Stiff person syndrome (SPS) is a rare, progressive syndrome that affects the nervous system, specifically the brain and spinal cord. Symptoms may include extreme muscle stiffness, rigidity and painful spasms in the trunk and limbs, severely impairing mobility. Spasms can generate enough force to fracture bone. People with SPS often have heightened sensitivity to noise, sudden movements, and emotional distress, which can set off muscle spasms. Persistent symptoms can lead to abnormal posturing of the spine, such as being hunched over. The syndrome affects twice as many women as men.
Treatment aims to control symptoms and improve mobility and function. While some people on treatment for SPS may maintain reasonable levels of activity, the majority become increasingly disabled over time. Treatment options depend on the symptoms and severity in each person and may include:
Benzodiazepines - these are drugs that slow down the nervous system and may relieve muscle spasms and anxiety. They are generally considered the best initial therapy for SPS. Examples include diazepam and clonazepam.
Baclofen - this is a muscle relaxant that may be used for people in whom benzodiazepines are not effective or not well-tolerated. Some people benefit from using baclofen in addition to benzodiazepines.
Immune modulating therapies - these may be considered in people with severe symptoms who do not experience relief with benzodiazepines and baclofen. Options may include intravenous immune globulin (IVIG) therapy, plasmapheresis (also called plasma exchange), and rituximab. However studies supporting the effectiveness and safety of these therapies for SPS are limited.
Physical therapy and occupational therapy are also an important part of management for SPS and may help with side effects of medications (such as weakness) in addition to symptoms of the disease.
Last updated: 3/14/2018
How can I learn about clinical trials involving stiff person syndrome?
You can also contact the Patient Recruitment and Public Liaison (PRPL) Office at the National Institutes of Health (NIH). We recommend calling 1-800-411-1222 to speak with a specialist who can help you determine if you are eligible for any clinical trials. If you are located outside the United States and would like to be contacted via telephone, you will need to contact the PRPL and provide your telephone number in full, including area code and international dialing prefix.
You can find information about participating in a clinical trial, as well as learn about resources for travel and lodging assistance, through the How to Get Involved in Research section of our website.
Last updated: 9/3/2016
We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please