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What is ‘Stiff-Person Syndrome’, the rare neurological disorder Celine Dion suffers from?

"While we’re still learning about this rare condition, we now know that this is what has been causing all of the spasms that I’ve been having," the singer said

The disorder has left her with difficulties in walking and singing (Source: Celine Dion/Instagram)
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French-Canadian singer Celine Dion has opened up about being diagnosed with Stiff-Person Syndrome (SPS), a rare neurological condition that makes the muscles spasm uncontrollably. The disorder has left her with difficulties in walking and singing due to which she will be unable to take part in planned shows in the UK and Europe next year.

“I’ve been dealing with problems with my health for a long time, and it’s been really difficult for me to face these challenges and to talk about everything that I’ve been going through…It hurts me to tell you that I won’t be ready to restart my tour in Europe in February,” Dion said, in an emotional video shared on Instagram.

Sharing that this condition affects “something like one in a million people”, Dion added, “While we’re still learning about this rare condition, we now know that this is what has been causing all of the spasms that I’ve been having.”

Further revealing how it is affecting “every aspect of my daily life”, the five-time Grammy winner singer shared, “Sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to.”

What is Stiff-Person Syndrome?

It is a syndrome of fluctuating but progressive muscle stiffness and spasm that preferentially affects axial (back and abdominal) muscles, neurological experts noted. “It is a central nervous system disorder, meaning encephalomyelopathy, in which myelopathic features predominate in most patients. It frequently affects women with a median onset of 35 to 40 years of age,” Dr Mohan Krishna J, Consultant Neurologist, Yashoda Hospitals, Hyderabad said.

What are its causes?

Dr Vipul Gupta, Chief, Neurointerventional Surgery and Co-Chief, Stroke Unit, Artemis Hospital, Gurugram said that while the cause behind SPS is not fully understood yet, “there have been reports that spasms occur at any random time and can be triggered by loud noises, touch, and emotional distress.”

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Explaining further, Dr Mohan said that this autoimmune disease is caused by antibodies to proteins, resulting in impaired GABAergic (Gamma amino butyric acid) inhibition of the motor nerves in the brain and spinal cord. “It is associated with GAD65 (Glutamic acid decarboxylase) and amphiphysin antibodies. 70 per cent of patients with GAD antibodies have diabetes mellitus as a comorbidity. It is associated with other autoimmune diseases like cerebellitis, myasthenia gravis, hypo/hyperthyroidism, rheumatoid arthritis, systemic lupus erythematosus,” he said.

He added that people with amphiphysin antibodies can have SPS as a paraneoplastic (a symptom of underlying malignancy) manifestation with predominant upper limbs or cranial nerve involvement. “The malignancies associated with stiff-person syndrome are breast cancer, ovarian cancer, and small cell carcinoma of the lung,” the expert said.

Common Symptoms

According to experts, this syndrome can manifest itself in many ways. “SPS is known to affect the body posture of a person due to severe pain and stiffness in muscles in the trunk (torso), arms and legs. But symptoms can also include a person having greater sensitivity to noise, touch, and emotional distress,” Dr Gupta said.

Dr Mohan elucidated that the symptoms can be heterogeneous — classical and focal. “In the classical form of the disease, the initial symptom is typically painful muscle stiffness in the lumbosacral and abdominal regions. It affects both agonist and antagonist muscles simultaneously. Paroxysmal muscle spasms are the norm and superimposed upon baseline muscle stiffness. Spasms are provoked by movement, tactile/emotional/auditory stimuli, cold weather, and intercurrent infection. Spasms may be powerful enough to break bones/dislocate joints. As the disease progresses muscle stiffness spreads to proximal muscles of the lower extremities, and spasms become more frequent and severe.”

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Whereas, in the focal presentation of the disease, “it can involve one limb only (stiff limb syndrome), neck muscles (restricted head movement), thoracoabdominal muscles (exercise intolerance). It is commonly associated with affective symptoms such as anxiety, phobias and depression. These symptoms coupled with unusual postures due to stiffness can lead to an erroneous diagnosis of hysteria.”

Treatment

Dr Mohan said that since it is a rare disease with heterogenous clinical manifestations, the diagnosis may be delayed by an average of six years following symptom onset.

“Treatment involves the use of both symptomatic agents to enhance GABAergic influences and Immuno modulating treatment aimed at the autoimmune basis of the disease. In patients with paraneoplastic Stiff Person Syndrome, identification and eradication of the underlying malignancy can alleviate symptoms. Corticosteroids are rarely used as Immuno modulating agents in Stiff Person Syndrome because of a high incidence of concurrent diabetes mellitus, he said, emphasising the need for awareness “in the community and among medical professionals” for “early diagnosis and good treatment outcomes”.

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First published on: 09-12-2022 at 12:30 IST
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