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This disease sounds threatening, but there is reason for optimism - Walla! health

2022-01-02T06:05:58.129Z


People with rare myasthenia gravis are very anxious - they feel muscle weakness, sometimes it takes time to understand why, and their condition quickly worsens


This disease sounds threatening, but there is reason for optimism

People who suffer from the rare disease of myasthenia gravis are very apprehensive - they feel muscle weakness, sometimes it takes time to understand why, and their condition quickly worsens.

At the same time, advanced treatments allow for effective treatment and a return to full function

Walla!

Health in collaboration with the Maggie Association

29/12/2021

Wednesday, 29 December 2021, 14:14 Updated: 15:21

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Myasthenia gravis is a neuro-immunological disease that causes weakness and severe fatigue of the muscles. In many cases the disease is characterized by drooping of the eyelids, weight loss, fatigue in chewing, change in speech tone and muscle weakness of the shoulders and thighs. These symptoms can be frustrating as they affect almost every aspect of life, but the good news is that advanced treatments available today allow for a real return to routine and a high quality of life.



First, before treating the disease, it should be diagnosed. And unfortunately - it is sometimes not easy. Dr. Keren Regev, Director of the Multiple Sclerosis Service at the Neuroimmunology Unit in the Immunology Department at Ichilov Hospital, explains that since the disease is very rare, awareness of it is low. There are currently about 1,000 patients in Israel, so it is not surprising Just miss it.



"The symptoms are relatively non-specific to the disease, so a person who complains of symmetrical limb weakness or difficulty speaking - will not necessarily be suspected of having the syndrome," Dr. Regev explained, "it takes time to see a neurologist. "Until the appropriate tests are done, so there is often a diagnostic delay."

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In collaboration with the Maggie Association

Symptoms can vary from patient to patient (Photo: ShutterStock)

How is myasthenia gravis treated?

To understand the treatments for this rare disease, it is important to understand how it works. Myasthenia gravis usually occurs in two age groups: between the ages of 18 and 30 (then it occurs mainly in women), and between the ages of 60 and 80 (in both sexes). The disease that arrives at an earlier stage is usually a classic autoimmune disease, whereas the later wave is often associated with thymus tumors.



The main problem with myasthenia gravis is that antibodies are formed that prevent the proper transfer of the single between the nerve and the muscle. "Single transfer is based on the fact that a neurotransmitter (a molecule that activates the nervous system) called acetylcholine is secreted from the end of the nerve and binds to a receptor across the muscle - this is what happens properly. In myasthenia gravis there is a blockage of this passage He should be linked to acetylcholine, "Dr. Regev explained.



There are two possible approaches to overcoming the problem.

"One approach is to increase the concentration of acetylcholine within the space between the nerve and the muscle so that it can compete more effectively with antibodies. We do this by inhibiting the enzyme that breaks it down," Dr. Regev explained, adding that this enzyme inhibition causes concentration acetylcholine is larger, then more likely not to bind the antibody to the receptor. "this drug basically we give almost every patient myasthenia," she added.



when that's not enough, there is a need for preparations that inhibit the immune system itself. "here we have a few options: Steroids;

Treatments that suppress the immune system and are not steroids;

Plasma replacement - filter the patient's blood and remove the antibodies;

"To give a drug that is an extraction of antibodies from healthy people, which leads to a decrease in the antibody activity of the sick person."

There are also preparations that relieve pain (Photo: ShutterStock)

And what does one do that these drugs do not help?

"All the drugs used in Israel for myasthenia gravis are not indicated for this disease and are used based on treatment experience in Israel and around the world. 10-15 percent of patients with myasthenia gravis are resistant to these treatments, but there is also effective treatment for them, which is a candidate for the 2022 health basket. humane which inhibits the activation of the immune system that could lead to the destruction complementary membrane neuromuscular junction, "explained Dr. Regev.



and another medical option - removal of the thymus gland in some patients." thymus was associated with many cases of myasthenia " Dr. Regev explained, "Even for people whose thymus gland appears normal on imaging examination, research has found that thymus removal leads to clinical improvement after two years of follow-up." Therefore today, for people who are suitable for the study population, young people with a disease that is defined as general and not limited to the eyes,It is advisable to consult a breast surgeon and consider the benefits of thymus removal. "

In the end manage to achieve improvement

"The most important thing to remember is that myasthenia gravis is a disease that can be treated very effectively, it is not a progressive or degenerative disease, and patients are likely to experience improvement and return to function," Dr. Regev stressed. I always remind my patients that while they climb up slowly after they have deteriorated rapidly - but in the end they arrive. "



For more information



funded by Neoprem Israel as an independent donation



2021-DEC-914

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Source: walla

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