Is Stiff-Man Syndrome progressive? How many people have Stiff Persons syndrome?

Celine Dion’s announcement of her diagnosis with Stiff Persons Syndrome (SPS) has led to a lot of interest in the disorder.

According to the The National Institute of Neurological Disorders and Stroke (NINDS), Stiff Person Syndrome (SPS), is a rare, progressive neurological disorder first described nearly 40 years ago.

Though an exact cause is not yet known, its cause has been attributed to both neurologic and psychiatric processes.

SPS affects twice as many women as men and it is frequently associated with other autoimmune diseases such as type-I diabetes, thyroiditis, vitiligo, and pernicious anemia.

The exact incidence and prevalence of SPS is unknown, although one estimate places the incidence at approximately 1 in 1,000,000 individuals in the general population.

The distribution of SPS between men and women indicates a female predominance and it usually becomes apparent sometime between 30-60 years of age.

However, SPS has been reported to occur in children and older adults as well.