Myasthenia Gravis Complications, Diagnosis and Treatments


What complications can occur with myasthenia gravis?

Myasthenic crisis:

When the muscles that control breathing are affected, it leads to a situation called myasthenic crisis where the breathing muscles start getting paralysed. This is a life threatening emergency and patient needs to be immediately intubated and connected to a ventilator to save life. Even if the ventilator is not available, putting an endotracheal tube (tube in the windpipe) and ventilating with a bag may save life, while the patient is being transported to a health facility with ventilator. On numerous occasions we have seen catastrophic situations and lives being lost on way to the hospital.

Tumors of thymus gland (thymoma):

In 10%-20% of patients, myasthenia gravis may be associated with a tumor of thymus gland called Thymoma. This can be suspected on a chest x-ray and confirmed by a CT scan. Although slow-growing in nature, the thymomas go on increasing in size if left untreated and have the potential to invade nearby structures like lining over the heart (pericardium), the nerve of the diaphragm (phrenic nerve), the veins and arteries around the heart (superior vena cava, aorta and pulmonary artery) and the lungs on either side. In late stages, it may spread to both chest cavities and may spread elsewhere in the body also.

Thyroid and other autoimmune diseases:

Patients of Myasthenia Gravis have a high incidence of thyroiditis, SLE, rheumatoid arthritis and other autoimmune diseases and need to be watched and treated for the same. Thyroid gland hormone overproduction or underproduction can further complicate the symptoms of Myasthenia Gravis.

How is myasthenia gravis diagnosed?

Physical Examination:

The doctor checks the muscle strength, muscle tone and reflexes and discovers weakening of the muscle action on repetitive action.

Blood Test:

Acetylcholine receptor antibody test is always done to check for presence of circulating antibodies in the system.

Edrophonium Test:

In this test, injections are given to check if the muscles gain their strength on injecting this chemical. If it results in immediate improvement in the muscle strength, it indirectly confirms the diagnosis of Myasthenia Gravis.

Electromyography (EMG):

This test involves insertion of an electrode to measure the electrical activity between the nerves and the muscles and it will show normal conduction of impulses through the nerve but a blockade occurring at the level of nerve–muscle junction.

CT Scan:

A CT scan of the chest is done to look for any tumor in the thymus gland.

How is Myasthenia Gravis treated?

There is no one-time magic cure for Myasthenia Gravis. However, with proper treatment, the condition can be controlled and managed enough to be able to lead a normal life. The treatment options are following:

Medications:

Cholinesterase Inhibitors:

It is a group of medicines which blocks the enzyme cholinesterase leading to better transmission of impulses are neuromuscular junction. These form the mainstay of treatment but need to be taken on a regular basis several times a day.

Steroids:

Steroids are sometimes given to limit the antibody production.

Immunosuppressive Agents:

Immunosuppressive agents are also used to decrease the antibody production thereby leading to improvement of symptoms.

Surgery (Removal of thymus – thymectomy):

For patients who have developed Thymoma, surgical removal of the thymus gland (thymectomy) is mandatory, otherwise the tumor will continue to grow and threaten life.

In patients without Thymoma, the role of surgery has been an area of intense debate. In older times, the surgery (thymectomy) was done by cutting open the breastbone (sternotomy), which involved a lot of trauma on the body, prolonged hospital stay, long time to recovery and left an unsightly scar in the area between the breasts. With young female patients forming a significant number of Myasthenia Gravis patients, this was a big hindrance to acceptance of surgery as a treatment option. However, the development of Keyhole surgery (VATS or Thoracoscopic thymectomy in the 1990s) and the applications of Robotic assistance (Robot-assisted thymectomy) has made things simpler and less invasive.

Robotic thymectomy involves making 3 holes (two 8 mm and one 12 mm) on one of the sides of the chest i.e. left or right and the entire gland from the root of the neck up to diaphragm and from one phrenic nerve to the other can be removed completely and safely. The patients undergoing robotic thymectomy are usually awoken out of anesthesia (extubated) on the table itself at the end of the procedure and usually do not need to go to the ICU. In our experience of over 100 such procedures at the All India Institute of Medical Sciences and Sir Ganga Ram Hospital, New Delhi, the patients were able to take a walk in the corridor the same evening, the chest tube was removed the next morning and the patients were discharged usually at 48 hours after surgery. More importantly, the patients are able to return to their work within a maximum of one week after the surgery, a huge-huge improvement over at least 2-3 months needed for the same after thymectomy by sternotomy.

Moreover, our experience at Centre for Chest Surgery and Institute of Robotic Surgery at Sir Ganga Ram Hospital as well as experience of other groups around the World; notably Dr. Jens C. Ruckert and his group at Berlin has shown that after robotic thymectomy over three-fourths of the patients experience improvement in their Myasthenia Gravis status – which may vary from complete remission (no medicines needs and no symptoms at all) to minimal improvement i.e. medications reduced or improved response. One fourth of the patients may not experience any benefit. The patients with Thymoma (especially those with invasive Thymoma) experience less benefit after robotic thymectomy than those without Thymoma. The best response is seen in younger patients, female patients, those who are operated within 1 year of diagnosis, and in those who has a swollen thymus gland (hyperplasia). Robotic thymectomy is offered routinely to patients of Myasthenia Gravis with or without Thymoma at Centre for Chest Surgery and Institute of Robotic Surgery at Sir Ganga Ram Hospital, New Delhi. We have country’s largest experience in minimal invasive thymectomy (VATS or Thoracoscopic thymectomy from 2000 to 2008 and Robotic Thymectomy from 2008 till date). Robotic thymectomy is safe, effective in over three-fourths of the patients, involves short hospital stay of about 48 hours and allows the ability to return to work within a week or 10 days after the surgery.

Long-term Followup:

Patients with Myasthenia Gravis need to be on long-term followup and are known to develop exacerbation of symptoms during periods of physical stress (including episodes of viral fever etc) as well as emotional stress. The dosages of drugs need adjustment according to the severity of symptoms. It is very important to be regular with medicines and to be under the follow-up of an expert in the field of Myasthenia Gravis.


Dr Arvind Kuamr


Comments for “Myasthenia Gravis Complications, Diagnosis and Treatments”

  1. Reply

    plz tell me the cost of myesthesiya gravis in total. my father also patient of mysthesiya gravis.

    Megharaj Dahal,
    • Reply

      For appointments, please call at 011-42252328.

      drarvind,

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