Celine Dion's struggle with stiff person syndrome

Canadian singer Celine Dion. (Courtesy)

When Canadian singer Celine Dion announced her diagnosis with Stiff Person Syndrome (SPS) in December 2022, it brought global attention to a rare neurological disorder that affects only one or two people per million.

This autoimmune condition, characterised by progressive muscle stiffness and painful spasms, has left many wondering about its causes, symptoms, and treatment options.

SPS, previously known as “stiff man syndrome,” predominantly affects women, with onset typically occurring between ages 20 and 50.

The World Health Organisation estimates the annual incidence at one case per million people, underlining its rarity. Despite its infrequency, SPS can have a profound impact on those affected, often leading to significant disability and reduced quality of life.

According to the National Organisation of Rare Disorders, SPS is caused by an autoimmune response that targets the central nervous system, particularly affecting the production of gamma-aminobutyric acid (GABA), a crucial inhibitory neurotransmitter.

The primary symptoms of SPS include, muscle stiffness, typically starting in the trunk and spreading to limbs, painful muscle spasms, often triggered by unexpected stimuli, heightened sensitivity to noise, touch, and emotional stress, unsteady gait and frequent falls, developing hunched postures over time.

As the disease progresses, some patients may become disabled. The fear of triggering spasms can lead to anxiety and social isolation, further impacting patients’ mental health.