What is stiff person syndrome — the incurable disease Celine Dion is battling?

Celine Dion, the ace Canadian singer, recently revealed she was diagnosed with stiff person syndrome (SPS).

The 54-year-old music star also disclosed that due to her health condition, she won’t be able to perform at her UK and Europe shows as planned next year.

Dion’s revelation has left a huge number of her fans worried about her wellness.

SPS, also called Moersch-Woltman syndrome, is an extremely rare disorder that affects one in a million people, according to Pavan Tankha, the medical director of comprehensive pain recovery at Cleveland Clinic.

Research shows that it affects twice as many women as men — indicating it affects more women.

What is stiff person syndrome (SPS)?

SPS is a rare, progressive syndrome that affects the nervous system, specifically the brain and spinal cord.

It causes muscle stiffness, rigidity and sometimes intense muscle spasms in the trunk and limbs which eventually affect posture, balance and the ability to use certain muscles.

These painful muscle spasms occur randomly or are triggered by noise, emotional distress and light physical touch.

“It frequently is associated with muscle spasms, which could be quite severe. These can cause falls, severe pain and significant disability,” said Emile Sami Moukheiber, a doctor at the Stiff Person Syndrome Centre in Johns Hopkins Medicine.

“Falls from severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather.”

What causes SPS?

The exact cause of SPS remains unknown. According to the National Institute of Neurological Disorders and Stroke, SPS is a “rare neurological disorder with features of an autoimmune disease.”

Researchers believe the disorder may be caused by an autoimmune reaction — when the body attacks its own nerve cells that control muscle movement.

It also said that “people with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain.”

SPS is more likely to affect people suffering from:

• Autoimmune disorders including diabetes, thyroiditis, vitiligo and pernicious anemia.
• Certain cancers including breast, lung, kidney, thyroid and colon.

What are the symptoms of Stiff Person Syndrome?

The symptoms of SPS typically develop between the ages of 20 and 60 years and the condition usually affects people during their 30s and 40s.

These symptoms include:

• Stiffness and rigidity in the trunk muscles
• Difficulty turning and bending
• Rigidity in the upper and lower limbs
• An irregular hunched posture
• Stiff gait and difficulty walking
• Painful muscle spasms
• Falls
• Exaggerated sudden responses to stimuli such as noise or emotions

People with SPS tend to also experience mental symptoms due to social and physical limitations, and anticipation of spasms.

These symptoms include:

• Depression
• Anxiety
• A fear of open spaces and participation in certain tasks that may trigger spasms

How can Stiff Person Syndrome be treated? 

SPS is not curable. However, there are treatments to control the symptoms and manage the pain.

The treatment depends on the symptoms noticed and their severity. The goal of the treatment is to manage symptoms and improve mobility and comfort.

• Anti-depressant and anti-epileptic to treat seizures
• Benzodiazepines; drugs to treat muscle stiffness and spasms
• Physical therapy to improve muscle tone and posture
• Disease-modifying therapies change the immune response and reduce or remove the harmful antibodies that are attacking healthy cells.
• Psychotherapy