Since singer Celine Dion announced she had Stiff Person Syndrome in December 2022, interest in the rare disease has grown.

Stiff person syndrome is a rare autoimmune neurological disorder that often causes muscle stiffness and painful spasms that come and go away and can worsen over time.

The disease was previously known as "stiff man syndrome", but its name has changed to "stiff person syndrome" as it is more common in women.

Causes of Stiff Person Syndrome

According to Johns Hopkins University, stiff person syndrome is an autoimmune disorder in which the body's immune system attacks healthy tissue via antibodies that work negatively affecting the production of a neurotransmitter called GABA.

GABA usually reduces or blocks certain nerve signals, but being disrupted causes neurons to function incorrectly, as the nervous system becomes highly excitable and causes physical symptoms such as muscle spasms as well as psychological symptoms, including anxiety.

Which age groups develop stiff person syndrome?

People between the ages of 40 and 50 usually develop "stiff person syndrome," but in rare cases, it occurs in children and the elderly.

"Stiff person syndrome" is thought to affect one or two people in a million.

Symptoms of "stiff person syndrome"

"Stiff person syndrome" causes painful muscle spasms and spasms that often begin in the legs and back. Cramps can also affect the abdomen, more often the upper torso, arms, neck and face.

Areas where muscle contractions occur can become stiff and lath-like. Depending on the affected part of the body, contractions can cause:

  • Difficulty walking
  • Stiffness due to constant cramps in the back or trunk
  • Instability and falls due to sudden convulsions that can lead to injuries
  • Shortness of breath if "stiff person syndrome" affects the chest muscles
  • Chronic pain
  • Changes in the spine leading to spinal cord compression (myelopathy)
  • Anxiety and agoraphobia due to falls caused by convulsions
  • Eye movement problems that cause double vision
  • Speech problems

Types of "stiff person syndrome"

  • Classic Stiff Person Syndrome

Most people with "stiff person syndrome" have the classic type. Symptoms include stiffness and cramps in the muscles of the lower back, legs (more than arms) and sometimes in the abdomen. Patients may experience frequent muscle cramps and can experience pain most of the day.

  • Partial Stiff Person Syndrome

This type is less common, and in which spasms and tight muscles are limited to a specific area, usually one leg.

  • Stiff Person Syndrome Plus

This type is a combination of traditional characteristics such as convulsions and stiffness, with symptoms indicating problems with the brainstem or weakness of the cerebellum.

"Stiff person syndrome" causes painful muscle spasms and spasms that often begin in the legs and back (German)

Treatment of "stiff person syndrome"

There's no cure for stiff person syndrome for now, but available medications may help reduce the severity of symptoms and improve quality of life:

  • Muscle relaxants
  • Drugs that affect the neurotransmitter GABA
  • Botulinum toxin, which is sometimes used in areas with frequent cramps
  • Serotonin reuptake inhibitors (SSRIs), to address physical symptoms caused by anxiety
  • Immunosuppressants

Living with "stiff person syndrome"

"Stiff person syndrome" affects patients in varying ways, with the type and severity of symptoms and how quickly they worsen from person to person. Patients can manage their symptoms by working with a specialist doctor and adhering to a treatment plan.

Does it require lifelong treatment?

Leaving treatment may cause more severe symptoms such as convulsions that limit movement, increase the risk of falling with injuries and reduce quality of life. Currently, treatment for stiff person syndrome treats the symptoms, but doesn't eliminate the cause.

Is "stiff person syndrome" fatal?

Death from "stiff person syndrome" is rare and usually not caused by the disease itself, but by complications such as blood clots or wound infections due to immobility. In some severe cases, chest muscle spasms can affect a person's ability to breathe.

Stiff person syndrome and Celine Dion

At the end of May 2023, Canadian singer Celine Dion said that "stiff person syndrome" still affects her ability to perform her concerts, and announced the cancellation of her world tour, according to dpa news agency.

In December 2022, Dion postponed several dates for her European tour and spring 2023 concerts after revealing she had been diagnosed with stiff person syndrome.

Dion, 55, a five-time Grammy winner, revealed her diagnosis on social media in a video statement in French and English.