What Is Stiff Person Syndrome? Symptoms and Causes
Céline Dion not too long ago introduced that she’s been identified with Stiff Individual Syndrome—the singer’s prognosis is the rationale behind the cancellation and postponement of her upcoming live performance dates. So, following this massive announcement, what’s Stiff Individual Syndrome, precisely? What are its signs and causes?
In an Instagram put up, Dion shared, “Just lately, I have been identified with a really uncommon neurological situation known as Stiff Individual Syndrome, which impacts one thing like one in 1,000,000 folks. Whereas we’re nonetheless studying about this uncommon situation, we now know that that is what has been inflicting all the spasms that I have been having.”
We spoke with each a neurologist and an assistant professor of Medical Neurology to seek out out extra about this unusual situation.
Associated: Céline Dion Opens Up About Uncommon Incurable Well being Prognosis
What Is Stiff Individual Syndrome?
Stiff Individual Syndrome (SPS) is a uncommon neurological dysfunction that causes stiffness and rigidity of muscle groups. As Cleveland Clinic describes, SPS “impacts the central nervous system (the mind and spinal wire).”
Signs of Stiff Individual Syndrome
Leah Croll, MD, Assistant Professor, Medical Neurology, Lewis Katz Faculty of Drugs at Temple College, explains essentially the most distinguished signs are:
- Stiffness affecting any variety of muscle groups
- Muscle spasms which might be triggered by loud noises and emotional upset
- Difficulties with strolling
Cleveland Clinic shares, “Folks with this situation first expertise a stiffening of the muscle groups of their trunk adopted, over time, by the event of stiffness and rigidity within the legs and different muscle groups within the physique.”
Sufferers with Stiff Individual Syndrome are additionally extra susceptible to develop nervousness, melancholy and phobias.
Associated: Cleveland Clinic Is Launching a Mind Research to Assist Perceive the Earliest Indicators of Neurological Problems—This is Why It is So Essential
Causes of Stiff Individual Syndrome
The reason for Stiff Individual Syndrome is, sadly, not recognized.
The illness comes from a disruption within the chemical signaling that happens between the central nervous system and the muscle groups, Dr. Croll states. The precise explanation for this disruption stays unclear, nevertheless it appears to be associated to an auto-immune course of.
“It was once thought-about an autoimmune dysfunction, nevertheless it’s not,” says Dr. Joey Gee, a neurologist with Windfall Mission Hospital in Southern California. “However folks with the situation do have biomarkers of antibodies to glutamic acid decarboxylase.”
According to Cleveland Clinic, “Twice as many ladies have stiff particular person syndrome as males. Signs can happen at any age however often develop between ages 30 and 60.” And SPS is more likely to be seen in people who’ve autoimmune problems (together with diabetes, thyroiditis, vitiligo and pernicious anemia) or cancers (akin to breast, lung, kidney, thyroid, colon and Hodgkin’s lymphoma).
How To Handle and Deal with Stiff Individual Syndrome
Sadly, there isn’t a treatment for Stiff Individual Syndrome, so administration is targeted on minimizing signs as a lot as doable and serving to sufferers to deal with their new actuality, Dr. Croll explains. This may occasionally entail medicines to chill out the muscle groups, immune system-modulating therapies and bodily remedy.
“The predominant therapy [for] Stiff Individual Syndrome is to extend the effectiveness of gamma-aminobutyric acid (GABA) by means of treatment,” says Dr. Gee. “GABA is an inhibitory neurotransmitter that decreases a nerve cell’s skill to ship, obtain or create chemical messages to different nerve cells.”
Benzodiazepines are one other therapy for these with Stiff Individual Syndrome, together with muscle relaxant remedy and different medicines that deal with GABA. Lengthy-term therapy additionally consists of immunotherapy, Dr. Gee provides.
Subsequent up: Every little thing You Have to Know About Aphasia, the Neurological Dysfunction Bruce Willis Is Battling
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