Recently, “My Heart Will Go On” songstress Celine Dion had to cancel her remaining shows for this year and next due to poor health, following her revelation in 2022 that she suffers from stiff-person syndrome (SPS).
As this is a rare condition that affects only a small amount of the global population – estimated to be between one and two million patients) – not many people would know much about SPS. So, what is it?
SPS is essentially a rare autoimmune neurological disorder characterised by progressive muscle spasms and stiffness. It mainly affects the brain and spinal cord, which control all voluntary muscle movements, and ultimately decreases one’s quality of life as it interferes with daily functioning.
The disease typically affects the back muscles, specifically the lower back and upper legs. It has also been reported that SPS affects more females than males, primarily between the ages of 30 and 60, and can remain stable in some cases or gradually get worse if not treated.
The causes of SPS remain unknown, although scientists speculate it is an autoimmune condition where antibodies attack proteins in the brain that control muscle movements. Symptoms, which may take months to several years to develop, include:
- limb stiffness;
- stiff torso muscles;
- a hunchback because of rigid back muscles;
- painful muscle spasms;
- walking limitations;
- sensory issues such as sensitivity to light and sound.
These symptoms also fall on a spectrum from mild to moderate. For some patients, spasms can be so strong that they may cause them to lose balance and fall; while for others, it could just be a muscle twitch.
The final stages of SPS may find sufferers’ facial muscles affected, along with their jaw and/or vocal cord, which is possibly what is happening with Dion. This can be serious as breathing-related muscles, too, may be compromised.
As the symptoms of SPS are very similar to other muscular diseases such as Parkinson’s, multiple sclerosis, and muscular dystrophies, your doctor may run a few tests to rule out these conditions.
Blood tests are carried out to check for the presence of glutamic acid decarboxylase antibodies, the levels of which are generally higher in SPS sufferers; while an electromyography (EMG) is also done to measure electrical activity in the muscles.
To date, there is yet to be an established treatment for SPS patients. Fortunately, symptomatic relief is available to enhance comfort and mobility. These include muscle relaxants, anti-seizure medications, benzodiazepines (e.g. Clonazepam, Diazepam), and/or steroids for inflammation.
Options such as physical therapy, massages, heat therapy, and acupuncture can also be considered to minimise spasms and improve one’s quality of life.
This article was written by DOC2US, a mobile application that allows you to talk to a doctor or any healthcare professionals via text chat at any time and from anywhere.