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Celine Dion Battles Stiff-Person Syndrome

Celine Dion Battles Stiff-Person Syndrome

by Dr. Trupti Shirole on Dec 20 2022 4:23 PM
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Highlights:
  • In Stiff-person Syndrome, the body incorrectly attacks its nerve cells, resulting in severe rigidity
  • Patients lose control of their motion and may experience scary muscle spasms
  • Singer Celine Dion informed fans that she would have to cancel her forthcoming February tour as she has been diagnosed with the rare autoimmune condition
Celebrity singer Celine Dion canceled and rescheduled a series of concerts after revealing in an emotional video that she had Stiff-person Syndrome. It is a very rare neurological condition that affects her singing. In a five-minute video released on Instagram in French and English, an emotional Celine Dion revealed she has been suffering from health concerns for a long time.
“I’ve been dealing with problems with my health for a long time, and it’s been really difficult for me to face these challenges and to talk about everything that I’ve been going through…it hurts me to tell you that I won’t be ready to restart my tour in Europe in February,” Celine Dion said (1 Trusted Source
Stiff-person Syndrome: What is Celine Dion's 'human statue' illness

Go to source
).
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What is Stiff-Person Syndrome

Stiff-person Syndrome (SPS) or Moersch-Woltman syndrome (MWS) is a rare autoimmune movement condition. It affects the central nervous system and affects roughly one in every million people. It is diagnosed in middle-aged people and causes chronic pain and spasms, which are usually triggered by noise, emotional distress, and light physical touch, as the patient, can experience muscle stiffening near the trunk and abdomen area, as well as stiffness around the legs and other muscles of the body.

The disorder causes an altered posture over time, and in severe cases, it can potentially impede a person’s ability to walk or move.

Stiff-person Syndrome is more common in people who have conditions, such as
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Types of Stiff-person Syndrome

  • Classical Person Man Syndrome:

    When rigidity and spasms occur in the back and stomach, as well as the thighs and neck on occasion. Over time, it might develop back curvature.
  • Stiff-limb Syndrome:

    Spasms mostly affects the legs and feet, occasionally causing them to become stuck in position. Hands can be impacted as well.
  • Jerking Stiff-person Syndrome:

    The rarest and most aggressive variant, which incorporates symptoms from both the others and affects the head and eyes (3 Trusted Source
    Stiff Person Syndrome-Rare Disease Database

    Go to source
    ).

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Cause of Stiff-person Syndrome

The specific cause of this illness is unknown, although it is thought that the condition emerges when the immune system destroys healthy cells over time.

Signs and Symptoms of Stiff-person Syndrome

It can take months or years for symptoms to appear. While some patients remain stable over time, others can deteriorate. Among the symptoms include muscle stiffness between the trunk and belly, stooped posture, painful muscle spasms, and difficult sleep (2 Trusted Source
Stiff-Person Syndrome

Go to source
).

Diagnosis of Stiff-person Syndrome

Because of its rarity and perplexing symptoms, which are sometimes misdiagnosed as Parkinson’s disease or multiple sclerosis (MS), diagnosing the syndrome can be difficult. However, if doctors suspect Stiff-person Syndrome, two tests can prove it.

The first scans the blood for antibodies to the previously stated protein, glutamic acid decarboxylase (GAD). High levels of GAD antibodies signal the possibility of Stiff-person Syndrome, while levels are also elevated in persons with type 1 diabetes.

The second test is an electromyogram (EMG), which evaluates muscle and motor neuron health. Doctors inject a needle directly into the afflicted muscles to record the electrical activity.

Treatment of Stiff-person Syndrome

Unfortunately, doctors cannot reverse or treat chronic illnesses. Nor can it be prevented. Treatments can, however, be offered to most individuals to assist reduce symptoms.

Diazepam and baclofen, both of which suppress spasms, can help regulate episodes and minimize stiffness. Some patients with more severe symptoms use therapy to modify their immune systems to increase y-Aminobutyric Acid (GABA) levels. In some circumstances, immunoglobin infusions can be used to modify antibody levels in the blood. Sedatives and steroids, among other medications, may be administered.

Meanwhile, patients are frequently offered physical and aquatic therapy to improve the function of their muscles.

Complications or Stiff-person Syndrome

Stiff-person Syndrome is characterized by restricted movement and muscle spasms.

These concerns can result in complications such as
  • Depression and anxiety
  • Broken or dislocated bones due to severe muscle spasms
  • Frequent falls
  • Sweating excessively (hyperhidrosis) (4 Trusted Source
    Stiff person syndrome (SPS)

    Go to source
    )

Prognosis of Stiff-person Syndrome

The prognosis for stiff person syndrome varies according to the individual’s symptoms. The severity of the syndrome and the rate at which it progresses differ from person to person.

Walking can grow increasingly difficult over time. Furthermore, a person’s ability to do regular, ordinary duties may deteriorate over time. Various treatments may help some patients reduce their symptoms. As the disease progresses, the increased danger of falling becomes a significant concern. Some persons may require the aid of a cane, walker, or wheelchair.

References:
  1. Stiff-person Syndrome: What is Celine Dion's 'human statue' illness - (https://www.hindustantimes.com/world-news/celine-dion-stiff-person-syndrome-celine-dion-health-stiff-person-syndrome-what-is-celine-dion-s-human-statue-illness-101670552577240.html)
  2. About Stiff-Person Syndrome - (https://www.ninds.nih.gov/health-information/disorders/stiff-person-syndrome)
  3. Stiff Person Syndrome-Rare Disease Database - (https://rarediseases.org/rare-diseases/stiff-person-syndrome/)
  4. Stiff person syndrome (SPS) - (https://my.clevelandclinic.org/health/articles/6076-stiff-person-syndrome)


Source-Medindia


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