Bivek Pegu (35) from Assam visited his eye doctor with drooping eyelids and double vision when he was 26. “I initially thought it was due to a bad hangover that would get better the next day. But it got worse until everyone at work noticed something was wrong with my eyes,” he says. Pegu was then diagnosed with a rare neurological condition called myasthenia gravis.
“Myasthenia gravis is an autoimmune condition affecting the nerves and muscles jointly by impairing the connection between them. As a result, the body’s skeletal muscles become weak,” says Dr Anmol Singh Rai, consultant neurologist at Shrimann Super Speciality Hospital, Jalandhar.
He cites the condition of a middle-aged woman who came to him with extreme tiredness. She had difficulty concentrating, getting up from the floor and walking up the stairs.
“Her condition was particularly bothersome in the evening, and she couldn’t find an explanation for it even after six months,” says Dr Singh.
Puzzled by her symptoms, her gynaecologist attributed her tiredness to menopause. However, her eye doctor had noticed her drooping eyes and double vision and suspected she had myasthenia gravis.
Role of the thymus gland
The thymus is a small pyramid-shaped gland located just below the thyroid and above the breastbone. It remains large in people with myasthenia gravis. The gland is the centre where immune cells are produced. It is highly active even before birth and grows rapidly until puberty, after which it begins to shrink as by then most immune cells are produced and available.
However, the thymus remains unshrunk in people with myasthenia gravis, where the excessive cluster of immune cells clump together like tumours (called thymomas). A 2022 study found that thymomas play a role in the abnormal production of antibodies that attack muscle cells and how they respond to nerves, leading to this condition.
The chemical switch
Nerve impulses are minute electrical signals that originate in the brain and travel across the nerves to a junction where they meet the muscles. Here, the nerve cell endings release a chemical called ‘acetylcholine’ that initiates muscle movements. The muscles move only when acetylcholine binds to specific structures called receptors on the muscles. But in the case of myasthenia gravis, the enlarged thymus releases immune cells that block, alter or destroy these receptors. This mistaken attack by the body’s immune system causes a disconnection between the nerves and the muscles.
When the muscles refuse to cooperate
According to a 2022 review article, the feature that makes myasthenia gravis stand out from other conditions that cause tiredness like anaemia, diabetes or muscle injury, is the fluctuating muscle weakness. Muscular weakness worsens with physical activities like climbing up stairs and gets better with rest.
Moreover, it affects the muscles in the throat making it difficult to chew and swallow food. “He would be fine during his morning breakfast but faced difficulty finishing his dinner because by evening, he was usually fatigued out,” says Dr Singh about a man who consulted him. He switched to liquids and soft food as he found it challenging to chew them. “This is because his facial muscles were particularly affected,” explains Dr Singh.
Other factors like infections, emotional stress, pregnancy, surgery, and certain medications can also play a role, he adds. The 2022 study also highlights subtle signs like coughing while or after swallowing food, taking time to finish eating and hoarseness, which are usually overlooked.
Slow writing or typing and frequent mistakes are the most prominent signs of myasthenia gravis. Weakness at the shoulders and hips can manifest as difficulty in getting up from a sitting position, climbing up the stairs, and raising the arms or hands, adds Dr Singh.
Is there a way out?
“Currently, myasthenia gravis has no known cure; however, early diagnosis and managing the condition with lifestyle changes can significantly improve the quality of life,” says Dr Singh.
Certain medications suppress the immune system and prevent the immune proteins from binding to the muscle receptors. A few other drugs help improve the communication between the nerves and muscles and stop the aberrant immune response that destroys the muscle receptors.
Dr Singh says that treatments like ‘plasmapheresis’ – a type of blood transfusion where the blood plasma is replaced from a donor — help remove the immune cells responsible for damaging the receptor sites. “These therapies can help decrease muscle weakness temporarily,” he says. A surgical option is removing the thymus gland, he adds.
Pegu has been managing his condition with a combination of medication and lifestyle management like exercise, avoiding strenuous activities, taking smaller meals, and eating a healthy diet, including plenty of fruits and vegetables. Dr Singh explains that avoiding excess stress and heat and staying adequately hydrated can prevent over-exhaustion. “Getting enough rest gives muscles a chance to recover,” he adds.
Making a difference
Pegu runs an online support group called Myasthenia Gravis India and conducts camps to bring awareness among the people.
During a campaign held on 3 December, observed as the International Day of Disabled Persons, Pegu came across many people with myasthenia gravis who had hidden their condition from their family members and peers. “More than half of the people refused to join the campaign because of the stigma associated with it,” he says.
Support groups for people with this condition are rare. “Newly diagnosed people have a hard time accepting their condition. Moreover, the treatments are expensive; therefore, this condition must be recognised to get assistance from society,” he says.
He appeals to people with the condition to accept and connect with support groups. “Such groups can share personal experiences and coping strategies to manage the symptoms and lead a happy life,” says Pegu.
