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Dec 9, 2020 · There are two main treatments for SPS: GABAergic (gamma-aminobutyric acid) therapy and immunotherapy. For treatment, we suggest starting with benzodiazepines as first-line treatment. We recommend adding levetiracetam or pregabalin if symptoms persist. As second-line therapy, we recommend oral baclofen over rituximab and tacrolimus.
- Overview
- Symptoms and Causes
- Diagnosis and Tests
- Management and Treatment
- Prevention
- Outlook / Prognosis
- Living With
Stiff person syndrome (SPS) is a rare chronic condition that causes muscle stiffness and painful muscle spasms. The severity of symptoms varies from person to person. There’s no cure for SPS, but treatment can help manage symptoms and slow the progression of the syndrome.
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OverviewSymptoms and CausesDiagnosis and TestsManagement and TreatmentPreventionOutlook / PrognosisLiving With
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What are the symptoms of stiff person syndrome?
The two main symptoms of stiff person syndrome are: Muscle stiffness or rigidity. Painful muscle spasms. SPS symptoms can spread to other areas of your body and/or get worse over time. Symptoms can take several months to a few years to develop. Some people’s symptoms remain the same for years. Others experience slowly worsening symptoms, including more severe spasticity/rigidity, which can limit their ability to perform activities of daily living.
What causes stiff person syndrome?
Researchers don’t know the exact cause of stiff person syndrome. But they think it’s an autoimmune condition, a condition in which your immune system attacks healthy cells for unknown reasons. Many people with SPS make antibodies against glutamic acid decarboxylase (GAD). GAD plays a role in making a neurotransmitter called gamma-aminobutyric acid (GABA), which helps control muscle movement. Researchers don’t yet understand the exact role that GAD plays in the development and worsening of SPS. It’s important to note that the presence of GAD antibodies doesn’t mean that someone has SPS. In fact, a small portion of the general population has GAD antibodies without any adverse effects. There are other antibodies associated with SPS, including glycine receptor, amphiphysin and DPPX (dipeptidyl peptidase-like protein 6) antibodies. There are also some people with SPS who have no detectable known antibodies. Research is ongoing to see if there are other potential antibodies that haven’t yet been discovered. Advertisement
How is stiff person syndrome diagnosed?
Stiff person syndrome can be difficult to, as since it’s rare and its symptoms are similar to other conditions such as ankylosing spondylitis, multiple sclerosis or other autoimmune conditions. A healthcare provider may use several tests to rule out these conditions and look for specific signs of stiff person syndrome. They’ll ask you questions about your symptoms and perform a physical exam and neurological exam. If your provider suspects stiff person syndrome, tests to confirm the diagnosis may include: Antibody blood test: A blood test can check for the presence of antibodies to GAD (or other relevant antibodies) and for other signs that might indicate or rule out other diseases. Electromyography (EMG): This test measures electrical activity in your muscles and can help rule out other causes of your symptoms. Lumbar puncture (spinal tap): During a lumbar puncture, a healthcare provider uses a needle to draw fluid from your spinal canal to check for the presence of antibodies to GAD. They’ll also look for other signs that might indicate or rule out other conditions.
What is the treatment for stiff person syndrome?
Treatment for stiff person syndrome is based on your symptoms. The goal of treatment is to manage symptoms and improve your mobility and comfort. The two main treatment strategies include: Medications and therapies for symptom management. Immunotherapy, or disease-modifying treatment. Your healthcare team may include several specialists, such as: Neurologists, specifically, neuroimmunologists. Occupational and physical therapists. Physical medicine and rehabilitation specialists. Speech therapists. Mental health specialists, such as psychologists.
Can I prevent stiff person syndrome?
As SPS is an autoimmune-related condition, there’s nothing you can do to prevent developing it.
What is the prognosis for stiff person syndrome?
Stiff person syndrome is a chronic (lifelong) condition. The prognosis (outlook) varies from person to person based on a few factors, including: Symptom severity. How quickly the condition progresses. How well treatment helps. Starting treatment soon after symptoms begin is essential to prevent or lessen the syndrome’s progression and avoid long-term complications. Most people with SPS improve with medications, but it can still be difficult to manage triggers that cause muscle spasms. Over time, walking can become more and more difficult. Your ability to perform daily, routine tasks may also decline over time. The increased risk of falls also becomes a growing concern as SPS worsens. Some people may need to use a cane, walker or wheelchair for assistance.
How do I take care of myself if I have stiff person syndrome?
If possible, try to find a healthcare provider who specializes in researching and treating stiff person syndrome. Because the syndrome is rare, this may be difficult. You may have to advocate for yourself to ensure you get the best medical care that can help you have the best quality of life. It’s common for people with SPS to experience anxiety or depression related to the condition. If you’re experiencing mental health symptoms, it’s important to talk to your provider or a mental health specialist, such as a therapist or psychologist. You and your family may also want to consider joining a support group to meet others who can relate to your experiences.
When should I see my healthcare provider?
If you have stiff person syndrome, you’ll need to see your healthcare team regularly to check if your treatment is working and to monitor the progression of your symptoms. If you notice new symptoms or side effects of your medications, talk to your healthcare provider.
What questions should I ask my doctor?
If you have stiff person syndrome, it may help to ask your healthcare provider the following questions: What treatment do you recommend based on my symptoms? What can I do at home to help my symptoms? What signs of complications should I look out for? What can I expect to happen to my health in the future? Are there any clinical trials for stiff person syndrome? A note from Cleveland Clinic Understanding your stiff person syndrome (SPS) diagnosis can be overwhelming. Your healthcare team will devise a management plan that’s unique to you and your symptoms. It’s important to make sure you’re getting the support you need and to stay attentive to your health. Know that your healthcare team will be there to support you and your family. Medically Reviewed Last reviewed by a Cleveland Clinic medical professional on 12/13/2022. Learn more about our editorial process.
A personalized SPS treatment plan typically addresses the syndrome’s autoimmune, neurologic, visual, mobility and pain aspects. Although currently, no therapy makes stiff person syndrome go away, treatment can help minimize the severity of symptoms and improve quality of life.
May 21, 2024 · Stiff-person syndrome (SPS, formerly called stiff-man syndrome) is an uncommon disorder characterized by progressive muscle stiffness, rigidity, and spasm involving the axial muscles, resulting in severely impaired ambulation [1,2].
Stiff person syndrome is a rare disease affecting only one or two people per million. It commonly causes muscle stiffness and painful spasms that worsen over time. They can be triggered by a variety of things including sudden movement, cold temperature or unexpected loud noises.
Jul 10, 2023 · Describe the clinical presentation and diagnostic criteria of stiff person syndrome (SPS). Summarize the management options for stiff person syndrome (SPS). Outline the importance of early recognition of patients with stiff person syndrome (SPS) and an interprofessional approach to improve outcomes.
Treatment consists of immunomodulatory therapies and symptom management. Stiff-person syndrome (SPS) is an autoimmune neuromuscular disorder characterized by severe and progressive rigidity, stiffness of truncal and proximal muscles, and painful muscle spasms. 1.
