What Is Stiff-Person Syndrome (SPS)?

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Table of ContentsView All

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Table of Contents

Symptoms

Types

Causes

Diagnosis

Treatment

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It is a rare condition that only affects one or two persons in every million people.It is estimated that fewer than 5,000 people in the United States live with this condition.

This article explores the types, causes, symptoms, and diagnosis of stiff-person syndrome, as well as some treatment options and coping strategies that may be helpful.

Symptoms of Stiff-Person Syndrome

The main symptoms of stiff-person syndrome are muscle stiffness and muscle spasms.

Muscle Stiffness

The muscles in the torso (chest, back, and abdomen) and sometimes the limbs (arms and legs) become stiff and rigid, which can be painful and uncomfortable.

The stiffness may spread to other areas of the body and either fluctuate in intensity or get progressively worse. Over time, the person may develop a hunched posture, which can make it hard for them to walk and perform other everyday activities.

Muscle Spasms

Muscle stiffness is often alternated with episodes of painful muscle spasms. The muscle spasms typically last a few seconds or minutes, but can sometimes last for a few hours. They may affect specific parts of the body or the entire body. The spasms may be so severe in intensity that they can cause the person to fall down.

Emotional SymptomsIn addition to physical symptoms, people with stiff-person syndrome may also experience emotional symptoms such as depression, anxiety, and phobias.For instance, someone with stiff-person syndrome may developagoraphobia, which is a fear of leaving home or going to open or crowded places. Leaving the house can be scary because a loud sound such as the sound of a car horn can trigger a disabling muscle spasm that may cause them to fall down.

Emotional Symptoms

In addition to physical symptoms, people with stiff-person syndrome may also experience emotional symptoms such as depression, anxiety, and phobias.For instance, someone with stiff-person syndrome may developagoraphobia, which is a fear of leaving home or going to open or crowded places. Leaving the house can be scary because a loud sound such as the sound of a car horn can trigger a disabling muscle spasm that may cause them to fall down.

In addition to physical symptoms, people with stiff-person syndrome may also experience emotional symptoms such as depression, anxiety, and phobias.

For instance, someone with stiff-person syndrome may developagoraphobia, which is a fear of leaving home or going to open or crowded places. Leaving the house can be scary because a loud sound such as the sound of a car horn can trigger a disabling muscle spasm that may cause them to fall down.

Types of Stiff-Person Syndrome

The different types of stiff-person syndrome include:

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Causes of Stiff-Person Syndrome

While there are different types of autoimmune disorders, stiff-person syndrome is a type of autoimmune disorder that causes the muscles to fail to work the way they should.This is challenging because we rely on the strength and support of our muscles for every action we do, including walking, sitting, standing, and typing.

However, the association between GAD antibodies and stiff-person syndrome is still being researched, because some people with GAD antibodies don’t have stiff-person syndrome and some people with stiff-person syndrome don’t have any unusual antibodies. Approximately 60% to 80% of people with stiff-person syndrome have GAD antibodies in their blood and spinal fluid.

These are some of the risk factors that can make a person more likely to develop stiff-person syndrome:

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Diagnosing Stiff-Person Syndrome

Stiff-person syndrome can be difficult to diagnose because it is a rare condition and its symptoms are often mistaken for those of other conditions such as multiple sclerosis, fibromyalgia, orParkinson’s disease.The diagnostic process may therefore involve several tests to confirm the symptoms and rule out other health conditions.

The diagnostic process may include the following tests:

Provisional Diagnosis vs. Differential Diagnosis

Treating Stiff-Person Syndrome

Treatment for stiff-person syndrome may involve medical and non-medical interventions.

Non-Medical Interventions

There are several therapies that help relax the muscles and relieve pain, such as:

Medications

The symptoms of stiff-person syndrome can be managed with medications such as:

Immunomodulating Therapies

Research shows that certain immunomodulating therapies such as plasmapheresis and intravenous immunoglobulin (IVIG) can also help improve the symptoms of stiff-person syndrome.

A Word From Verywell

If you or a loved one have been diagnosed with stiff-person syndrome, you may find it difficult to cope with the symptoms and reality of living with the condition. It can be helpful to visit a mental healthcare provider to process your feelings and develop coping strategies.

You may also benefit from joining asupport groupof people living with stiff-person disorder or other autoimmune conditions, as they can be a source of advice, resources, and a shared sense of understanding.

7 SourcesVerywell Mind 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.National Organization for Rare Disorders.Stiff-person syndrome.Ortiz JF, Ghani MR, Morillo Cox Á, et al.Stiff-person syndrome: A treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995National Institutes of Health.Stiff-person syndrome.Buechner S, Florio I, Capone L.Stiff-person syndrome: A rare neurological disorder, heterogeneous in clinical presentation and not easy to treat.Case Rep Neurol Med. 2015;2015:278065. doi:10.1155/2015/278065National Institute of Neurological Disorders and Stroke.Stiff-person syndrome.Johns Hopkins Medicine.What are common symptoms of autoimmune disease?Yale Medicine.Stiff-person syndrome.

7 Sources

Verywell Mind 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.National Organization for Rare Disorders.Stiff-person syndrome.Ortiz JF, Ghani MR, Morillo Cox Á, et al.Stiff-person syndrome: A treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995National Institutes of Health.Stiff-person syndrome.Buechner S, Florio I, Capone L.Stiff-person syndrome: A rare neurological disorder, heterogeneous in clinical presentation and not easy to treat.Case Rep Neurol Med. 2015;2015:278065. doi:10.1155/2015/278065National Institute of Neurological Disorders and Stroke.Stiff-person syndrome.Johns Hopkins Medicine.What are common symptoms of autoimmune disease?Yale Medicine.Stiff-person syndrome.

Verywell Mind 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.

National Organization for Rare Disorders.Stiff-person syndrome.Ortiz JF, Ghani MR, Morillo Cox Á, et al.Stiff-person syndrome: A treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995National Institutes of Health.Stiff-person syndrome.Buechner S, Florio I, Capone L.Stiff-person syndrome: A rare neurological disorder, heterogeneous in clinical presentation and not easy to treat.Case Rep Neurol Med. 2015;2015:278065. doi:10.1155/2015/278065National Institute of Neurological Disorders and Stroke.Stiff-person syndrome.Johns Hopkins Medicine.What are common symptoms of autoimmune disease?Yale Medicine.Stiff-person syndrome.

National Organization for Rare Disorders.Stiff-person syndrome.

Ortiz JF, Ghani MR, Morillo Cox Á, et al.Stiff-person syndrome: A treatment update and new directions.Cureus. 2020;12(12):e11995. doi:10.7759/cureus.11995

National Institutes of Health.Stiff-person syndrome.

Buechner S, Florio I, Capone L.Stiff-person syndrome: A rare neurological disorder, heterogeneous in clinical presentation and not easy to treat.Case Rep Neurol Med. 2015;2015:278065. doi:10.1155/2015/278065

National Institute of Neurological Disorders and Stroke.Stiff-person syndrome.

Johns Hopkins Medicine.What are common symptoms of autoimmune disease?

Yale Medicine.Stiff-person syndrome.

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