Stiff Person Syndrome: Causes, Symptoms,Treatment
Stiff Person Syndrome (SPS) is a rare autoimmune movement disorder that affects the central nervous system. It causes severe muscle stiffness, rigidity, and painful spasms in the trunk.
It starts with the muscles in your trunk and abdomen. Muscle spasms and stiffness may come and go in the early stages. However, after a while, the stiffness becomes continuous.
It stiffens your leg muscles over time, followed by other muscles such as your arms and even your face. The treatment options aim to ease your symptoms while also improving your comfort.
Researchers are still unable to pinpoint the exact source of this disorder. However, they suspect that it is the outcome of an autoimmune reaction. The immune system attacks the central nervous system, which controls muscular movement. As a result, a person suffers from this condition.
What is Stiff Person Syndrome?
Stiff person syndrome (SPS) is a fairly rare autoimmune neurological disorder. It is also known as Moersch-Woltman syndrome. It was previously known as stiff man syndrome.
SPS affects the brain and spinal cord. The muscles of the trunk tighten first in people with SPS. Over time, patients develop stiffness and rigidity in their legs and other muscles. SPS can sometimes induce painful muscle spasms.
The good news is that SPS is a rare disease. SPS affects only one in a million people. As a result, your chances of getting affected are low. However, if left untreated, it can seriously impair your quality of life.
What Causes Stiff Person Syndrome?
You don’t have to study neurology to understand the root cause. Let us break it down for you.
Researchers are not sure of the exact reason that causes stiff-person syndrome. But medical research suggests that it may be a result of an abnormal autoimmune neurological disorder. It’s a disease in which your immune system targets healthy cells.
Many persons with this condition produce some particular antibodies. They target an enzyme called glutamic acid decarboxylase (GAD). GAD is a protein found in some neurons. It helps to produce gamma-aminobutyric acid (GABA). GABA is in charge of controlling muscular movement.
Scientists believe that persons with SPS have an immune system that wrongly attacks the GAD enzyme. This reduces GABA levels in the body.
According to medical research, some people with SPS have antibodies to amphiphysin. Amphiphysin is a protein involved in transmitting signals from one neuron to another.
According to clinical trials, individuals with these antibodies may develop some cancers. They include:
- Breast cancer.
- Lung cancer.
- Colon cancer.
How exactly GAD plays a role in the development of stiff person syndrome is still unknown.
As a matter of fact, some people with SPS do not have anti-GAD antibodies.
Risk Factors Associated with SPS
It is unknown what causes stiff-person syndrome. The autoimmune disorder, however, is extremely rare. This syndrome affects around one out of every million persons.
Furthermore, women are twice as likely as males to suffer from SPS. People between the ages of 30 and 60 are the most likely to develop it.
Conditions with Increased Risk of Developing SPS
- Rheumatoid arthritis.
- Pernicious anemia.
- Type 1 and 2 diabetes.
Symptoms of Stiff Person Syndrome
Symptoms of stiff person syndrome might appear anywhere from a few months to a few years. Some patients remain stable for years, while others slowly worsen.
The trunk and belly muscles are the first to stiffen and expand in most patients.
Other related symptoms include:
- Extremely painful muscle spasms.
- Stiff muscles in the trunk.
- Sensitivity to light, noise, and sound.
- Posture problems. You may hunch over.
- Difficulties in walking.
- Limbs become stiff.
- Aggressive sweating.
Muscle spasms can be extremely painful. They might even cause you to fall when you’re standing. If you are upset, your spasms will be more painful. Sudden movements, loud noises, and other factors might trigger spasms.
You may notice greater stiffness and rigidity in the later stages. Muscle stiffness can spread to other parts of your body, including the face.
This includes the muscles used for eating and speaking. Also, there may be damage to your breathing muscles.
This results in possibly deadly breathing issues.
Stiff-Person Syndrome may Trigger other Diseases.
- Several cancers including colon, lung, breast.
- Thyroid problems.
- Diabetes mellitus.
- Pernicious anemia.
Diagnosis of Stiff Person Syndrome
Your doctor will perform a physical exam and a review of your medical history. This way, he can determine if you have SPS. If your doctor suspects SPS, tests to confirm the diagnosis may include:
A blood test determines the presence of glutamic acid decarboxylase (GAD) antibodies. A blood test can detect antibodies against GAD in 60-80 percent of people with SPS. But, the absence of GAD antibodies does not rule out SPS. And high levels of GAD antibodies can help to confirm the diagnosis.
It searches for signs that suggest the presence of GAD antibodies. Lumbar puncture also helps in the diagnosis of various diseases.
Your doctor may arrange an electromyography (EMG) test. It can detect muscular electrical activity.
SPS can be diagnosed in conjunction with epilepsy. It is occasionally confused with other autoimmune diseases. They include multiple sclerosis (MS) and Parkinson’s disease.
Dr. Scott D. Newsome, Associate Professor of Neurology, Johns Hopkins School of Medicine, conducted research.
According to the research, SPS presents itself in a wide range of signs and symptoms. As a result, it mimics other diseases, delaying diagnosis.
Treatment of Stiff Person Syndrome
SPS has no known cure. Your symptoms will guide your treatment for stiff person syndrome. The goal of treatment is to ease the symptoms while also improving mobility and comfort.
- Immune modulating therapies
You should think about it as it provides the best result. Intravenous immunoglobulin (IVIG) treatment is one option. Clinical trials have demonstrated that intravenous immunoglobulin (IVIG) is effective.
It is also well-tolerated in terms of relieving the symptoms related to SPS. IVIG is often used as a therapy for immune-mediated diseases, including SPS. IVIG works by reducing the number of antibodies attacking healthy tissues.
- Autologous stem cell transplant
It is the process where your blood and bone marrow cells are first collected. They are then multiplied before being returned to your body. This is an experimental treatment that you can consider if all other options fail.
It is a process in which your blood plasma is exchanged for fresh plasma. This process lessens the number of antibodies in your body.
You can treat muscle spasms and stiffness with one or more of the following medications:
- Pain medications
- Muscle relaxers
- Benzodiazepines are used to relieve anxiety and muscle tension. In addition, high doses of these drugs are used by doctors to treat muscle spasms.
- A muscle relaxer called Baclofen. Doctors use it for people with stiff person syndrome in whom benzodiazepines are not effective. In addition, some SPS-affected people benefit from using Baclofen in addition to Benzodiazepines.
- Tiagabine is an anti-seizure medication.
- Gabapentin is a type of medication that is used to treat nerve pain and seizures.
There are often severe depression and emotional distress associated with SPS. In such a situation, antidepressants may help.
Your doctor may recommend you to a physical therapist in addition to drugs. SPS cannot be treated with physical therapy alone.
However, according to medical research, it may help with drug side effects. Also, the exercises may significantly help with your:
- Emotional well-being.
- Overall day-to-day function.
- Range of motion.
Complications of Stiff Person Syndrome
Stiff person syndrome (SPS) causes muscle spasms. These problems can pretty much hamper your daily life. You may have problems including:
- Frequently falling from standing.
- Severe anxiety and depression.
- Excessive sweating.
- Broken bones.
- Hunching over.
- Emotional distress.
SPS can also increase your risk of having several other conditions. They include:
- Type-I and Type-II diabetes.
- Thyroid problems.
The Prognosis for Stiff Person Syndrome
Because of your lack of stability, you’re more likely to fall if you have this condition. This increases your chances of suffering injuries and maybe becoming permanently disabled.
The prognosis for stiff person syndrome varies according to the individual’s symptoms.
Furthermore, the severity of the syndrome and the rate of decline vary from person to person.
Everyone reacts differently to a treatment. For example, some SPS-affected people respond well to drugs and physical therapy. But others may not respond as well.
Contact your doctor if you get muscle spasms or stiffness in your trunk, arms, or legs. If you have any of the risk factors, talk to your doctor about stiff person syndrome.
You could also call a local university or tertiary medical center. This is because these institutions have the most up-to-date technologies in the field of rare diseases.
You can find other related info at the National Institute of Neurological Disorders and Stroke. National Institute of Neurological Disorders and Stroke collects and verifies all trials related to SPS.
Some FAQ Associated with Stiff Person Syndrome
1. What is the Life Expectancy of Stiff Person Syndrome?
The life expectancy for people with SPS ranges from 6 to 28 years. There are numerous SPS therapies available. The treatment options can reduce your discomfort and provide you with relief. They do not, however, cure the condition. Among these treatments, intravenous immunoglobulin (IVIG) therapy is the most effective. For details about the clinical trials, visit the Stiff Person Syndrome Information Page.
2. Can a Person with Stiff Person Syndrome Have Gluten in Their Diet?
According to medical research, people with SPS are gluten sensitive. So, you should avoid gluten in your diet if you have stiff person syndrome.
3. Is Stiff Man Syndrome Progressive?
Stiff man syndrome, a.k.a stiff person syndrome, is a rare progressive disorder. It causes progressive muscle stiffness, rigidity, and spasms. In the early stage, muscle stiffness may come and go. But, after a while, the stiffness becomes constant.
4. Is Stiff Person Syndrome Terminal?
Stiff person syndrome is a rare terminal condition. There are many treatment options available, but none can cure it. The treatment options aim to ease your symptoms while also improving your comfort.
Over a while, SPS can limit your ability to move or walk. The overall course of SPS can range from 6-28 years from the onset of symptoms until death.
5. Why is My Body So Tight and Stiff?
SPS is an autoimmune disorder that causes muscle stiffness, rigidity, and spasm. In this condition, your body develops an antibody that attacks an enzyme. This enzyme is known as glutamic acid decarboxylase (GAD). GAD helps in the production of a neurotransmitter called gamma-aminobutyric acid (GABA). GABA helps to control muscle movement.
As a result, your body grows increasingly tight and stiff over time.
6. Can Stiff Person Syndrome be Cured?
There is no cure for this rare disease. In addition, doctors can confuse it with other autoimmune diseases. Multiple sclerosis (MS) and Parkinson’s disease are two examples. However, don’t worry. Some treatment options can help you to cope with the symptoms. They can also get you relief. Among them, the best option is intravenous immunoglobulin (IVIG) therapy. IVIG works by decreasing the number of antibodies that attack healthy tissues.
You can get the peer-reviewed clinical trials at the Stiff Person Syndrome Information Page.
7. Is Stiff Person Syndrome a Disability?
Over a while, stiff person syndrome can make you disable. In this condition, the symptoms develop slowly. After a while, the symptoms may become severe, causing disability. Are you planning to get the benefits from SSA? Then please remember that you need to meet SSA’s basic requirements for disability. You must be unable to work at a substantial gainful activity level due to your medical condition.
Furthermore, your condition must have lasted, or be likely to last, for at least 12 months in a row.
8. What Causes a Person to Walk Stiffly?
In stiff person syndrome, over time, leg muscles become stiff. As leg muscles become stiffer and stiffer, people with stiff person syndrome walk stiffly.
9. How do You Get Stiff Man Syndrome?
Scientists are still unclear as to what causes SPS. However, research suggests that it results from a faulty autoimmune response in the brain and spinal cord.
Stiff person syndrome is not a transmissive disease. This means you can’t get this disorder from other people. However, scientists believe that genetics may play a role in causing SPS in combination with other factors.
10. Does Exercise Help Stiff Person Syndrome?
According to medical research, exercise can indeed help stiff-person syndrome.
The report suggested many helpful exercises. They include:
- ROM exercises for stiff areas.
- Lower back exercises.
- Pelvic tilt.
- Isometric abdominal exercises.
The report demonstrated that these exercises are beneficial for:
- Low back pain.
- For improving one’s function and ability to perform day-to-day tasks.
So if you are suffering from SPS, we highly recommend daily exercise.
11. Can Stiff Person Syndrome Kill You?
Stiff limb syndrome, a.k.a stiff person syndrome, can horrifyingly kill you. You could die alone, frozen, and unable to move or call for help.
SPS results from a faulty autoimmune response in the brain and spinal cord. It causes symptoms like severe muscle stiffness, rigidity, and spasms.
There are a variety of therapy methods available, but none of them can completely cure it. SPS might make it difficult to move or walk for a long time. SPS can last anywhere from 6 to 28 years, from when you first notice symptoms to when you die.
12. Can COVID-19 Medicine Tocilizumab Cure SPS?
According to a study, researchers saw improvement in SPS symptoms in a patient by using tocilizumab. However, to confirm this, we need more research.
Dr. Stephanie Shieh, PharmD was born in Kentucky and raised in Southern Los Angeles. She attended the Western University of Health Sciences and has been a practicing pharmacist for 7 years. The most rewarding part of her job is seeing her patients happy – whether it’s helping them understand their medication, catching a drug interaction, or being able to communicate with their doctor to improve their therapy. She is currently precepting students from Marshall B. Ketchum University. In her free time, she enjoys spending quality time with her husband and 2 daughters. They love playing outside, doing arts and crafts, and having family dinner.