Celine Dion suffers from Stiff-Person Syndrome: Know all about this rare neurological condition

World-famous singer Celine Dion, who shot to fame with her song “My heart will go on”, has been battling Stiff-Person Syndrome, a rare neurological ailment that affected her ability to walk and sing. As a result, she took a break from live performances last year. The lack of muscle control has been one of the 55-year-old singer’s challenges, as revealed in an interview by her sister Claudette Dion, who has also expressed concern about whether Celine Dion will ever come back to the stage.

Her concerns are legitimate considering how uncommon the illness is to treat. Stiff-Person Syndrome affects just 1 in a million people, mostly women, according to the US National Institute of Health (NIH). It is frequently associated with other autoimmune disorders.

This is not the first time someone has spoken about Celine Dion’s health condition. The singer was greatly affected when her Courage World Tour was canceled in May 2023 due to this neurological problem. In an Instagram post, she had herself apologised to her followers and acknowledged that even in perfect health, touring can be challenging. This had displayed her immense resolve to be stronger in the face of tough times. Celine Dion has been receiving treatment and support for Stiff-Person Syndrome from physicians as well as family members.

What is Stiff-Person Syndrome?

Stiff-Person Syndrome (SPS) is an uncommon central nervous system condition characterised by rigidity and painful muscle spasms of mostly axial and proximal limb muscles, as per a StatPearl report. Frederick Moersch and Henry Woltman initially reported it in 1956, drawing on a case series of 14 individuals with progressively increasing and decreasing tightness in their thigh, abdominal, and spinal muscles. Moersch-Woltman Syndrome is another term for this illness, which was once known as stiff-man syndrome.

“Stiff-Person Syndrome (SPS) is a rare problem that affects the brain and nerves. It makes muscles stiff and causes painful spasms in the arms and legs. SPS happens when the body’s defence system attacks itself. It is an autoimmune condition with a genetic predisposition and is also associated with certain malignancies,” says Dr Pawan Ojha, Neurologist, tells HealthShots.

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The estimated prevalence in the general population is 1 to 2 cases per million, with females being affected twice as often as males, regardless of race. Symptoms typically develop between the ages of 20 and 60, the doctor cites from the NIH.

This syndrome most commonly causes muscle stiffness and painful spasms that come and go and can worsen over time. Classic Stiff Person Syndrome, Partial Stiff Person Syndrome, and Stiff Person Syndrome Plus are the three types of Stiff Person Syndrome, explains counselling psychologist Divya Mohindroo.

What are the symptoms of stiff-person syndrome?

Stiff-Person Syndrome usually begins with rigidity and stiffness of the trunk muscles, resulting from continuous contraction of abdominal and paraspinal muscles. Patients often describe difficulties bending and turning. Over time, the rigidity spreads to the proximal upper and lower limbs, explains Dr Ojha.

Typically, a person will feel muscle stiffening in the torso and limbs, along with episodes of violent muscle spasms. Muscle spasms can involve the entire body or only a specific area. These spasms can last a few seconds, minutes, or, occasionally, a few hours. Spasms can be triggered by unexpected or loud noises, physical touch or stimulation, changes in temperature, including cold environments and stressful events. The rigidity causes pain and an aching discomfort.

These symptoms can fluctuate in severity without a clear reason or trigger. The muscle spasms can be so severe that they cause the person to fall down. As stiffness increases, some people develop an abnormal posture that can make it difficult to walk or move. These symptoms can lead to difficulty walking and, over time, even greater disability.

People with Stiff Person Syndrome are also more likely to have symptoms of depression and anxiety.

What causes Stiff-Person Syndrome?

People with Stiff Person Syndrome have unique antibodies in their blood that are made by the body. These antibodies, which block the glutamic acid decarboxylase (GAD) enzyme, are called anti-GAD65 antibodies. GAD helps make the gamma-aminobutyric acid (GABA) neurotransmitter. When GABA is produced in the body in the right amount, it reduces or blocks certain nerve signals. If GABA doesn’t function as expected, nerve cells can act in incorrect ways. In people with Stiff-Person Syndrome, the nervous system becomes hyperexcitable without the proper amount of GABA. This results in physical symptoms such as muscle spasms as well as psychological symptoms, including anxiety and depression.

The common triggers of SPS symptoms are because of GABA pathway disruption, explains Divya Mohindroo.

SPS is often associated with other autoimmune disorders, including type 1 diabetes, and thyroid disorders. Cancer is rarely associated with SPS (in less than 5% of cases). When cancer is found in people with SPS, it is most commonly breast or lung cancer, and it is identified within a few years after SPS symptoms start. Cancer associated with SPS is called paraneoplastic SPS.

What is the treatment of Stiff-Person Syndrome?

“The diagnosis of stiff-person syndrome is typically confirmed through blood tests and electromyography (EMG). While there is no cure for SPS, various treatments are available to help manage symptoms. Treatment options include medications and therapies for symptom management, as well as immunotherapy or disease-modifying treatment.”

Stiff Person Syndrome (SPS) is a rare neurological disorder characterised by muscle stiffness and spasms. While the primary management of SPS typically involves medical treatments like medications, physical therapy, and other interventions targeting the neurological aspects, support from a mental health expert can play a valuable role in addressing the emotional and psychological aspects of living with a chronic condition.

Here are ways in which a mental health expert can provide support:

1. Emotional support

Coping with a chronic condition like SPS can be emotionally challenging. A mental health expert, such as a psychologist or counselor, can provide a safe space for individuals to express their feelings, fears, and concerns.

2. Stress management

Stress can exacerbate symptoms in some neurological conditions. Mental health professionals can teach stress management techniques, such as relaxation exercises, mindfulness, and other coping strategies, to help individuals manage stress and improve their overall well-being.

3. Cognitive Behavioral Therapy (CBT)

CBT is a therapeutic approach that focuses on changing negative thought patterns and behaviors. It can be beneficial for individuals with chronic conditions in managing anxiety, and depression, and improving overall mental well-being.

4. Quality of life improvement

Mental health experts can work with individuals to set realistic goals, improve self-esteem, and enhance the overall quality of life despite the challenges posed by SPS.

5. Support groups

Participating in support groups, either in-person or online, can connect individuals with SPS to others facing similar challenges. Sharing experiences, advice, and encouragement within a supportive community can be emotionally uplifting.

6. Educational support

A mental health expert can provide education about SPS and help individuals and their families better understand the condition. This can empower individuals to make informed decisions about their treatment and lifestyle.

7. Counseling for caregivers

Caregivers of individuals with SPS may also face emotional and mental health challenges. Mental health experts can provide counseling and support for caregivers, helping them cope with the demands of caregiving and maintaining their well-being.

People with SPS need to have a comprehensive healthcare team that includes neurologists, physical therapists and mental health professionals working collaboratively to address both the physical and emotional aspects of the condition.

Coordination between healthcare providers ensures a holistic approach to care, improving overall outcomes and the well-being of individuals living with Stiff-Person Syndrome.