Can Stiff-Person Syndrome Be Managed? Here Are 3 Things to Know
Singer Céline Dion announced in December 2022 that she diagnosed with a medical condition called stiff-person syndrome. The rare neurological disease caused her to cancel her 2023 tour dates. In a video on her Instagram, she detailed how spasms had interfered with walking and singing. Although the only affects about 1 or 2 in a million people, there is still much researchers don’t fully understand bout the disorder. Here are a few things you should know about stiff-person syndrome.
What is stiff-person syndrome?
The National Institute of Neurological Disorders and Stroke, a division of the National Institutes of Health, defines stiff-person syndrome as a neurological disorder with features of an autoimmune disease. Symptoms may include:
- Stiff muscles in the torso, arms and legs
- Greater sensitivity to noise, touch and emotional distress, which can cause muscle spasms
- Frequent falls and change in balance
There are several types of stiff-person syndrome:
- Classic stiff-person syndrome appears in 70% to 80% of patients affected by the condition and may coexist with other autoimmune disorders. Patients usually have symptoms that worsen with physical and emotional stress, cold weather and infection.
- Variants of stiff-person syndrome can involve specific parts of your body or involve major incoordination. Stiffness can eventually involve other muscles, but it remains most in one limb.
- Progressive encephalomyelitis with rigidity and myoclonus, known as SPS-plus syndrome, is a more extreme variant causing decreased consciousness, eye movement issues and autonomic dysfunction. This type requires management in a hospital due to involuntary functions in the body.
People may develop a change in posture over time and may become too disabled to walk or move. Celine Dion mentioned in her video that her health concerns were severe with persistent muscle spasms. The condition can slowly worsen and lead to permanent disability and death if not treated. Some spasms can last for several hours and can require a visit to the emergency room.
Patients have described complications like bending and turning and may walk like a “tin man.” Stiff-person syndrome could lead to other long-lasting orthopedic irregularities, such as abnormal postures and joint abnormalities.
How is stiff-person syndrome diagnosed?
Stiff-person syndrome can often be misdiagnosed as an anxiety disorder. A physician will conduct a physical exam and order laboratory tests that measure the presence of specific antibodies to determine the proper diagnosis of stiff-person syndrome. Antibody levels can be elevated in “the hundreds.” Low antibody levels may typically be considered normal for most patients and are frequently found in the healthy population.
How do you manage stiff-person syndrome?
Managing stiff-person syndrome can be done with treatments focusing on reducing pain and improving mobility. A neurologist may prescribe muscle relaxants, and medications such as diazepam or lorazepam, with high dosages in some cases. Other recommendations depending on severity, could include immunotherapy. Treatment is personalized.
Who can get stiff-person syndrome?
Stiff-person syndrome affects twice as many women as men, regardless of race. Most patients develop symptoms between the ages of 20 and 60, most commonly in their 30s and 40s. Only 5% of cases of SPS have been reported in children. Stiff-person syndrome is also associated with other autoimmune diseases, such as:
- Type-1 Diabetes: Once called insulin-dependent or juvenile diabetes. It usually develops in children, teens and young adults, but it can happen at any age. It is when the pancreas doesn’t make insulin or makes little.
- Thyroiditis: Swelling of the thyroid gland. It causes either unusually high or low levels of thyroid hormones in the blood.
- Vitiligo: Long-lasting autoimmune disorder that causes patches of skin to lose color
- Pernicious anemia: An autoimmune disease that manifests as megaloblastic anemia due to vitamin B12 deficiency. Anemia is a blood disorder in which the number of red blood cells is lower than usual.
Stiff-person syndrome is also more common in people with certain kinds of cancer, including breast cancer, lung cancer, kidney cancer, thyroid cancer, colon cancer and lymphomas. However, the reason for these links is still unknown.
Schedule an appointment with an Ochsner neurologist to learn more about stiff-person syndrome.