cases, the stiffness of muscles can make it hard to walk or move.
Another symptom of SPS is episodes of violent muscle spasms, which can last from a few seconds to a few hours. Environmental stimuli, such as loud noises or touch, and emotional stress can trigger them. The muscle spasms can be so severe that the person falls down.
How is SPS diagnosed?
A definitive SPS diagnosis can be made with a blood test measuring the level of GAD antibodies, as most people with the condition have them elevated. Examination of the comprehensive medical history and other tests, such as electromyography or spinal tap, are also recommended.
The condition, however, is often misdiagnosed as Parkinson's disease, multiple sclerosis, fibromyalgia, or psychosomatic illness.
What is the treatment for SPS?
There is no cure for stiff person syndrome, but some symptoms can be managed with benzodiazepines, such as diazepam and clonazepam, which are anti-anxiety and muscle-relaxing drugs.
To alleviate muscle spasms, baclofen or gabapentin can be prescribed.
Other treatment options include intravenous immunoglobulin (IVIG), plasmapheresis, rituximab, and autologous stem cell transplant.
Non-medication options, such as physical therapy or message, can also be effective when given along with medicine.
Can SPS be prevented?
As the cause of stiff person syndrome is still unknown, the disease cannot be prevented.