Stiff Person Syndrome

What is Stiff Person Syndrome?

Stiff Person Syndrome : A rare condition called Stiff Person Syndrome (SPS) affects both the immune system and the neurological system. The muscles start to tense uncontrollably as a result, which produces stiffness, pain, spasms, and difficulties moving.

This disease is in news due to celebrity star Celine Dion is suffering from this disease SPS (Stiff Person Syndrome).

Stress, noise, touch, or emotional anguish might cause the spasms. The affected person’s respiration and speech may also be impacted by the illness.

SPS is thought to be an autoimmune illness, in which the body’s immune system unintentionally destroys its own tissues, while the actual source of the condition is unknown.

Although SPS can affect anybody, it is more prevalent in women and those between the ages of 30 and 60. One in a million persons globally are impacted by it.

SPS cannot be cured, however there are treatments that can help patients live better lives by managing their symptoms. These include medications like immune suppressants, anti-anxiety pills, and muscle relaxants; physical treatment like massages and stretches; and psychological support like stress reduction and counselling.

A rare and incapacitating neurological condition affecting the brain and spinal cord is known as stiff person syndrome (SPS).

It results in discomfort, muscle spasms, and stiffness that can make it difficult to move around and carry out daily tasks. Type 1 diabetes and thyroiditis are two autoimmune disorders that SPS frequently co-occurs with.

Cause of Stiff Person Syndrome

Although the precise aetiology of SPS is uncertain, it is believed to be an autoimmune reaction that targets the protein glutamic acid decarboxylase (GAD), which is involved in the production of the chemical gamma-aminobutyric acid (GABA). GABA is a neurotransmitter that controls muscle tone and curbs overly tight muscles.

There is no cure for SPS, but treatments can help manage the symptoms and improve the quality of life. Some of the treatments include:

  • Oral or intravenous medications that reduce muscle spasms, such as diazepam, baclofen, or gabapentin.
  • Intravenous immunoglobulin (IVIg), which contains antibodies from healthy donors that may help modulate the immune system and reduce inflammation¬≤¬≥.
  • Plasma exchange, which removes harmful antibodies from the blood and replaces them with fresh plasma.
  • Rituximab, which is a monoclonal antibody that targets a type of immune cell called B cells that produce antibodies.
  • Physical therapy, which can help improve mobility, strength, and balance.

Some ongoing researches are exploring new ways to diagnose, treat, and understand SPS. For example:

  • A study is looking into the role of GAD antibodies in the pathogenesis of SPS and whether they can be used as biomarkers for diagnosis and prognosis. – A study is looking into the genetic factors that may predispose people to develop SPS or influence their response to treatment.

Visit the websites of the Genetic and Rare Diseases Information Centre (GARD) or the National Institute of Neurological Disorders and Stroke (NINDS) if you’re interested in learning more about SPS or taking part in a clinical study.

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