Table of Contents Hide
- What is Stiff Person Syndrome?
- Is There a High Risk for Stiff Person Syndrome in the Elderly?
- What Causes Stiff Person Syndrome?
- What are the Symptoms of Stiff Person Syndrome?
- How do Medical Professionals Determine if a Patient Has Stiff Person Syndrome?
- How is the Condition of the Stiff Person Handled or Treated?
- What are the Complications of Stiff Person Syndrome?
- How can I Prevent Stiff Person Syndrome?
- How bleak (or bright) is the Outlook for Those Afflicted By Stiff Person Syndrome?
- When Is It Time to Visit a Doctor?
- What Should I Inquire About From My Doctor?
Céline Dion, one of my favorite artists of all time, recently announced that she has been diagnosed with Stiff-Person Syndrome, a rare neurological disorder, and will have to reschedule her upcoming 2023 tour dates.
The Canadian singer broke her silence on her health problems in a video, saying they “affect every aspect of my daily life.”
She said, “I’ve had health issues for a long time, and it’s been really difficult for me to face these challenges and to talk about everything that I’ve been going through.” “Recently, I was diagnosed with the stiff-person syndrome, an extremely uncommon neurological disorder that affects maybe one in a million people.”
Painful spasms that can break bones result from the condition, which affects the brain and spinal cord. In order to gain a thorough understanding of this illness, please read the entire article.
What is Stiff Person Syndrome?
Rare autoimmune movement disorder affecting the central nervous system, also known as “stiff person syndrome” (the brain and spinal cord). People with this condition first develop stiffness and rigidity in their trunk muscles, and then it spreads to their legs and other muscles over time.
Muscle spasms can be extremely painful, and stiff person syndrome (also known as Moersch-Woltman syndrome and formerly stiff man syndrome) is a known contributor. Muscle spasms can happen for no apparent reason, or they could be prompted by something as simple as a loud noise, intense emotion, or gentle touch.
Stiff person syndrome is characterized by a gradual change in posture. In extreme cases, you may find it difficult to move around. It may take years of treatment to alleviate symptoms and ensure a satisfactory quality of life for some people who suffer from this condition.
It is believed that stiff person syndrome is one of many diseases with similar symptoms that start in one part of the body and spread to others.
Is There a High Risk for Stiff Person Syndrome in the Elderly?
The condition known as “stiff person syndrome” occurs rarely. About one in every one hundred thousand people has been identified as having this syndrome. Stiff person syndrome affects twice as many women as men. The onset of symptoms is at age 30-60, but it can happen at any time.
People with certain diseases are more likely to exhibit stiff person syndrome.
- Diabetes, thyroiditis, vitiligo, and pernicious anemia are just some of the autoimmune diseases.
- Breast, lung, kidney, thyroid, colon, and Hodgkin’s lymphoma are just some of the cancers that fall under this category.
What Causes Stiff Person Syndrome?
The origin of the stiff person syndrome is still a mystery to scientists. They think it’s an autoimmune disease, in which the body’s immune system mistakenly attacks healthy tissue. Antibodies produced by many people with this condition target a specific enzyme called glutamic acid decarboxylase (GAD). Gamma-aminobutyric acid (GABA) is a neurotransmitter involved in the regulation of muscle contraction. GAD is required for its production. Stiff person syndrome is characterized by a deficiency in the neurotransmitter GABA because the immune system mistakenly attacks the GAD enzyme.
Although occasionally present, amphiphysin antibodies are a separate protein that can cause this syndrome. This protein is localized in nerve endings and plays a role in facilitating communication between neurons.
However, the precise function of GAD in the emergence and progression of stiff person syndrome remains unclear. There are cases of the syndrome where GAD antibodies are not present.
What are the Symptoms of Stiff Person Syndrome?
The onset of stiff person syndrome symptoms may occur months or even years after the initial diagnosis. While the condition of others gradually worsens over time, some patients’ conditions remain stable for years.
This disease is characterized by a gradual but progressive stiffening and enlargement of the muscles of the trunk and abdomen. Intense aches, pain, and stiffness in the muscles are all symptoms. The degree of stiffness may fluctuate at first, but it should settle into a steady state. Muscles become stiff all over as we age, not just in the legs but in the arms, neck, and face as well. Some people have hunched over because their mobility is restricted. Stiffness like this can make it difficult to move around and walk if it becomes severe enough.
Muscle spasms are also painful symptoms. These spasms typically last for a few seconds, but they can persist for minutes or even hours in extreme cases. The spasms can become so intense that the person loses control of their body and falls, breaking bones or dislocating limbs. There is typically an increase in muscle stiffness due to the spasms. Spasms can happen for no apparent reason, or they can be triggered by something like a sudden loud noise, physical touch, extreme cold, or emotionally taxing situation. Muscle spasms can affect the entire body or just a localized region. Spasms tend to lessen overnight.
How do Medical Professionals Determine if a Patient Has Stiff Person Syndrome?
Many medical conditions share symptoms with this disease. These include tetanus, multiple sclerosis, and muscular dystrophy. In order to rule out these possibilities and look for signs of stiff person syndrome, your doctor may order a battery of tests.
Possible diagnostic procedures if your doctor suspects stiff person syndrome are:
- Blood analysis: Antibodies against GAD and amphiphysin are measured in your blood, along with other markers that may help diagnose or rule out other diseases. Antibodies against GAD are present in 60%-80% of people with stiff person syndrome.
- EMG, or electromyography: In order to detect consistent motor activity in the muscles, a machine measures electrical activity in the muscles.
- Lumbar puncture (spinal tap): A lumbar puncture is a procedure in which a needle is used to remove fluid from the spinal canal so that your doctor can test it for antibodies to GAD and other possible diseases.
Also Read: Tips for Staying Hydrated
How is the Condition of the Stiff Person Handled or Treated?
In order to treat this disease effectively, it is necessary to first identify its symptoms. Treatment aims to reduce painful symptoms and increase range of motion and patient comfort.
To alleviate muscle stiffness and spasms, your doctor may prescribe benzodiazepines (like diazepam and clonazepam) or baclofen. Maybe anti-seizure medication can help with the discomfort. In some people, taking anti-inflammatories or corticosteroids occasionally helps with pain flares.
IVIG, plasmapheresis, rituximab, and autologous stem cell transplant are additional treatment options. The best treatment plan for your unique set of symptoms will be developed in collaboration with your healthcare provider.
Physical therapy, massage, water therapy, heat therapy, acupuncture, and other similar treatments can be effective (when used in conjunction with medication).
What are the Complications of Stiff Person Syndrome?
Muscle spasms and inability to move are hallmark symptoms of stiff person syndrome. Some of the complications that can result from these problems are:
- Depression and anxiety.
- Muscle spasms are so intense that bones dislocate or break.
- Continual tumbling.
- Excessive perspiration (hyperhidrosis).
How can I Prevent Stiff Person Syndrome?
The root of the stiff person syndrome is a mystery to medical researchers. Currently, we have no idea how to stop it.
How bleak (or bright) is the Outlook for Those Afflicted By Stiff Person Syndrome?
The outlook for people with stiff person syndrome varies from case to case. The symptoms and rate of decline associated with this syndrome are highly individual.
Walking can become increasingly difficult as we age. As one age, they may also lose the capacity to carry out simple, routine tasks. Some patients may find relief from their symptoms after undergoing one of several potential treatments. As the disorder progresses, the elevated risk of falling becomes increasingly worrying. It’s possible that some people will require the use of a mobility aid such as a cane, walker, or wheelchair.
When Is It Time to Visit a Doctor?
If you are experiencing spasms or stiffness in your back, neck, shoulders, arms, or legs, it is important to contact your doctor immediately. Ask your doctor about stiff person syndrome if you have any of the risk factors, especially an autoimmune condition.
What Should I Inquire About From My Doctor?
Inquire about the following if you suffer from stiff person syndrome:
- Based on my symptoms, what medication would be most effective?
- What warning signs should I be aware of in case of complications?
- What can I anticipate for my health in the future?