Table of ContentsView AllTable of ContentsSymptomsEffectsTreatmentDiagnosisSpecialists to SeeLiving With SPS
Table of ContentsView All
View All
Table of Contents
Symptoms
Effects
Treatment
Diagnosis
Specialists to See
Living With SPS
Stiff person syndrome (SPS) is a rare disorder that causes disabling muscle stiffness and spasms. It is an autoimmune neurological disorder that occurs when the body’s immune system attacks certain enzymes involved in muscle relaxation.
This condition affects approximately 1 out of 1 million people worldwide, with symptoms beginning between ages 20 and 50.Treatment can help slow progression and control symptoms, but there is no cure for stiff person syndrome. People can develop breathing difficulties as the condition progresses.
This article describes the symptoms, diagnostic process, and treatments of stiff person syndrome.
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Symptoms: How Does Stiff Person Syndrome Feel?
Stiff person syndrome causes painful stiffness, difficulty walking, problems maintaining balance, and trouble with moving the way you want to.As the condition worsens, it can cause falls, difficulty swallowing, vomiting, or constipation. It may make it hard to breathe.
If you have stiff person syndrome, you can have trouble with day-to-day activities, including self-care. And you might notice that you get startled easily, and the pain can become constant.The symptoms of stiff person syndrome are expected to worsen over time gradually.
The effects of stiff person syndrome occur due to muscle stiffness and muscle spasms, described as follows:
Triggers
People who have stiff person syndrome can be highly sensitive to sounds or touch. If you have been diagnosed with stiff person syndrome, you may experience worsening symptoms in response to triggers.
Triggers can include cold temperatures, sounds, touch, stress, or being startled.These triggers are so common that it is impossible to avoid them. You can also have worsening symptoms without any triggers.
Neurological and Autoimmune Effects in SPS
Stiff person syndrome is considered an autoimmune disease, which is a disease that develops when the body attacks its own tissue.
The symptoms occur due to a deficit ofgamma-aminobutyric acid(GABA) in the nerves that control your muscles. GABA is a neurotransmitter that helps balance nerve activity throughout the body, preventing excess nerve activity. In stiff person syndrome, this deficiency prevents muscles from relaxing as they should.
Stiff person syndrome develops when the immune system attacks glutamic acid decarboxylase (GAD), an enzyme that is involved in production of GABA.
What Are Autoimmune Diseases?
Risk Factors
There is no known underlying hereditary pattern or risk factor for developing stiff person syndrome. The condition is extremely rare, and there isn’t a gene or environmental exposure known to cause it.
Some people who have stiff person syndrome also have other immune system disorders, but having an immune system disorder does not mean that you are at risk for stiff person syndrome.
Sometimes stiff person syndrome develops as aparaneoplastic disorder.Aparaneoplasticdisorder is a condition in which cancer causes immune proteins to attack a very specific part of the body. Paraneoplastic syndromes are rare, and having stiff person syndrome does not necessarily mean that you have underlying cancer.
Life Expectancy With Stiff Person SyndromeThere is no specific prediction of how many years a person can live after a diagnosis of stiff person syndrome. This condition is not fatal, and people are able to survive for many years after diagnosis, but it can reduce life expectancy.Certain complications associated with stiff person syndrome can reduce life expectancy—these include severe breathing problems, infections, injuries due to falls, and overall decline in health due to the lack of self-care abilities.
Life Expectancy With Stiff Person Syndrome
There is no specific prediction of how many years a person can live after a diagnosis of stiff person syndrome. This condition is not fatal, and people are able to survive for many years after diagnosis, but it can reduce life expectancy.Certain complications associated with stiff person syndrome can reduce life expectancy—these include severe breathing problems, infections, injuries due to falls, and overall decline in health due to the lack of self-care abilities.
There is no specific prediction of how many years a person can live after a diagnosis of stiff person syndrome. This condition is not fatal, and people are able to survive for many years after diagnosis, but it can reduce life expectancy.
Certain complications associated with stiff person syndrome can reduce life expectancy—these include severe breathing problems, infections, injuries due to falls, and overall decline in health due to the lack of self-care abilities.
Treatments to Manage SPS Pain
Some treatments are available for managing stiff person syndrome. The treatments include medications or other interventions that can help with symptom management and immune treatments to control the underlying condition.
Treatments to relieve symptoms include:
Immune-modifying medications have many side effects, including increasing the risk of infections, and there are no well-established protocols for their use in the treatment of stiff person syndrome.
There is no cure for stiff person syndrome, but research is ongoing to find better treatments and a potential cure.
SPS Diagnostic Process
Generally, the diagnosis of stiff person syndrome can take weeks, months, or longer.It is such a rare disorder that more common neurological disorders are usually considered first before stiff person syndrome is diagnosed.
The diagnosis of stiff person syndrome begins with a review of your symptoms, health history, family history, and a physical examination.
Your evaluation will then involve a consideration of common conditions that can cause symptoms similar to those of stiff person syndrome—multiple sclerosis (MS),Parkinson’s disease,radiculopathy(pinched nerve),neuropathy, Guillain-Barré syndrome, lupus, and others.
Depending on the stage of your condition and the signs and symptoms affecting you at the time of your evaluation, further testing would likely be needed.
Tests that you may need include:
There is no definitive test that defines stiff person syndrome, but most people with the condition have anti-GAD antibodies.If your neurologist is considering the possibility that you might have stiff person syndrome, they will order this blood test for you.
SPS vs. MS
Similarities between SPS and MS include:
Differences between SPS and MS include:
Specialists Involved in SPS Care
If you are diagnosed with stiff person syndrome, you will need medical care from a variety of healthcare providers. You may see some of these providers more frequently during the initial diagnostic process and others during the ongoing treatment phase of your care.
Providers that you may see include:
Well-Being Tips With SPS
If you’ve been diagnosed with stiff person syndrome, it can be beneficial to join a support group or enroll in a clinical trial so you have access to the most updated information and treatments for this uncommon disease.
Coping involves practical strategies to help manage your symptoms and maintain your comfort while maximizing your quality of life. You and your loved ones may also need to seek professional support for help in dealing with the emotional aspects of living with this disease.
Tips for living with stiff person syndrome include:
Summary
Stiff person syndrome is a rare autoimmune neurological disorder. It causes muscle stiffness and spasms, which can interfere with your ability to move and manage daily self-care and other tasks. It can also cause discomfort or pain.
Because the condition is so rare, it can take time to get a diagnosis of stiff person syndrome—other, more common neurological conditions are usually considered first. If you are diagnosed with stiff person syndrome, you can expect lifelong care, usually involving a team of healthcare professionals.
Your treatment will include immune therapies to help manage and potentially slow down the disease, as well as symptomatic therapy to reduce the pain and spasms of stiff person syndrome. You may benefit from joining a support group or enrolling in a clinical trial to help you get access to the most updated treatments for this condition.
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6 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Bheemavarapu B, Singh A, Ranjini NJ, Abhilash Meda VS, Patil D.Recurrent acute on chronic respiratory failure in stiff person syndrome.Case Rep Neurol. 2023;15(1):187-191. doi:10.1159/000532093National Institute of Neurological Diseases and Stroke.Stiff person syndrome.Chia NH, McKeon A, Dalakas MC, et al.Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria.Ann Clin Transl Neurol. 2023;10(7):1083-1094. doi:10.1002/acn3.51791Koshorek J, Wang Y, Maldonado DP, et al.The many faces of gastrointestinal dysfunction in stiff person syndrome spectrum disorders.Front Neurol. 2023;14:1273256. doi:10.3389/fneur.2023.1273256Ortiz JF, Ghani MR, Morillo Cox Á, Tambo W, Bashir F, Wirth M, Moya G.Stiff-person syndrome: a treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995Dalakas MC.Therapies in stiff-person syndrome: advances and future prospects based on disease pathophysiology.Neurol Neuroimmunol Neuroinflamm. 2023;10(3):e200109. doi:10.1212/NXI.0000000000200109
6 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Bheemavarapu B, Singh A, Ranjini NJ, Abhilash Meda VS, Patil D.Recurrent acute on chronic respiratory failure in stiff person syndrome.Case Rep Neurol. 2023;15(1):187-191. doi:10.1159/000532093National Institute of Neurological Diseases and Stroke.Stiff person syndrome.Chia NH, McKeon A, Dalakas MC, et al.Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria.Ann Clin Transl Neurol. 2023;10(7):1083-1094. doi:10.1002/acn3.51791Koshorek J, Wang Y, Maldonado DP, et al.The many faces of gastrointestinal dysfunction in stiff person syndrome spectrum disorders.Front Neurol. 2023;14:1273256. doi:10.3389/fneur.2023.1273256Ortiz JF, Ghani MR, Morillo Cox Á, Tambo W, Bashir F, Wirth M, Moya G.Stiff-person syndrome: a treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995Dalakas MC.Therapies in stiff-person syndrome: advances and future prospects based on disease pathophysiology.Neurol Neuroimmunol Neuroinflamm. 2023;10(3):e200109. doi:10.1212/NXI.0000000000200109
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Bheemavarapu B, Singh A, Ranjini NJ, Abhilash Meda VS, Patil D.Recurrent acute on chronic respiratory failure in stiff person syndrome.Case Rep Neurol. 2023;15(1):187-191. doi:10.1159/000532093National Institute of Neurological Diseases and Stroke.Stiff person syndrome.Chia NH, McKeon A, Dalakas MC, et al.Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria.Ann Clin Transl Neurol. 2023;10(7):1083-1094. doi:10.1002/acn3.51791Koshorek J, Wang Y, Maldonado DP, et al.The many faces of gastrointestinal dysfunction in stiff person syndrome spectrum disorders.Front Neurol. 2023;14:1273256. doi:10.3389/fneur.2023.1273256Ortiz JF, Ghani MR, Morillo Cox Á, Tambo W, Bashir F, Wirth M, Moya G.Stiff-person syndrome: a treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995Dalakas MC.Therapies in stiff-person syndrome: advances and future prospects based on disease pathophysiology.Neurol Neuroimmunol Neuroinflamm. 2023;10(3):e200109. doi:10.1212/NXI.0000000000200109
Bheemavarapu B, Singh A, Ranjini NJ, Abhilash Meda VS, Patil D.Recurrent acute on chronic respiratory failure in stiff person syndrome.Case Rep Neurol. 2023;15(1):187-191. doi:10.1159/000532093
National Institute of Neurological Diseases and Stroke.Stiff person syndrome.
Chia NH, McKeon A, Dalakas MC, et al.Stiff person spectrum disorder diagnosis, misdiagnosis, and suggested diagnostic criteria.Ann Clin Transl Neurol. 2023;10(7):1083-1094. doi:10.1002/acn3.51791
Koshorek J, Wang Y, Maldonado DP, et al.The many faces of gastrointestinal dysfunction in stiff person syndrome spectrum disorders.Front Neurol. 2023;14:1273256. doi:10.3389/fneur.2023.1273256
Ortiz JF, Ghani MR, Morillo Cox Á, Tambo W, Bashir F, Wirth M, Moya G.Stiff-person syndrome: a treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995
Dalakas MC.Therapies in stiff-person syndrome: advances and future prospects based on disease pathophysiology.Neurol Neuroimmunol Neuroinflamm. 2023;10(3):e200109. doi:10.1212/NXI.0000000000200109
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