Celine Dion, the legendary Canadian singer, recently revealed that she has stiff person syndrome (SPS).
The 54-year-old singer also revealed that she will be unable to perform at her planned UK and European shows next year due to her health.
Many of Dion’s fans are concerned about her health as a result of her revelation.
According to Pavan Tankha, the medical director of comprehensive pain recovery at Cleveland Clinic, SPS, also known as Moersch-Woltman syndrome, is an extremely rare disorder that affects one in a million people.
According to research, it affects twice as many women as men, indicating that it affects more women.
What is stiff person syndrome (SPS)?
SPS is a rare, progressive neurological disorder that affects the brain and spinal cord.
It causes muscle stiffness, rigidity, and occasionally intense muscle spasms in the trunk and limbs, affecting posture, balance, and the ability to use specific muscles.
These excruciating muscle spasms occur at random or are triggered by noise, emotional distress, or light physical touch.
“It is frequently associated with muscle spasms, which can be severe. “These can result in falls, severe pain, and significant disability,” said Emile Sami Moukheiber, a doctor at Johns Hopkins Medicine’s Stiff Person Syndrome Centre.
“Falls caused by severe spasms are extremely common. These spasms can be triggered by startle, strong emotions, or cold weather.”
What causes SPS?
The precise cause of SPS is unknown. SPS is a “rare neurological disorder with autoimmune features,” according to the National Institute of Neurological Disorders and Stroke.
The disorder, according to researchers, could be caused by an autoimmune reaction, in which the body attacks its own nerve cells that control muscle movement.
“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,” according to the study.
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