when ottawa neurologist dr. elizabeth pringle learned that singer céline dion had been diagnosed with stiff person syndrome, she knew immediately what that would mean for her performing career.
pringle has treated patients with the extremely rare neurological disorder and has seen the devastation it can wreak on their lives.
“when i first read the news i thought: ‘that is too bad. this isn’t a good thing for someone in that business to have.’”
not only does stiff person syndrome severely impact a person’s ability to move, but sounds and stress — which are part of a high profile performing career — can trigger painful muscle spasms.
the internationally renowned quebec-born pop singer announced last week that she had been diagnosed with the progressive disorder that got its name because its symptoms usually include rigid muscles in a person’s torso, arms and legs, giving them a gait that has been compared to the tin man in the wizard of oz.
patients also have heightened sensitivity to sound, distress and touch.
stiff person syndrome can be difficult to diagnose and its mechanism is not completely understood, said pringle.
it is thought that the immune system in people with the disease mistakenly attacks an enzyme which plays a role in producing a vital neurotransmitter called gamma-aminobutyric acid (gaba). that decreases the amount of gaba in the body, leading to painful and debilitating symptoms. it is often diagnosed in middle age, said pringle, and is more common in women than men.
there is no cure, but the symptoms are often managed with oral therapy, including high doses of
benzodiazepines
, such as valium, said pringle. treatments such as botox and ultrasound are also used to manage symptoms.