Myasthenia gravis 101: Your guide to a complex medical condition

January 7, 2022

This medical condition, which occurs primarily in young women and older men, causes severe muscle weakness.

People diagnosed with myasthenia gravis suffer from several disconcerting symptoms. They get tired when they chew food, their eyelids tend to droop and sometimes even their tone of speech changes due to muscle weakness. The good news is that there are treatments which help to alleviate symptoms. Here’s everything you need to know about this disease.

Myasthenia gravis, or in Hebrew “tired muscle”, is a deceptive disease. It causes weakness and severe muscle fatigue, but our lives are so busy that many people attribute this unusual group of symptoms to overload or cumulative fatigue. But really, it’s a neuro-immunological disease that’s very important to recognize, diagnose and treat quickly, as it can greatly disrupt life and cause great suffering to those affected by it and their families.

What is myasthenia gravis?
“The word myasthenia gravis means acute weakness in Latin,” explained Dr. Shahar Shelly, a specialist in neurology, neuromuscular diseases and peripheral neuroimmunology in the neurology department at Sheba Medical Center.
“It’s actually a clinical syndrome, part of an autoimmune disease in which the immune system attacks the body due to the self-production of antibodies.”

These antibodies, explained Shelly, bind to a protein (the receptor in the muscle cell) and this protein is indirectly responsible for nerve conductivity and is located on the wall of the muscles, at the points of connection between the motor nerve and the muscle.

What are the symptoms?
The clinical symptoms in most patients begin with drooping of the eyelids, especially in the evenings, accompanied by double vision. Often there is also weight loss, fatigue while chewing and coughing after drinking fluids.There may be a change in speech tone, difficulty breathing while lying down and also drooping of the head towards the end of the day. The person afflicted seems to bend their head slightly, as the neck muscles get tired of holding the head upright, explained Shelly. There is also weakness of the limb muscles like the shoulders and the thighs, especially after doing even moderate physical activity. When people with MG get up in the morning, they feel normal, but towards the end of the day the weakness is noticeable.
These symptoms create a diagnostic difficulty. “Because we all actually get tired at the end of the day and we all live a very intense lifestyle, it’s very difficult to suspect that anyone who claims to be tired at the end of the day has myasthenia gravis,” said Shelly. It’s therefore very important to be aware of the disease and pay attention to drooping of the eyelids, double vision, sudden weight loss, difficulty swallowing or the neck drooping.

How is it diagnosed?
Shelly explained that “the disease is diagnosed by neurologists who specialize in muscular or neuromuscular diseases. The diagnosis is clinical, according to the patient’s medical history and is combined with antibody blood tests and neurotransmitter testing (EMG) by an experienced neurologist.” He added that through various tests, what the patient looks like, blood tests and the neurotransmitter test it’s possible to diagnose this disease. It’s important that these patients also undergo a computerized imaging of the chest to look for an enlargement of the thymus.
However, Dr. Shelley noted that many patients with myasthenia gravis find it difficult to get a diagnosis. He says that it’s crucial to raise awareness of this disease, because people who suffer have trouble getting diagnosed.
“I frequently see patients who haven’t yet been diagnosed for three or four years, which is a serious problem since every year that goes without treatment, patients have difficulty being treated so that they can return to normal functioning.”

Who is at risk for developing MG?
In Israel, there are at least 800 to 1000 myasthenia gravis patients. “It’s a disease of young women and older men. It can occur in women aged 18 to 30, and in men especially over the age of 50,” explained Shelly.

How is it treated?
The disease has treatments that mainly suppress the immune system such as steroids or other drugs that “press on the brakes of the immune system.”
Shelly explained that “because it’s an autoimmune disease, these drugs in all sorts of indirect or direct ways contribute to a decrease in the amount of auto-antibody production. These treatments have been proven to be effective and they allow for a return to a life routine alongside the disease.”
However, in 10 to 15% of patients the disease is resistant to treatment, so they need other solutions. “There are patients who don’t respond to standard treatment and their condition continues to worsen,” said Shelly. He added that in these cases, there’s a new treatment which the government is considering adding to the basket of drugs paid for through the national health funds. It has been very effective for patients in Israel and worldwide. In Israel, there are 12 patients who reported a significant improvement in their condition with this new treatment.

Simply put, the new treatment works in a unique way by “preventing the activation of a defective mechanism in the immune chain. In fact, the drug attaches to the inflammatory conductor and stops it. Like a dam in the middle of a river, you prevent further problems downstream,” explained Shelly, adding that because it is a disease that causes muscle weakness, can affect the swallowing muscles, respiratory muscles, limb muscles, and even cause life-threatening respiratory failure, it is important to hear more about treatment options and to know about medications that can change patients’ lives.

Does the disease increase the risk of other diseases?
Many myasthenia gravis patients have a tendency to develop other autoimmune diseases. There is also a benign cancer most often in the breast called thymoma, so those diagnosed should also do a chest CT to rule out a tumor. Also, it is now known that thymus removal helps to gain control of the disease.

Shelly explained, “For years researchers didn’t understand the link between the diseases, but recently the explanation is that thymus damage causes the development of an autoimmune disease, such as myasthenia gravis.”

Sponsored by Neoprem Israel as an independent column.

Published: JANUARY 6, 2022 The Jerusalem Post, By WALLA! In partnership with MGI

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