Myasthenia Gravis: Treatment Options and Blood Washing Explained

Myasthenia Gravis

My family member (male, 27 years old) recently had difficulty opening one of his eyelids and was diagnosed at the hospital with a condition called "myasthenia gravis." Initially, the hospital prescribed medication, and his condition gradually improved.
However, after more than a month, not only did his eyelid become even more difficult to open, but he also experienced double vision and drooping.
Upon returning for a follow-up, the hospital determined that his condition had worsened, and the doctor recommended a procedure called plasmapheresis for treatment.

I found discussions online regarding this disease, and most treatments mentioned involve thymectomy, with very few discussions about plasmapheresis.
We discussed this with the doctor, who stated that only patients with thymoma require such surgery, so we should first proceed with plasmapheresis treatment.

When people think of plasmapheresis, they often associate it with dialysis.
I would like to ask if this treatment is truly effective for my family member's condition.
Is plasmapheresis the same as dialysis? Are there any risks involved? What does the entire treatment process entail? Will he need to rely on plasmapheresis to manage his condition like dialysis patients do? Are there any other treatment options available for this disease?

An, 20~29 year old female. Ask Date: 2008/07/23

Dr. Zeng Kongyan reply Neurology

Dear Ms./Mr.
An,
Myasthenia gravis is caused by a problem in the body's autoimmune system, where acetylcholine antibodies, which should not be present in the body, occupy the binding sites at the neuromuscular junction, leading to a sensation of muscle weakness.
There are many potential causes, with thymoma being the most common.
Certain medications, such as antibiotics or those used to treat rheumatoid arthritis, can also trigger the condition.
The letter mentions that thymectomy is indeed a surgical procedure performed when the cause is due to a thymoma; if the cause is not a thymoma, then resection is not necessary.
In the early stages of the disease, if the condition worsens, plasmapheresis (commonly referred to as "blood washing") must be used as treatment.
This involves replacing the patient's plasma with immunoglobulin or plasma from another individual, aiming to reduce the acetylcholine antibodies in the patient's body and restore the function of the neuromuscular junction.
This procedure is similar to dialysis; however, if the patient's condition improves after five treatments, medication can be used for management, and further plasmapheresis may not be necessary.
Plasmapheresis can be used again in cases of acute exacerbation.
Your family member's physician is a qualified professional, and the treatment being used is appropriate.
Please discuss this further with them.
Thank you for your letter, and I wish you peace, health, and well-being.
Sincerely,
Kong-Yen Tseng
New Tainan Hospital, Department of Health

Reply Date: 2008/07/23

More Info

Myasthenia Gravis (MG) is a chronic autoimmune neuromuscular disorder characterized by varying degrees of weakness of the skeletal muscles, which are responsible for breathing and moving parts of the body. The hallmark of MG is muscle weakness that worsens after periods of activity and improves after periods of rest. The condition is caused by an error in the transmission of nerve impulses to muscles, primarily due to the body’s immune system producing antibodies that block or destroy nicotinic acetylcholine receptors at the neuromuscular junction.

In your case, your family member is experiencing worsening symptoms, including eyelid drooping (ptosis) and diplopia (double vision). The initial treatment usually involves medications such as anticholinesterase agents (like pyridostigmine) that help improve communication between nerves and muscles. However, in some cases, as you've noted, the condition may not respond adequately to medication alone, leading to the consideration of more aggressive treatments.

Plasmapheresis (Blood Washing)
Plasmapheresis, often referred to as "blood washing," is a procedure that removes plasma from the blood and replaces it with a substitute. This treatment can be effective in managing severe exacerbations of MG, particularly when rapid improvement is needed, such as in cases of myasthenic crisis or when patients are not responding to conventional therapies. The process involves the following steps:
1. Blood Collection: Blood is drawn from the patient.

2. Separation: The blood is processed through a machine that separates the plasma from the blood cells.

3. Replacement: The plasma is replaced with a substitute solution (such as saline or albumin) and returned to the patient.

Plasmapheresis can help reduce the levels of circulating antibodies that are interfering with neuromuscular transmission, leading to temporary improvement in muscle strength. However, it is important to note that this is not a permanent solution; the effects typically last for a few weeks to months, and patients may require repeated treatments.

Comparison with Dialysis
While both plasmapheresis and dialysis involve the removal of substances from the blood, they serve different purposes. Dialysis is primarily used to remove waste products and excess fluid from the blood in patients with kidney failure, while plasmapheresis is aimed at removing specific antibodies or harmful substances from the plasma.
Risks and Considerations
Plasmapheresis is generally considered safe, but like any medical procedure, it carries some risks, including:
- Infection at the access site
- Allergic reactions to replacement fluids
- Low blood pressure
- Electrolyte imbalances
It is crucial to discuss these risks with the healthcare provider to ensure that the benefits outweigh the potential complications.

Other Treatment Options
In addition to plasmapheresis, there are other treatment options for Myasthenia Gravis:
1. Immunosuppressive Therapy: Medications such as corticosteroids (prednisone) and other immunosuppressants (azathioprine, mycophenolate mofetil) can help reduce the immune response that is damaging the neuromuscular junction.

2. Thymectomy: Surgical removal of the thymus gland can be beneficial, especially in patients with thymoma or generalized MG. It can lead to remission or improvement in symptoms for some patients.

3. Monoclonal Antibodies: Newer treatments, such as rituximab or eculizumab, target specific components of the immune system and have shown promise in treating MG.

4. Lifestyle Modifications: Patients are often advised to manage stress, avoid extreme temperatures, and get adequate rest, as these factors can exacerbate symptoms.

In conclusion, plasmapheresis can be an effective treatment for acute exacerbations of Myasthenia Gravis, but it is not a cure. Ongoing management may involve a combination of therapies tailored to the individual’s needs. It is essential to maintain open communication with the healthcare team to monitor the condition and adjust treatment as necessary. If there are concerns about the diagnosis or treatment plan, seeking a second opinion from a neurologist specializing in neuromuscular disorders may also be beneficial.

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