“While we’re still learning about this rare condition, we now know this is what’s been causing all of the spasms that I’ve been having,” Dion mentioned.
What is stiff particular person syndrome?
Stiff particular person syndrome is characterised by muscle rigidity and spasms, heightened sensitivity to stimuli resembling sound and lights, and emotional misery that may trigger muscle spasms, based on the National Institute of Neurological Disorders and Stroke.
“It frequently is associated with muscle spasms, which could be quite severe. These can cause falls, severe pain and significant disability,” mentioned Dr Emile Sami Moukheiber of the Stiff Person Syndrome Centre at Johns Hopkins Medicine.
“Falls from severe spasms are very common. These spasms can be precipitated by startle, severe emotions, cold weather.”
These spasms may be robust sufficient to fracture bone, and any fall can result in extreme damage.
Dion mentioned in her video that spasms have an effect on “every aspect” of her every day life, “sometimes causing difficulties when I walk and not allowing me to use my vocal cords to sing the way I’m used to.”
The syndromes signs may trigger nervousness.
“Many patients, if not all, have an anxiety that is intrinsic to the disease and that anxiety actually feeds on the physical ailments of the disease that people can have,” Dr. Scott Newsome, director of the Stiff Person Syndrome Centre, mentioned in a video on the organisation’s web site.
Who is affected by stiff particular person syndrome?
The situation impacts about one in one million folks, and most normal neurologists will see just one or two instances of their lifetimes, Moukheiber mentioned.
The first case of stiff particular person syndrome was reported within the Fifties, based on Newsome, and the illness was traditionally known as “stiff man syndrome.”
Since then, it has been discovered to have an effect on twice as many ladies than males, and the identify was modified to stiff particular person syndrome to keep away from confusion.
What causes stiff particular person syndrome?
Stiff particular person syndrome is believed to have options of an autoimmune illness, based on the National Institute of Neurological Disorders and Stroke.
Although the precise trigger is not clear, based on the institute, “people with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain.”
Because of the rarity of the illness and the anomaly of its signs, folks will typically search look after persistent ache earlier than they get neurological care. On common, it takes about seven years for somebody to obtain a prognosis of stiff particular person syndrome, Newsome says.
“Sometimes, [patients] get labelled crazy,” he mentioned, “because on exam early on, there aren’t the hallmark features of stiff person syndrome.”
How is stiff particular person syndrome handled?
There is not any identified treatment for stiff particular person syndrome, however medicines can ease the signs. Immunoglobulin medicines may help decrease sensitivity to gentle or sound triggers, doubtlessly serving to head off falls or spasms.
Pain relievers, anti-anxiety medicines and muscle relaxers may be part of remedy for this illness. The Stiff Person Syndrome Centre additionally makes use of botulinum toxin injections.
“If left untreated, the disease can cause severe impairment of daily living,” Moukheiber mentioned.
But with remedy, signs may be managed.
Dion, who mentioned she has struggled together with her well being for some time, mentioned she has an incredible crew of medical professionals and her kids’s assist.
“I’m working hard with my sports medicine therapist every day to build back my strength and my ability to perform again,” she mentioned. “But I have to admit it’s been a struggle.”
For a performer like Dion, loud noises and vibrant lights may act as triggers to muscle spasms.
“This is a very challenging illness that might take a toll on her if it is not treated aggressively, timely and appropriately,” Moukheiber mentioned.
