Priyanka Chopra's co-star Celine Dion’s rare neurological disorder: Important things you know about illness

By Team NewsableFirst Published Dec 9, 2022, 9:29 AM IST
Highlights

Popular Grammy Award winner Celine Dion on Thursday (Dec 08) announced the news about her unusual neurological disorder–Stiff Person Syndrome. Know 5 essential things to know about the disorder

To the dismay of many admirers, iconic Canadian singer and Grammy Award winner Celine Dion posted to Instagram on Thursday to warn her followers and well-wishers that she is suffering from a neurological illness known as Stiff Person Syndrome. As a result of her difficulty walking and singing, the 54-year-old singer stated that she will be unable to perform planned gigs in the UK and Europe next year. Ths singer made her film debut with Priyanka Chopra starrer ‘Love Again.' 

About Stiff Person Syndrome
According to the National Institute of Neurological Disorders and Stroke, the top organisation established in the USA, “Stiff-person syndrome (SPS) is an uncommon, degenerative neurological condition. Symptoms include tight muscles in the trunk (torso), arms, and legs and increased sensitivity to noise, touch, and mental stress, which can cause muscular spasms."

"People with SPS may develop aberrant, sometimes hunched-over postures over time." Some patients may be unable to walk or move at all. Many frequently fall because they do not have the typical reflexes to catch themselves. This can result in severe injury. People with SPS may be scared to leave their homes because street sounds, such as the sound of a car horn, can provoke spasms and falls," according to the US-based institution.

Cure and treatment of Stiff Person Syndrome?
According to the National Institute of Neurological Disorders and Stroke, "oral diazepam (an anti-anxiety and muscle relaxant medicine) or pharmaceuticals that relieve muscular spasms, such as baclofen or gabapentin, ameliorate some symptoms."

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“A research financed by the National Institute of Neurological Disorders and Stroke (NINDS) demonstrated that intravenous immunoglobulin (IVIg) therapy is helpful in lowering stiffness, sensitivity to noise, touch, and stress and for improving gait and balance for persons with SPS. IVIg comprises immunoglobulins (natural antibodies generated by the immune system) taken from thousands of healthy donors,” the report states. However, there is no effective treatment for the illness as of yet.

How to diagnose this disorder?
A blood test that analyses the amount of glutamic acid decarboxylase (GAD) antibodies can provide a reliable diagnosis. Most persons with SPS have raised (higher) levels of GAD antibodies. Antibody titers are significant for the diagnosis of SPS, the official data of the National Institute of Neurological Disorders and Stroke revealed.

Also Read: What is Stiff person syndrome? A glance at its causes and symptoms

Who can get affected by stiff person syndrome?
According to CNN, the disorder affects around one million people. Most general neurologists will only witness one or two cases in their careers, according to Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center at Johns Hopkins Medicine.

“The first case of stiff person syndrome was reported in the 1950s, according to Newsome, and the disease was historically referred to as ‘stiff man syndrome’. Since then, it has been discovered that women are affected twice as much as males, and the name has been modified to stiff person syndrome to avoid misunderstanding," the doctor explained.

What is the cause of stiff person syndrome?
According to the National Institute of Neurological Disorders and Stroke, "stiff person syndrome is suspected to have hallmarks of an autoimmune illness." Though the specific aetiology of the disease is unknown, "patients with SPS have increased levels of GAD, an antibody that acts against an enzyme involved in the manufacture of a key neurotransmitter in the brain," according to the leading university in the United States. Because of the disease's rarity and the vagueness of its symptoms, patients frequently seek care for chronic pain before seeking neurological therapy, according to the institute's statistics.

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