Ever since Celine Dion, the popular superstar, announced the cancellation of her 2023 tour due to a diagnosis of Stiff Person Syndrome, or SPS, the disease has been in the spotlight. It is often misdiagnosed due to its similarity to severe stiffness in muscles and spasms in limbs or in backs.
Although SPS may not be fatal, symptoms are so severe that they impact a person’s quality of life. It is estimated to be one person out of approximately one million individuals.
As reported by UT Southwestern, research indicates that SPS and its antibodies protect the body from infections. Yet in SPS, the antibodies are autoantibodies that target the body’s normal areas. At least eighty percent of individuals with SPS harbor GAD autoantibodies. GAD is an enzyme that assists in creating GABA, a neurotransmitter that slows brain activity. One theory holds that actions of autoantibodies and the immune system cause less GABA resulting in stiff muscles and overactive nerves.
Although there is no cure for SPS, medications are available that relax the muscles as well as immunotherapy that controls symptoms. Muscle spasms may last either seconds or hours. Triggers may be cold temperatures, loud noises, or stress.
Available Treatment
Health care providers may order exams to rule out more common conditions and order blood tests to check for antibodies to GAD. The goal is to manage symptoms and improve mobility. Neurologists may prescribe muscle relaxants to help activate the GABA receptor. In SPS, very high doses are often required.
Some patients do well with muscle relaxants alone. GAD antibodies may be reduced through immunosuppressant medications or immunotherapy in severe cases. However, since SPS is extremely rare, there are currently very few clinical trials conducted to test these treatments.
Physical therapy and occupational therapy may provide relief from muscle pain. Cognitive behavioral therapy helps patients handle the emotional triggers causing muscle spasm. It also can help them develop skills for living with chronic conditions.
Many patients return to their normal activities once an effective combination of therapies has been found.
