Stiff Person Syndrome (SPS) is a very rare, long-term condition that causes painful muscle spasms and debilitating muscle stiffness. Scientists don’t yet fully understand stiff person syndrome, but it is thought to be an autoimmune disorder – where your immune system attacks healthy cells – that involves the messages sent between your brain and spinal cord.
The people most likely to suffer from stiff person syndrome are:
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Born female.
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Between the ages of 30 and 60 – although it can also affect children and the elderly.
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People who have another autoimmune disease – such as type 1 diabetes, vitiligo, or celiac disease.
This condition is so rare that it is hard to know how many people suffer from it. It is thought to affect 1-2 people per million worldwide.
Stiff Person Syndrome was originally called Stiff Man Syndrome, until the name was updated to reflect that the disorder can affect people of any age or biological sex. However, women are twice as likely to develop this disorder as men.
When Celine Dion first took to social media to share her diagnosis, she explained that her muscle spasms affected every aspect of her daily life, causing her to struggle with simple activities like walking. Prioritizing her health and recovery, the singer ultimately canceled her ‘Courage World Tour’ to build her strength back up.
The severity of stiff person syndrome varies from person to person, but the two main symptoms are common:
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muscle stiffness – Also called muscle stiffness, which usually affects your back, chest and abdomen and sometimes your arms and legs.
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muscle cramps – Can occur in a specific area or throughout your body, and can last anywhere between a few seconds and, sometimes, a few hours.
Muscle stiffness causes pain that may feel better or worse at different times for no apparent reason. They can also cause pain through associated complications, such as developing abnormal posture.
Muscle spasm causes severe pain. Unlike harshness, these can have predictable triggers – some can be avoided to some extent, but not all. For this reason, some people with tough guy syndrome develop anxiety.
Muscle Cramp Triggers:
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Unexpected or loud noises – As a result, some people develop agoraphobia, a fear of crowded places or leaving the house.
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Tension.
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Light physical contact.
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Changes in temperature – especially if it gets cold.
Death associated with this neurological disorder is rare. When this occurs, it is not a direct result of the tough guy syndrome, but rather a result of its complications. These complications may include serious injury, wound infection, or blood clots caused by immobility.
Stiff Person Syndrome is a long-term condition, which means it stays with you throughout your life. Although there is no cure, treatment can help reduce symptoms, improve quality of life, slow its progression, and reduce the risk of other health complications.
The first step is to get a diagnosis, but because the condition is so uncommon it is easy to mistake it for other conditions with similar symptoms, such as multiple sclerosis (MS). However, a doctor can confirm stiff person syndrome through a blood test that checks for related antibodies, electromyography (EMG) that tests muscle activity, and a spinal tap that looks for antibodies in your spine.
In an interview with Vogue, the Canadian-born singer, who has sold more than 250 million albums, explains how she manages the condition: “Five days a week I undergo athletic, physical and vocal therapy. I work on my toes, my knees, my calves, my fingers, my singing, my voice.”
There are two main treatment strategies:
symptom management
Living with SPS can be made easier with medications that help soothe painful muscle spasms and relax muscle stiffness. Diazepam is often the first medication given for stiff man syndrome because it can help alter the disturbed signals sent between the brain and muscles.
Some painkillers can also affect these signals and effectively reduce pain in some people. Muscle relaxants, as the name suggests, work by loosening your muscles, which can help treat both muscle stiffness and spasms.
Many treatments can also help people continue with as many daily activities as possible. These include physiotherapy, heat therapy, acupuncture, massage and hydrotherapy.
Disease-modifying treatments – recent advances
There is some evidence that a type of immunotherapy called intravenous immunoglobulin (IVIG) treatment can improve symptoms, although this is not the case for everyone. This involves people with healthy immune systems donating antibodies that are transferred through a needle or catheter into someone with stiff neck syndrome.
in 2022 National Institute of Neurological Disorders and Stroke (NINDS) Conducted a study that showed promising results for this technology. Many participants felt less stiffness, became less sensitive to triggers such as noise, touch and stress, and their balance improved.
A smaller, preliminary study of another treatment was conducted in 2022. Although new research is currently underway, it raises hopes that therapeutic plasma exchange may be a safe new treatment method in the future. Here, a person’s blood plasma is replaced with albumin, a man-made substance that can treat certain diseases.
Stiff person syndrome is not curable, but ongoing research offers hope that this disorder may become more manageable and less debilitating in the future.
