What is this Stiff Person Syndrome that Celine Dion has?

Dion performing on the opening night of her world tour ‘Courage’ in Quebec City, Canada, in September 2019. The songstress had to cancel her remaining tour shows for 2023 and 2024 due to being diagnosed with Stiff Person Syndrome. — AFP

One of my favourite songs of all time is the Titanic song My Heart Will Go On sung by Celine Dion. I grew up practically singing it every day. So, I was very sad to hear that she has Stiff Person Syndrome. This is the first time I have heard of this disease. What is it?

Most people have not heard of Stiff Person Syndrome because it is very rare.

It is an autoimmune disorder that affects your neurological system.

It has a wide plethora of symptoms, depending on how severe the disorder is.

It mostly develops in people aged 40 to 50, but it can also occur in children and elderly people.

It is so rare that it only affects one or two persons out of a million.

It is called a syndrome because it is a spectrum of disorders, rather than just one disorder.

And it occurs more commonly in women than men, as do other autoimmune disorders.

What do you mean by autoimmune? Is it something to do with my immune system?

Yes. When your immune system is acting normally, it will defend your body against infections and diseases.

However, if it is not functioning properly, it will mistake your own healthy tissues, organs and cells for a foreign invader.

And it will start attacking them as though they were bacteria or viruses or tumour cells.

When this occurs, it is called an autoimmune disease.

It can affect any part of your body, and it has different names, depending on where and how they attack your body.

An autoimmune disease can be every bit as debilitating and dangerous as an infection from outside your body.

It can even be life-threatening.

Unfortunately, autoimmune diseases are all associated with one another.

So, if you have Stiff Person Syndrome, you are also more likely to have type 1 diabetes mellitus, thyroid diseases, pernicious anaemia, and even certain types of cancer.

Why does Stiff Person Syndrome occur then?

People with this syndrome have a certain type of antibody called anti-GAD65 in their blood.

These antibodies block the glutamic acid decarboxylase (GAD) enzyme, which helps make the gamma-aminobutyric acid (GABA) neurotransmitters that are essential to our nervous system.

GABA’s function is to reduce or block certain nerve signals.

In Stiff Person Syndrome, when the anti-GAD65 antibodies ultimately block GABA from performing its usual functions, the nervous system becomes easily excited.

This then leads to the physical symptoms of Stiff Person Syndrome.

What exactly are the symptoms of this syndrome?

Patients have painful muscle contractions and spasms that often begin in the legs, back, abdomen, upper trunk, arms, neck and face – in order of the most common areas affected.

These spasms can happen episodically, especially when the patient is surprised or startled, or when she moves suddenly.

The symptoms can also be triggered by the cold, stress, emotional outbursts, some exercise, or even touching.

Therefore, you must not creep up on a patient with Stiff Person Syndrome or have any sort of argument with her!

Once triggered, the muscle contractions will occur.

The symptoms depend on the part of the patient’s body that is triggered.

Once contracted, the muscles will become stiff and board-like.

If the legs are triggered, the contractions may cause difficulty in walking and standing.

The person may even fall down due to imbalance.

If the back or trunk is triggered, the person may develop a stiff and rigid posture.

Over time, there can be an exaggerated curve in the patient’s lower back due to continued stiffness and changes in spinal alignment that can cause cord compression.

If the chest is triggered, there can be shortness of breath.

There can also generally be pain due to so much stiffness.

The symptoms don’t usually appear in any specific order, and seem to be different from one patient to the next.

They can also gradually worsen over months and years.

The less common types of Stiff Person Syndrome can give you double vision, slurred speech and lack of coordination.

Celine Dion’s family has stated that there is no cure. Is this true?

Unfortunately, yes.

But there is treatment to help patients live and cope with the disease.

There are medications to help with the muscle contractions and to reduce stiffness, such as muscle relaxants, and drugs that affect the GABA neurotransmitter pathway.

Botox is sometimes used when there is too much tightness.

Immunosuppressants can also be used to help suppress the immune system from attacking the tissues.

A lot of physical therapy is required to help with the stiffness and spasms.

Most of all, there is a lot of avoidance for patients and loved ones, i.e. avoiding the triggers that cause these episodes.

If managed well, a patient with Stiff Person Syndrome can live a long time and as close to normal as possible.

Dr YLM graduated as a medical doctor, and has been writing for many years on various subjects such as medicine, health, computers and entertainment. For further information, email [e-mail]. The information provided is for educational and communication purposes only, and it should not be construed as personal medical advice. Neither The Star nor the author gives any warranty on accuracy, completeness, functionality, usefulness or other assurances as to such information. The Star and the author disclaim all responsibility for any losses, damage to property or personal injury suffered directly or indirectly from reliance on such information.