The 2024 Paris Olympics opening ceremony was a historic event, featuring an unprecedented open-air boat parade down the Seine. It also marked the highly anticipated return to the stage of five-time Grammy winner Celine Dion. Performing from a stage atop the glittering Eiffel Tower, Dion delivered a stunning rendition of "Hymne a l' amour" by Edith Piaf to close the ceremony. This marked her first performance since being diagnosed with Stiff Person Syndrome in December 2022.

This rare autoimmune neurological disorder affects fewer than 5,000 people in the U.S., according to the Genetic and Rare Disease Information Center (GARD), and is characterized by muscle stiffness and painful spasms that can worsen over time. While there is no cure for this progressive condition, medications and therapies can help manage the symptoms.

In an emotional Instagram video two and a half years ago, Dion revealed her diagnosis and announced she would postpone her 2023 tour to focus on her health. But what is stiff person syndrome? Let’s check!

What Is Stiff Person Syndrome?

Stiff Person Syndrome (SPS) is a rare and progressive neurological disorder that affects the central nervous system, leading to severe muscle stiffness and painful spasms. Stiff Person Syndrome is an autoimmune disease in, which the body's immune system mistakenly attacks its tissues. In the case of SPS, the immune system targets the central nervous system, particularly the spinal cord and brain, disrupting normal muscle function.

Researchers have found that many individuals with SPS have elevated levels of antibodies against glutamic acid decarboxylase (GAD), an enzyme involved in the synthesis of the neurotransmitter gamma-aminobutyric acid (GABA). GABA is crucial in regulating muscle tone and preventing excessive muscle contraction. When GAD antibodies interfere with GABA production, it can result in muscle stiffness and spasms, which are characteristic of SPS.

Other potential triggers for SPS include genetic factors and associations with other autoimmune diseases such as type 1 diabetes, thyroiditis, and pernicious anaemia.

The symptoms of Stiff Person Syndrome can vary widely among individuals, but they typically include:

- Muscle Stiffness: Persistent and severe muscle stiffness, particularly in the trunk and limbs, is the hallmark of SPS. This stiffness can lead to difficulty with movement and posture.

- Muscle Spasms: Sudden, painful muscle spasms or jerks, often triggered by stress, sudden movements, or external stimuli like loud noises.

- Postural Abnormalities: Over time, muscle stiffness and spasms can cause abnormal postures, such as a hunched back or an inability to bend.

- Impaired Mobility: Difficulty walking or moving, leading to a significant reduction in the quality of life.

- Pain: Chronic pain resulting from muscle stiffness and spasms.

In severe cases, SPS can affect the muscles responsible for breathing and swallowing, posing serious health risks.

While there is no cure for Stiff Person Syndrome, several treatment options can help manage its symptoms and improve the quality of life for those affected:

1) Medications:

- Benzodiazepines: Drugs like diazepam and clonazepam can help relax muscles and reduce spasms.

- Baclofen: A muscle relaxant that can be effective in reducing stiffness.

- Immunotherapy: Treatments like intravenous immunoglobulin (IVIG) or plasmapheresis can help reduce the immune system's attack on the nervous system.

- Anticonvulsants: Medications such as gabapentin or pregabalin may help alleviate muscle spasms and pain.

2) Physical Therapy: Regular physical therapy can help maintain muscle flexibility, improve mobility, and prevent joint contractures. Stretching and strengthening exercises tailored to the individual's needs are essential components of treatment.

3) Stress Management: Techniques such as yoga, meditation, and biofeedback can help manage stress, which is known to exacerbate symptoms of SPS.

4) Supportive Care: Occupational therapy and assistive devices, such as braces or mobility aids, can help individuals with SPS maintain independence and improve their daily functioning.