What Is Stiff Person Syndrome, the Rare Disorder Celine Dion Has?
Celine Dion announced Thursday that she was diagnosed with stiff person syndrome.
The rare condition can cause sometimes painful muscle spasms and falls.
There are treatment options available, but the right treatment depends on the patient’s symptoms.
Celine Dion surprised fans on Thursday when she revealed insight about her health: She has a rare condition called stiff person syndrome.
“I’ve been dealing with problems with my health for a long time, and it’s been really difficult for me to face these challenges and to talk about everything that I’ve been going through,” the 54-year-old singer said on Instagram, before noting that she won’t be able to restart her tour in Europe in February.
“Recently, I’ve been diagnosed with a very rare neurological condition called stiff person syndrome, which affects something like one in a million people,” she shared in the post’s video. “While we’re still learning about this rare condition, we now know that this is what has been causing all of the spasms that I’ve been having.”
The comments of her post were flooded with well-wishes, but the message naturally raises questions about stiff person syndrome and what it means for someone’s health. Here’s what you need to know.
What is stiff person syndrome?
Stiff person syndrome, aka Moersch-Woltman syndrome, is a rare neurological disorder that has characteristics of an autoimmune disease, according to the National Institute of Neurological Disorders and Stroke (NINDS).
The condition impacts the central nervous system and people with stiff person syndrome typically have stiffening in the muscles of their trunk, followed by stiffness and rigidity in the legs and other muscles, according to the Cleveland Clinic. The condition can cause painful muscle spasms that happen randomly or can be triggered by noise, emotional distress, and light physical touch, the organization says.
Stiff person syndrome can cause someone to have an altered posture, Cleveland Clinic says and, in severe cases, it can limit a person’s ability to walk or move.
The condition is very rare—about one out of every one million people have been diagnosed with it, the Cleveland Clinic says, but twice as many women have stiff person syndrome as men. “It may be a little under-diagnosed,” says Nicholas Lannen, M.D., who is a neurologist with Corewell Health West in Grand Rapids, Mich. “But this is a condition that’s so rare, many neurologists will never even see it.”
Stiff person syndrome symptoms
Symptoms of stiff person syndrome typically develop between the ages of 30 and 60, the Cleveland Clinic says.
According to the Yale Medicine, symptoms can include:
muscle stiffening in the torso and limbs
episodes of violent muscle spasms, that can be so severe that they cause the person to fall down
difficulty walking
depression
anxiety
How is stiff person syndrome diagnosed?
Because it’s so rare, stiff person syndrome can be misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia, NINDS says. It can also be confused with a spinal cord injury, says Amit Sachdev, M.D., medical director for neurology and director of the Muscle Dystrophy Clinic at Michigan State University.
The condition is diagnosed with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies in the blood, NINDS explains. (People with stiff person syndrome have higher-than-usual levels of GAD, an antibody that works against an important neurotransmitter in the brain.
“It’s very important if someone suspects that they have stiff person syndrome, to get seen by the right specialist because there can be many mimicking conditions,” Dr. Lannen says.
Stiff person syndrome treatment
“The goal of treatment is symptoms and mobility improvement,” says Nizar Souayah, M.D., a neurology professor at Rutgers New Jersey Medical School. But treatment for stiff person syndrome is based off of symptoms. “There are two things to recognize in stiff person syndrome,” says Dr. Sachdev. “The immune system is attacking the spinal cord and that attack needs to be stopped using immune suppression. The spinal cord has been damaged and the symptoms of spinal cord injury need to be addressed using medicines that reduce spasms.”
That can mean using medications like benzodiazepines or baclofen to treat muscle stiffness and spasms, along with anti-seizure medications, according to the Cleveland Clinic. Intravenous immunoglobulin (IVIG), plasmapheresis, rituximab, and autologous stem cell transplant, may also be used, per the Cleveland Clinic, along with things like physical therapy, massage, water therapy, heat therapy, and acupuncture.
What is the prognosis with stiff person syndrome?
It depends on when it’s diagnosed. “We know how to stop the progression of this disease with immune suppression,” Dr. Sachdev says. “If caught before significant disability, it does not have to be fatal or reduce life expectancy.”
Medications can improve symptoms of stiff person syndrome but won’t cure the condition, NINDS says. Most people with the disorder will have frequent falls and can be injured as a result. With the right treatment, symptoms are usually controlled, NINDS says. Still, “many patients progressively decline,” Dr. Souayah says.
But while symptoms of the disorder can get worse with time, it’s not a given, says William Buxton, M.D., board certified neurologist and director of Neuromuscular and Neurodiagnostic Medicine and of Fall Prevention at Pacific Neuroscience Institute at Providence Saint John’s Health Center in Santa Monica, Calif. “The handful of people I’ve seen with stiff person syndrome have not been particularly progressive—it’s not universally progressive,” he says. Still, Dr. Buxton says, “most people have some degree of decline over time.”
What research is being done?
The NINDS says that it’s doing research on the disorder and has given grants to major medication institutions around the country. There was a study that looked into using the drug rituximab, but it “proved ineffective” in treating people with stiff person syndrome, NINDS says.
Research that’s happening now is focused on understanding the cause of stiff person syndrome and how anti-GAD antibodies may help, per NINDS.
You Might Also Like
