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In December 2022, Canadian singer Celine Dion revealed to the world that she suffers from Stiff Person Syndrome (SPS). In a recent interview, the 56-year-old elaborated on the severe impairment that is the rare neurological disorder, which causes muscle stiffness, painful spasms, and emotional distress. Dion, who has not performed in years and has canceled several concerts, was forced to abandon her Courage World Tour for 2024.
“It feels like someone is strangling you. It feels like someone is squeezing your larynx/pharynx. It goes into spasms,” Dion emotionally told Kotb. We speak to medical professionals to get a deeper insight into this disorder.
Celine Dion and her long battle with Stiff Person Syndrome
In an Instagram video in 2022, the Canadian singer revealed that she was diagnosed with the painful condition. She had to cancel several concerts and performances due to her struggles with SPS in the period following her diagnosis. Initially, Dion had trouble keeping voice control, but in the more advanced stages of the disease, the singer has shared that she experienced muscle spasms (resulting in broken ribs), trouble walking and breathing, among other troubles.
what is this about
“Stiff person syndrome is an autoimmune disorder that causes muscle stiffness, especially in the torso and limbs, leading to painful cramps. It is also associated with other autoimmune disorders like type-1 diabetes, thyroid and vitiligo,” says Dr Shailesh Rohatgi, HOD – Department of Neurology, DPU Superspeciality Hospital, Pune.
This disorder can affect anyone, but it is more prevalent in women aged between 30 and 60 years. “There are also rare cases seen in children and the elderly. It is highly likely that SPS can develop at a young age and appear years later,” says Rohatgi, adding that it develops when the body starts making antibodies against its own antigens. “In this case, it attacks a specific protein called glutamic acid decarboxylase (GAD). These are antibodies that act on receptors in the brain, eventually leading to SPS. This rigidity can occur throughout the body or affect certain areas,” explains the neurologist.
symptoms get worse over time
Dr Ishu Goel, deputy consultant neurologist at Sir HN Reliance Foundation Hospital, Mumbai, explains that “general stiffness throughout the body” and “stiff, plastic-like gait” are among the earliest symptoms that patients usually come with. “What is important to note is that this is not present from birth, but develops over a few months. It is only when the symptoms become very painful that patients visit the neurology OPD,” says Goel.
The pain reaches such an extent that patients are unable to move or even sleep. “If the respiratory muscles are affected, they may also complain of breathlessness. Abnormal posture may develop in the lower back due to persistent spasms over some time. It is also usually associated with a lot of anxiety due to the unexpected fall. In very, very advanced stages, there may be difficulty in speaking and abnormalities in eye movement,” she says.
No, it is not fatal
Though SPS is not a fatal disorder, if not diagnosed and treated timely, it can lead to adverse complications like lung infections and bed sores, Rohatgi said, adding, “Though the treatment is subjective depending on the severity of the condition, IVIG (intravenous immunoglobulin) and immunosuppressants are given as needed. Plasma exchange is also done – the blood and plasma are separated, after which the plasma is filtered for harmful antibodies.”
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