Around 100 Swiss have the same diagnosis as Céline Dion

"A celebrity coming out of the closet is the best thing that can happen for a rare disease. It could help a lot of people," Stephan Rüegg told the Keystone-SDA news agency. Rüegg is a senior physician at the University Hospital Basel and a specialist in stiff person syndrome. "A single person can make an incredible difference," said Bettina Balint. The doctor treats sufferers at Zurich University Hospital and is, among other things, Chair of the International Movement Disorder Society's Task Force on Stiff Person Spectrum Disorders.

Stiff person syndrome is an autoimmune disease. The body's immune system attacks an enzyme that plays a crucial role in muscle tension and relaxation. If it fails, the nervous system goes crazy: the muscles contract in a flash and become as stiff as a board, especially under stress.

Emotions, both positive and negative, or acoustic stimuli can also cause the muscles to suddenly stiffen. The effects of this can be drastic. In the documentary, Dion talks about ribs that were broken due to muscle tension. Rüegg tells of his aunt, who suffers from stiff person syndrome, who, after being honked at by a driver, became so stiff in the middle of a pedestrian crossing that she fell over.

No official figures

Stiff person syndrome is an extremely rare disease. There are no official figures on the number of people affected. Rüegg estimates that there are between 80 and 100 people affected throughout Switzerland. He arrives at this figure by extrapolating from the 13 patients with the syndrome who are being treated at the University Hospital Basel. Eight people with the syndrome are being treated at Zurich University Hospital.

In specialist books, the frequency of the syndrome is estimated to be even lower: one in a million people are affected, according to an old work from the USA. However, experts believe that this figure is a clear underestimate.

Disease is incurable

There is no cure for stiff person syndrome. It is a progressive disease. The symptoms get worse over time. As a rule, muscle stiffness begins in the trunk and abdomen, but can then spread to muscles throughout the body. According to Rüegg, the symptoms can be brought under control with medication. "But this treatment is not without its challenges," said Rüegg. Patients take the sedative Valium, for example, to relax the muscles.

Doctors also use various medications to try and keep the antibodies responsible for the attacks on the tension-control enzyme in check. This can slow down the progression of the disease.

Stage comeback for Céline Dion?

However, stiff person syndrome is often only diagnosed when it is already well advanced and sufferers have already endured a long ordeal. This is also the case with Dion. The singer felt her first symptoms in 2008. She received her diagnosis more than ten years later. "On average, it takes six to seven years for patients with stiff person syndrome to receive the correct diagnosis," said Balint. According to the experts, becoming aware of the disease could lead to this period being shortened.

Despite the serious illness, the singer is convinced that she will soon be back on stage. "I'm rather skeptical," said Rüegg. With well-adjusted medication, patients can lead a relatively good life, but being on stage for several hours in a highly emotional and exciting situation is a major challenge.