As Celine Dion is diagnosed with neurological disease, what is 'Stiff Person Syndrome'?

  • Celine Dion has been forced to cancel tour dates after being diagnosed with Stiff Person Syndrome (SPS)

  • The singer has shared the types of symptoms she has experienced, including spasms that affect her daily life

  • Dion is among the one in a million people thought to be affected by SPS, a rare neurological health condition

  • Read the full article to find out more about what living with the disorder is like and what treatments there are

Watch: Celine Dion cancels tour due to incurable 'Stiff Person Syndrome'

Celine Dion has had to reschedule and cancel tour dates due to being diagnosed with a rare neurological disease called Stiff Person Syndrome (SPS).

Sharing the news in an intimate Instagram post, she said her condition has caused spasms "affecting every aspect" of her daily life, sometimes including difficulty when she walks, and not allowing her to use her vocal cords how she's used to.

The singer, 54, said she has a "great team of doctors" supporting her, as well as her children giving her hope, and a sports therapist to help her build back strength and boost her ability to perform again.

But what exactly is SPS, which the star says "affects something like one in a million people" and what are the signs?

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What is Stiff Person Syndrome?

SPS is an extremely rare condition that affects the brain and nervous system and has features of an autoimmune disease, according to the National Institute of Health (NIH). It affects twice as many women as men.

It is associated with diseases like diabetes, thyroiditis, vitiligo and pernicious anaemia, and is often misdiagnosed as Parkinson's disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia.

While the cause is still unknown, research suggests it is the result of an 'autoimmune response gone awry in the brain and spinal cord'. A diagnosis can be made with a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies in the blood, as people with SPS have higher levels of this.

GAD works against an enzyme involved in the production of an important neurotransmitter (chemical messengers your body can't function without) in the brain.

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Celine Dion. (Getty Images)
Celine Dion has recently been diagnosed with SPS, which affects more women than men. (Getty Images)

Signs of Stiff Person Syndrome

SPS is characterised by fluctuating muscle rigidity in the trunk and limbs, and a heightened sensitivity to stimuli like noise, touch and emotional distress, which can trigger muscle spasms.

Abnormal postures, such as being hunched over and stiffened, are also associated with the condition. Some people with SPS can be too disabled to walk or move, and may feel scared to leave the house because street noises can trigger spasms and falls.

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Stiff Person Syndrome treatment

The NIH states that people with SPS respond to high doses of diazepam (used to treat anxiety, muscle spasms and seizures or fits) and several anticonvulsants (the medication type used for epilepsy).

It points out that a recent study funded by the National Institute of Neurological Disorders and Stroke (NINDS) demonstrated the effectiveness of intravenous immunoglobulin (antibodies produced by the body’s immune system) in reducing stiffness and lowering sensitivity to noise, touch and stress.

These types of medicines, along with pain relievers, are thought to improve SPS symptoms, but won't entirely cure the disorder. But while many people with SPS can have frequent falls, leading to severe injuries, symptoms can be well controlled with the appropriate treatment.

Speak to your GP if you have signs of SPS, and visit the Genetic Alliance for information on what other support is out there.