What is rigid person syndrome, celine dion disease

What is Stiff Person Syndrome, Celine Dion's disease

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Celine Dion was diagnosed with Stiff Person Syndrome, prompting the singer to cancel shows. Understand better about the condition of the artist.

One of the greatest voices of the last decades, Celine Dion, made a report this Thursday (8) through two videos on his Instagram informing about his current tour. In the recording, the singer makes public, in a very emotional way, that she had to postpone some shows while canceling others, because she was diagnosed with Stiff Person Syndrome🇧🇷 Understand the following disease better:

What is Rigid Person Syndrome?

A Stiff Person Syndrome — known by acronyms SPS (in English) or SPR — is a rare neurological disorder characterized by progressive muscle rigidity and repeated episodes of painful spasms, one of the most cited symptoms by Celine Dion🇧🇷 Muscle stiffness has variations, with worst-case and best-time scenarios, and usually occurs along with the spasms.

What is rigid person syndrome, celine dion disease
Image: MTL Blog

Twitching can occur randomly or be triggered by a variety of different reasons, including a sudden noise or light physical contact. The severity and progression of Stiff Person Syndrome vary from one person to another, and if left untreated, PRS can potentially progress to cause difficulty walking and significantly affect the patient's ability to perform daily tasks🇧🇷 While the exact cause of PRS is unknown, it is believed to be an autoimmune disorder and sometimes occurs along with other conditions of the same type.

Stiff Person Syndrome has been described in the medical literature under many different and confusing names. Originally described as "stiff man syndrome“, the name has been changed to reflect that the disorder can affect individuals of any age and gender — with women being the most affected.

Symptoms

The main symptoms associated with PRS are progressive muscle stiffness and floating — ranging from mild to severe and vice versa — that occurs in conjunction with muscle spasms. The severity and progression of PRS can vary from one person to another. Symptoms usually develop over a period of months and can remain stable for many years or worsen more slowly.

In some people, symptoms can be stabilized through medication, and affected individuals should talk to their doctor and medical team about their specific case, associated symptoms, and overall prognosis.

In many cases, Stiff Person Syndrome starts slowly over several months or even a few years, with affected individuals initially experiencing painful discomfort and stiffness, especially in the lower back or legs. At first, the stiffness may come and go, but gradually becomes fixed🇧🇷 the shoulders, neck and hips can also be affected.

What is rigid person syndrome, celine dion disease
Image: ScienceDirect

As the disease progresses, the muscle stiffness of the leg develops and is usually more pronounced on one side than the other, this leads to a slow, stiff gait. As stiffness increases, affected individuals may develop a somewhat stooped posture due to the external shape of the upper spine. In some individuals the stiffness may progress to also reach the arms or the neck. face.

What is rigid person syndrome, celine dion disease
Image: Time News

Spasms of the abdominal muscles can make individuals feel full faster than normal, leading to unintentional weight loss🇧🇷 Spasms involving the chest and breathing muscles can be severe and may require emergency medical treatment with ventilatory support. Spasms can last for several minutes, but most often last for hours. Sudden withdrawal of medication in individuals with PRS can result in a life-threatening situation with extremely severe muscle spasms. Sleep generally suppresses the frequency of contractions.

Causes

The exact cause of Stiff Person Syndrome is still is not known🇧🇷 Some studies in the medical literature indicate that it may be an autoimmune disease. the riots autoimmune are caused when the body's natural defenses — antibodies — act against "foreign" or invading organisms that begin to attack healthy tissue for unknown reasons.

What is rigid person syndrome, celine dion disease
Image: Medical News Today

Most of those affected have antibodies against glutamic acid decarboxylase, a protein in inhibitory nerve cells that is involved in the creation of the main inhibitory neurotransmitter called gamma-aminobutyric acid, or GABA. O GABA helps control muscle movement and prevent hyperexcitability in the brain and spine.

Symptoms of PRS can develop when the immune system mistakenly attacks certain neurons that produce glutamic acid decarboxylase, leading to a GABA deficiency in the body. Less commonly, individuals with SPR will have antibodies to amphiphysine, a protein involved in transmitting signals from one nerve cell to another.

affected individuals

Stiff Person Syndrome is an extremely rare condition, with exact incidence and prevalence unknown, although one estimate puts recurrence at approximately 1 in 1 million of people. The distribution of PRS between males and females indicates female predominance and usually becomes apparent sometime between 30 to 60 years old🇧🇷 However it has been reported that the disease occurs in children and older adults as well.

Treatment

Treatment of Stiff Person Syndrome is directed towards the specific symptoms that are apparent in each individual, which usually requires a multi approach, including non-drug interventions such as stretching, heat therapy, aquatic therapy, massage therapy, acupuncture, etc. Medications that are considered therapies targeting GABA, such as benzodiazepines, specifically diazepam e clonazepam, are used to treat muscle stiffness and episodes of spasms.

What is rigid person syndrome, celine dion disease
Image: Summit Health

Affected individuals may also try baclofen, usually administered in combination with benzodiazepines. Other medications reported to be beneficial in a small number of individuals include anticonvulsant medications, including vigabatrin, valproate, pregabalin e gabapentin🇧🇷 Remembering that so far there is no cure for Rigid Person Syndrome, and therapeutic intervention is always necessary to minimize symptoms.

Ever heard of this disease? Tell us in the comments!

See also:

What is eryspella🇧🇷 Understand Jair Bolsonaro's illness.

Source: Rare Diseases.

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