In an Instagram post, Dion shared, “Recently, I’ve been diagnosed with a very rare neurological condition called Stiff Person Syndrome, which affects something like one in a million people. 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.” We spoke with both a neurologist and an assistant professor of Clinical Neurology to find out more about this uncommon condition.
What Is Stiff Person Syndrome?
Stiff Person Syndrome (SPS) is a rare neurological disorder that causes stiffness and rigidity of muscles. As Cleveland Clinic describes, SPS “affects the central nervous system (the brain and spinal cord).”
Symptoms of Stiff Person Syndrome
Leah Croll, MD, Assistant Professor, Clinical Neurology, Lewis Katz School of Medicine at Temple University, explains the most prominent symptoms are:
Stiffness affecting any number of musclesMuscle spasms that are triggered by loud noises and emotional upsetDifficulties with walking
Cleveland Clinic shares, “People with this condition first experience a stiffening of the muscles of their trunk followed, over time, by the development of stiffness and rigidity in the legs and other muscles in the body.” Patients with Stiff Person Syndrome are also more prone to develop anxiety, depression and phobias.
Causes of Stiff Person Syndrome
The cause of Stiff Person Syndrome is, unfortunately, not known. The disease comes from a disruption in the chemical signaling that occurs between the central nervous system and the muscles, Dr. Croll states. The exact cause of this disruption remains unclear, but it seems to be related to an auto-immune process. “It used to be considered an autoimmune disorder, but it’s not,” says Dr. Joey Gee, a neurologist with Providence Mission Hospital in Southern California. “But people with the condition do have biomarkers of antibodies to glutamic acid decarboxylase.” According to Cleveland Clinic, “Twice as many women have stiff person syndrome as men. Symptoms can occur at any age but usually develop between ages 30 and 60.” And SPS is more likely to be seen in individuals who have autoimmune disorders (including diabetes, thyroiditis, vitiligo and pernicious anemia) or cancers (such as breast, lung, kidney, thyroid, colon and Hodgkin’s lymphoma).
How To Manage and Treat Stiff Person Syndrome
Unfortunately, there is no cure for Stiff Person Syndrome, so management is focused on minimizing symptoms as much as possible and helping patients to cope with their new reality, Dr. Croll explains. This may entail medications to relax the muscles, immune system-modulating therapies and physical therapy. “The predominant treatment [for] Stiff Person Syndrome is to increase the effectiveness of gamma-aminobutyric acid (GABA) through medication,” says Dr. Gee. “GABA is an inhibitory neurotransmitter that decreases a nerve cell’s ability to send, receive or create chemical messages to other nerve cells.” Benzodiazepines are another treatment for those with Stiff Person Syndrome, along with muscle relaxant therapy and other medications that address GABA. Long-term treatment also includes immunotherapy, Dr. Gee adds. Next up: Everything You Need to Know About Aphasia, the Neurological Disorder Bruce Willis Is Battling
Sources
Leah Croll, MD, Assistant Professor, Clinical Neurology, Lewis Katz School of Medicine at Temple UniversityDr. Joey Gee, a neurologist with Providence Mission Hospital in Southern CaliforniaStiff Person Syndrome. Cleveland Clinic.
