Stiff Person Syndrome- Everything you need to know

SPS is a rare, progressive neurological disorder that can make the muscles in the trunk (torso), arms, and legs stiff and harder to move

In 2022 Canadian singer Celine Dion was diagnosed with Stiff Person Syndrome, and ever since then, she has been battling this challenging health issue. It is good to be aware of what disease you may be exposed to, and if this neurological disorder can happen to a singer with so much of resources to keep herself happy, it can totally strike you.

What is Stiff-Person Syndrome?

Stiff-person syndrome (SPS) is a rare, progressive neurological disorder that can make the muscles in the trunk (torso), arms, and legs stiff and harder to move. It also makes a person sensitive to noise, touch, and emotional distress, which can set off muscle spasms.

People with SPS can develop a hunched-over posture over time and some may get too disabled to walk or move. Many with serious conditions even fall frequently because they lack normal reflexes to catch themselves. This can lead to injuries. Some are even scared of leaving the house as small things like the sound of a car horn, can trigger spasms and falls.

Are you liable to get stiff-person syndrome?

SPS affects females more than males. People with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo, and pernicious anemia are also prone to this condition.

Although scientists are yet to understand the causes of SPS, research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord.

Diagnosis and treatment

Diagnosis

It is important to go through all the checkups to detect SPS as it often gets misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia. You can have a definitive diagnosis through a blood test that measures the level of glutamic acid decarboxylase (GAD) antibodies.

People affected by SPS have often been seen with higher levels of GAD antibodies. Antibody titers are important for the diagnosis of SPS. A titer is a laboratory test that measures the presence and amount of antibodies in the blood.

Treatment

SPS is treatable, and the symptoms can be kept under control. While several symptoms of this condition can be treated with oral diazepam (an anti-anxiety and muscle relaxant drug), some need drugs like baclofen or gabapentin to alleviate muscle spasms.

According to a study funded by the National Institute of Neurological Disorders and Stroke (NINDS), intravenous immunoglobulin (IVIg) treatment is effective in reducing stiffness, and sensitivity to noise, touch, and stress. It also helps improve gait and balance for people with SPS. IVIg contains immunoglobulins (natural antibodies produced by the immune system) that are derived from thousands of healthy donors.

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