W
hen you think of stiff muscles, you might be thinking of a situation following an intense workout or overdoing it on the dance floor. But for people with Stiff Person Syndrome (SPS), it’s a daily struggle to move even the smallest of muscles. SPS is a rare neurological disorder that causes muscle stiffness and spasms and can make everyday tasks a challenge. Imagine the surprise and awareness raised when our all-time favorite Canadian singer, Celine Dion announced a break from all tours till 2024 following her diagnosis with the condition that affects about one in one million people (mostly women).
What is Stiff Person Syndrome?
Stiff Person Syndrome is a neurological disorder that causes muscles to stiffen and spasm uncontrollably. It can affect any muscle in the body, but it’s most common in the arms, legs, and back. The stiffness can be mild or severe, and it can come and go in episodes. SPS is thought to be caused by a problem with the way the brain and spinal cord communicate. While the exact cause of SPS is not fully understood, there are several theories about what may trigger it.
One theory is that SPS could be caused by a problem with the neurotransmitter GABA (gamma-aminobutyric acid). GABA is a chemical that helps control muscle movement and prevent muscle spasms. In people with SPS, GABA may not be working properly, causing the muscles to become stiff and spastic. Another theory is SPS may be caused by an autoimmune disorder, where the body’s immune system attacks the cells that make GABA. A third theory is that SPS could be caused by a malfunction in the central nervous system. This could be due to various factors, including inflammation or damage to the brain or spinal cord.
One specific area of the brain that may be involved is the cerebellum, which is responsible for controlling muscle movement and coordination. In some people with SPS, the cerebellum may be overactive, causing the muscles to become stiff and spastic. Other areas of the brain, such as the basal ganglia and thalamus, may also be involved.
What are the symptoms of Stiff Person Syndrome?
The symptoms of SPS can vary from person to person and range from mild to severe, but some of the most common symptoms include:
- Muscle stiffness, especially in the neck, back, and arms.
- Tremors or shaking of the arms or legs.
- Problems with balance and coordination.
- Pain in the muscles or joints.
- Excessive sweating.
- Difficulty swallowing.
- Difficulty speaking.
- Emotional changes, such as anxiety or depression.
Types of SPS
- Classic Stiff Person Syndrome: This type of SPS is also called pure SPS as it’s not associated with any other medical conditions. It usually begins with stiffness in the neck, back, or arms, but it can spread to other parts of the body over time. The stiffness can come and go, but it’s often worse when a person is stressed or tired. The stiffness can be severe enough to interfere with daily activities, like walking and dressing. Some people with classic SPS also experience muscle cramps and weakness.
- Stiff Limb Syndrome: Stiff Limb Syndrome (SLS) is a rarer form of SPS characterized by stiffness and spasms in one or more limbs. It can affect any limb, but it’s most commonly seen in the legs. The symptoms of SLS are similar to classic SPS, but they’re usually limited to the affected limb. In some cases, the stiffness can spread to other parts of the body but this is less common. The symptoms of SLS can be very disabling, and they can interfere with daily activities. Treatment for SLS typically involves medications to help control stiffness and spasms.
- Other types of SPS: There are a few rarer forms of SPS, including paraneoplastic SPS and familial SPS. Paraneoplastic SPS is a form of SPS associated with certain types of cancer. On the other hand, Familial SPS is a genetic form of SPS that can run in families. These are both rare forms of SPS, and they can be difficult to diagnose.
Treatment of SPS
There are several treatment options available for people with SPS. The goal of treatment is to reduce the symptoms and improve the quality of life. These treatments include:
- Medication: The most common treatment for Stiff Person Syndrome is medication, and the main types of medication used are muscle relaxants, anticonvulsants, and botulinum toxin injections. Muscle relaxants work by blocking the signals that cause the muscles to spasm. Anticonvulsants help reduce muscle stiffness and pain. Botulinum toxin injections are used temporarily to relax the muscles. These treatments can be effective but they may cause side effects like drowsiness, dizziness, nausea, and confusion. Some people may also experience a dry mouth, blurred vision, or rash. If a person experiences any of these side effects, they should speak to their doctor. The doctor may need to adjust the dose of the medication or try a different medication.
- Therapy: Physical therapy can help stretch and strengthen the muscles, and improve balance and coordination. Occupational therapy can help with daily activities. Assistive devices, such as walkers and canes, can help patients stay mobile.
- Surgery: This may also be an option for some people with SPS. Surgery is typically only considered if medications aren’t working well or if the symptoms are severe. Surgery can include procedures to relieve pressure on the spinal cord or nerves.
Featured image: @celinedion/Instagram
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