What is Stiff-Person Syndrome? Celine Dion’s Diagnosis, Explained - The New York Times

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Celine Dion canceled her upcoming circuit aft being diagnosed with this uncommon neurological condition. Here’s what we know.

A photograph  of vocalist  Celine Dion arriving astatine  a Paris manner  amusement   successful  2019.
Credit...Lucas Barioulet/Getty Images

Dec. 8, 2022, 6:57 p.m. ET

Pop superstar Celine Dion announced Thursday that she is canceling and rescheduling her planned 2023 circuit dates aft being diagnosed with stiff idiosyncratic syndrome, a uncommon neurological condition.

The syndrome, which causes progressive stiffness successful the assemblage and terrible musculus spasms, is “exquisitely rare” and affects possibly one successful a cardinal people, according to Dr. Pavan Tankha, the aesculapian manager of broad symptom betterment astatine Cleveland Clinic.

Here’s what to cognize astir the diagnosis, symptoms and attraction of the condition.

Stiff idiosyncratic syndrome is simply a uncommon autoimmune neurological information that affects the cardinal tense strategy and tin origin rigidity passim the assemblage and achy musculus spasms. It was archetypal coined successful the 1920s (as “stiff antheral syndrome”) aft doctors described patients falling over similar “a woody man.”

The nonstop origin of the information is not clear, but “the immune strategy is involved,” said Dr. Scott Newsome, the manager of the Stiff Person Syndrome Center astatine Johns Hopkins Medicine. The syndrome is hard to diagnose, Dr. Newsome said, and whitethorn beryllium underrecognized.

The syndrome itself is not deadly, but it tin importantly impact a patient’s prime of life. And similar galore chronic conditions, the associated complications tin pb to shortened beingness expectancy.

Anyone astatine immoderate property tin get the condition, said Dr. Richard Nowak, an adjunct prof of neurology astatine Yale School of Medicine, but it’s astir emblematic for radical successful mediate adulthood, betwixt 30 and 60, and tin beryllium associated with highly stressful events. Like galore different immune conditions, it’s much communal successful women than men, Dr. Newsome said.

Certain autoimmune conditions and cancers have been tied to a flimsy summation successful hazard for processing the syndrome, Dr. Tankha said, including diabetes, thyroiditis, vitiligo, bosom cancer, thyroid cancer, lung crab and colon cancer. However, the hazard remains low.

Stiff idiosyncratic syndrome often begins with stiffness successful the torso and abdomen, which tin past dispersed to the legs, arms and face, Dr. Tankha said. At first, this alteration successful mobility whitethorn beryllium infrequent, but implicit clip it tin go constant, origin radical to locomotion somewhat hunched implicit oregon suffer the quality to locomotion altogether. People besides tin acquisition achy musculus spasms oregon continuous aching.

The duration of the spasms tin beryllium anyplace from seconds to hours, Dr. Tankha said, and they tin beryllium truthful terrible that they interruption bones oregon origin patients to autumn down. The musculus spasms tin beryllium triggered by a assortment of biology factors, specified arsenic loud noises, acold temperatures and affectional stress, Dr. Newsome said. The beingness of the chronic symptom tin besides pb immoderate patients to develop anxiety, slump and phobias of going extracurricular oregon trying caller activities.

Symptoms and their severity alteration idiosyncratic to person, according to Dr. Newsome. “It’s an idiosyncratic disease,” helium said.

There is nary cure for stiff idiosyncratic syndrome, truthful doctors absorption connected grounds and symptom management. The attraction for the information is often a patchwork of medicine and non-medication interventions, Dr. Newsome said.

The rigidity and spasming tin beryllium treated with musculus relaxers and Botox injections. More terrible symptoms are treated with immunotherapy and immunosuppressants, Dr. Newsome said. Tapering down the body’s immune effect tin assistance alleviate symptoms, said Dr. Senda Ajroud-Driss, an subordinate prof of neurology astatine Northwestern Feinberg School of Medicine.

For the non-pharmacological treatments, patients whitethorn acquisition cognitive behavioral therapy and different psychotherapy to assistance them mitigate the affectional triggers that punctual musculus spasms, arsenic good arsenic to make skills for surviving with a chronic condition.

Other types of treatments, specified arsenic carnal therapy, aqua therapy, vigor therapy and acupuncture tin supply alleviation for immoderate patients.

Diagnosing the information takes a operation of tools, Dr. Nowak said. Because of however uncommon it is, stiff idiosyncratic syndrome is typically diagnosed by archetypal ruling retired other, much communal conditions.

Healthcare providers volition past execute a carnal and neurological exam, bash humor work, measure the stiffness of muscles, instrumentality an MRI and different imaging tests and administer a spinal tap.

It’s important to retrieve that portion determination are communal symptoms that tin beryllium associated with stiff idiosyncratic syndrome, the information is precise rare, Dr. Nowak said.

“Every ache and symptom is not needfully stiff person,” helium said.

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