What is stiff person syndrome? Diagnosis, symptoms and treatment - CNN

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TOPSHOT - Canadian vocalist  Celine Dion performs connected  the opening   nighttime  of her caller   satellite   circuit  "Courage" astatine  the Videotron Centre successful  Quebec City, Quebec, connected  September 18, 2019. (Photo by Alice Chiche / AFP) (Photo by ALICE CHICHE/AFP via Getty Images)

Celine Dion reveals diagnosis of a uncommon neurological disorder

02:35 - Source: CNN

CNN  — 

Stiff idiosyncratic syndrome is simply a uncommon neurological information that tin instrumentality years to diagnose and tailor treatment.

Singer Celine Dion announced successful an Instagram video Thursday that she is canceling immoderate performances due to the fact that she has been diagnosed with the condition.

“While we’re inactive learning astir this uncommon condition, we present cognize this is what’s been causing each of the spasms that I’ve been having,” Dion said.

Stiff idiosyncratic syndrome is characterized by musculus rigidity and spasms, heightened sensitivity to stimuli specified arsenic dependable and lights, and affectional distress that tin origin musculus spasms, according to the National Institute of Neurological Disorders and Stroke.

“It often is associated with musculus spasms, which could beryllium rather severe. These tin origin falls, terrible symptom and important disability,” said Dr. Emile Sami Moukheiber of the Stiff Person Syndrome Center astatine Johns Hopkins Medicine. “Falls from terrible spasms are precise common. These spasms tin beryllium precipitated by startle, terrible emotions, acold weather.”

These spasms tin beryllium beardown capable to fracture bone, and immoderate autumn tin pb to terrible injury.

Dion said successful her video that spasms impact “every aspect” of her regular life, “sometimes causing difficulties erstwhile I locomotion and not allowing maine to usage my vocal cords to sing the mode I’m utilized to.”

The syndromes symptoms tin besides origin anxiety.

“Many patients, if not all, person an anxiousness that is intrinsic to the illness and that anxiousness really feeds connected the carnal ailments of the illness that radical tin have,” Dr. Scott Newsome, manager of the Stiff Person Syndrome Center, said successful a video connected the organization’s website.

The information affects astir 1 a cardinal people, and astir wide neurologists volition spot lone 1 oregon 2 cases successful their lifetimes, Moukheiber said.

The archetypal lawsuit of stiff idiosyncratic syndrome was reported successful the 1950s, according to Newsome, and the illness was historically referred to arsenic “stiff antheral syndrome.”

Since then, it has been recovered to impact doubly arsenic galore women than men, and the sanction was changed to stiff idiosyncratic syndrome to debar confusion.

Stiff idiosyncratic syndrome is thought to person features of an autoimmune disease, according to the National Institute of Neurological Disorders and Stroke.

Although the nonstop origin isn’t clear, according to the institute, “people with SPS person elevated levels of GAD, an antibody that works against an enzyme progressive successful the synthesis of an important neurotransmitter successful the brain.”

Because of the rarity of the illness and the ambiguity of its symptoms, radical volition often question attraction for chronic symptom earlier they get neurological care. On average, it takes astir 7 years for idiosyncratic to person a diagnosis of stiff idiosyncratic syndrome, Newsome says.

“Sometimes, [patients] get labeled crazy,” helium said, “because connected exam aboriginal on, determination aren’t the hallmark features of stiff idiosyncratic syndrome.”

There is nary known cure for stiff idiosyncratic syndrome, but medications tin easiness the symptoms. Immunoglobulin medications tin assistance little sensitivity to airy oregon dependable triggers, perchance helping caput disconnected falls oregon spasms.

Pain relievers, anti-anxiety medications and musculus relaxers tin beryllium a portion of attraction for this disease. The Stiff Person Syndrome Center besides uses botulinum toxin injections.

“If near untreated, the illness tin origin terrible impairment of regular living,” Moukheiber said.

But with treatment, symptoms tin beryllium controlled.

Dion, who said she has struggled with her wellness for a while, said she has a large squad of aesculapian professionals and her children’s support.

“I’m moving hard with my sports medicine therapist each time to physique backmost my spot and my quality to execute again,” she said. “But I person to admit it’s been a struggle.”

For a performer similar Dion, large noises and agleam lights could enactment arsenic triggers to musculus spasms.

“This is simply a precise challenging unwellness that mightiness instrumentality a toll connected her if it is not treated aggressively, timely and appropriately,” Moukheiber said.

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