In a heartfelt Instagram video, Celine Dion announced Thursday that she is cancelling or postponing several shows due to recent complications with Stiff-Person Syndrome, or SPS. While there’s only a one in a million chance of getting this neurological disease, it can be incredibly debilitating.
The disease is exactly as it sounds. According to the Cleveland Clinic, symptoms are characterized by a stiffness in muscles throughout the body, causing difficulty with movement and activities of daily living. At early stages, the stiffness comes about with a trigger, like a sudden noise or physical touch. As the disease progresses, the muscles hold their stiffness. In severe cases, the ability to walk is lost.
Painful muscle spasms can also occur, lasting anywhere from seconds to hours. They can affect the entire body or a small portion. In some cases, they have the potential to be violent enough to dislocate a joint or break a bone.
Symptoms typically arise between the ages of 30 and 60 but can come at any time, according to Yale Medicine. Women are twice as likely to get it than men, and scientists are not sure why that is. Those with other autoimmune disorders, like diabetes and thyroiditis, are more likely to develop SPS.
Very little is known about this disease and what causes it. Researchers believe it is an autoimmune disease, meaning the body mistakenly attacks its own healthy cells. They believe the body is attacking an enzyme called glutamic acid decarboxylase, or GAD. This enzyme plays a role in the creation of the neurotransmitter gamma-aminobutyric acid, or GABA. While GABA wears many hats, one of its main functions is helping control muscle movement. When the body attacks the GAD cells, less GABA is created, leaving muscle control difficult.
The disease is often misdiagnosed as other similar conditions like multiple sclerosis and muscular dystrophies. Doctors do blood tests or a spinal tap to measure the presence of antibodies to GAD, according to Johns Hopkins Medicine. Another route of diagnosis is electromyography, or EMG. EMG measures the electrical signals in your muscles, to see levels of motor activity.
There’s no cure for SPS, although there are several drugs and therapies that help minimize symptoms. Benzodiazepines, like diazepam, are often used. According to the National Institutes of Health, these drugs enhance the effect of GABA, making muscle control easier. Anti-seizure drugs may also be prescribed to lessen pain.
Non-drug options like physical therapy, massage therapy, and acupuncture are often used to limit pain and increase muscle mobility as much as possible. Pain and symptom management is the treatment goal until more research on SPS is done.
Cori Ritchey, NASM-CPT is an Associate Health & Fitness Editor at Men’s Health and a certified personal trainer and group fitness instructor. You can find more of her work in HealthCentral, Livestrong, Self, and others.
