Stiff Person Syndrome Explained

World famous singer, #CelineDion has revealed that she has been suffering from Stiff Person Syndrome, an incurable neurological disorder which has caused her to postpone her 2023 tour.

But what exactly is Stiff Person Syndrome? Read on to learn more!

Affecting only a small amount of the population (1-2 per million), SPS is a rare condition that not many know about. 

Stiff Person Syndrome (SPS)

SPS is essentially a rare autoimmune neurological disorder that is characterized by progressive muscle spasms and stiffness. It mainly affects the brain and spinal cord that controls all of our voluntary muscle movements and can ultimately decrease one’s quality of life as it interferes with our daily functioning. SPS typically affects the muscles at the back, specifically in the lower back as well as the upper legs. It has also been reported that SPS can be seen affecting females more than males from age 30-60, and can remain stable in some cases or may gradually get worse if not treated accordingly. 

What causes SPS?

SPS are triggered by a lot of factors such as sudden movement, cold temperature,  stress, unexpected loud noises or situations that require a heightened emotional response and when that happens, the painful muscle spasms may last for a few minutes up to hours. In terms of what actually causes SPS, the answer still remains unknown. Scientists have however predicted it to be an autoimmune disease where patients with SPS have antibodies that attack proteins in the brain whose function is to control muscle movements.

What are the signs and symptoms of SPS?

As the name suggests, SPS 'most notable indicator is muscle stiffness. Symptoms of SPS may take months to several years to develop. The symptoms are: 

• Limb stiffness

• Stiff muscle at the trunk 

• Developing hunched back due to posture problems from rigid back muscles

• Painful muscle spasms

• Walking limitation

• Sensory issues such as sensitivity to light, noise and sound
   

Just like most diseases, symptoms of SPS also fall on a spectrum from mild to moderate. For some, SPS spasms can be so strong that may cause them to fall due to the muscle spasms that cause them to lose the ability to catch themselves while for others it's just a sudden muscle twitch.

In the final stages of SPS, you may also find your facial muscles to be affected by SPS together with your jaw and/or vocal cord. This can be serious as breathing-related muscles may be compromised and affect our breathing.
 

How do I know if I have SPS?
Because the symptoms of SPS are very similar to other muscular diseases such as Parkinson’s disease, multiple sclerosis and muscular dystrophies, your doctor may run a few tests to rule out these conditions and to verify if you’re actually suffering from SPS.

Tests to confirm SPS are:

• Blood tests to test for presence of glutamic acid decarboxylase (GAD) antibodies. People who have SPS will generally have higher levels of GAD antibodies

• Electromyography (EMG) done to measure electrical activity in your muscles to look for continuous motor activity in the muscles
   

Treatment for SPS

To date, there is yet to be an established treatment for patients with SPS since researchers are still investigating the cause of SPS. However treatments as symptomatic relief are available with the goal of treatment is to manage symptoms adequately and enhance mobility along with comfort. These may include: 

• Muscle relaxants (ie: Baclofen)

• Benzodiazepines (ie: Clonazepam, Diazepam etc) 

• Anti-seizure medications (ie: Gabapentin) that may reduce pain

• Steroids if have inflammation
   

All in All

If you or someone you know has SLS, fret not as you are not alone. While there is no existing treatment targeting SLS, non-pharmacological treatment works for some too. Options such as physical therapy, massage, heat therapy, acupuncture etc can be considered to minimise the spasms and as a result improve your quality of life.

This article is written by Janelle Leong, Bpharm(Hons)(DOC2US), 
reviewed by Dr. Ahmad Haniffan, MBBS​ (DOC2US)​

References

1. Stiff Person Syndrome - Yale University. Available at: https://www.yalemedicine.org/conditions/stiff-person-syndrome. Accessed on 11th December 2022

2. Stiff Person Syndrome: Symptoms, Causes, and Treatment - Healthline. Available at: https://www.healthline.com/health/stiff-person-syndrome#types. Accessed on 11th December 2022

3. SLS - Cleveland Clinic. Available at: https://my.clevelandclinic.org/health/articles/6076-stiff-person-syndrome. Accessed on 11th December 2022

4. https://www.usatoday.com/story/life/health-wellness/2022/12/08/celine-dion-stiff-person-syndrome-diagnosis-explained/10857523002/. Accessed on 11th December 2022

Written By

Janelle Leong, Bpharm(Hons)

Reviewed By

Doc2us Medical Board