STIFF PERSON SYNDROME

POPSTAR CELINE Dion revealed that she had been diagnosed with Stiff Person Syndrome (SPS), a rare neurological condition that triggers uncontrollable muscle spasms and a progressive stiffening of the body. The disorder made it difficult for her to walk and sing, and forced her to reschedule and cancel shows in Europe, she said in a video on Instagram.

Dion, a five-time Grammy winner and one of the best-selling music artists of all time, said the condition, which affects “something like one in a million people” had affected “every aspect of my daily life”.

THE CONDITION

SPS is an autoimmune condition of the central nervous system, which causes fluctuating but progressive stiffness and spasms mainly in the back and abdominal muscles. The condition was first described as ‘stiff-man syndrome’ by FP Moersch and HW Woltman in a paper published in 1956.

SPS does not by itself cause death, but it can severely impact the quality of life of the patient, and trigger complications that can lower life expectancy. While anyone of any gender can get the condition, it has been medically associated with high-stress events, and is more common in women, especially in the age group of 30-60 years.

The causes behind SPS are 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.”

“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.”

“Paroxysmal muscle spasms are the norm and superimposed upon baseline muscle stiffness.”

The spasms, may be powerful enough to break bones or dislocate joints. “As the disease progresses muscle stiffness spreads to proximal muscles of the lower extremities, and spasms become more frequent and severe.”

THE TREATMENT

Since SPS is a rare disease with heterogeneous manifestations, the diagnosis may be delayed by an average of six years after the onset of symptoms.

There is no cure for the condition, and treatment focuses on the management of symptoms and pain. Muscle relaxers and injections of Botox are sometimes used, and immunotherapy and immunosuppressants may be prescribed in serious cases. Some doctors may prescribe physiotherapy and acupuncture, alongside therapy using heat and water.

“Treatment involves the use of both symptomatic agents and immuno-modulating treatment aimed at the autoimmune basis of the disease.”

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