A GP Writes: Stiff Person Syndrome

Stiff Person Syndrome has been in the media over the last week as Celine Dion has revealed she has been diagnosed with this condition and has postponed the remainder of her world tour as a result.

What is Stiff Person Syndrome?

• Stiff person syndrome is a very rare and progressive debilitating neurological and autoimmune condition.
• It occurs in one in every one million people.
• The cause of stiff person syndrome is unclear.
• It is twice as common in women than in men and is usually diagnosed in adulthood.

What are the Symptoms?

• Stiff person syndrome can cause worsening and severe muscle stiffness and rigidity together with painful spasms in the trunk and limbs.
• Extreme stiffness can affect posture and result in chronic pain and impaired mobility.
• This can be a very frightening condition, and affected people may become very fearful and depressed.
• People often lose the ability to work and are likely to require assistance with walking.
• As the condition progresses, people may lose the ability to bend and walk. The term ‘human statues’ has been used to describe this advanced stage of stiff person syndrome.

Diagnosis

• There are certain lab tests that can aid the diagnosis. However, stiff person syndrome is largely a clinical diagnosis- meaning the diagnosis is based on the patient’s symptoms.

Treatment

• As stiff person syndrome is so rare, it has not been possible to conduct large trials to test certain treatments. There is no cure.
• Treatment is focussed on managing the different symptoms.
• Multiple medications are used in combination, including muscle relaxants, anticonvulsant medication, steroids, Botox, and stem cell transplantation.
• Physiotherapy and rehabilitation are a major part of the treatment.
• This is a developing area of medicine, and it is hoped more definitive treatments will be identified.

Celine Dion hopes to continue her World Tour in 2024 once her condition has stabilised. She has said

“I have a great team of doctors working alongside me to help me get better and my precious children, who are supporting me and giving me hope. I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again, but I have to admit, it’s been a struggle”.

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