Lambert-Eaton syndrome is a rare and progressive disease affecting over two million people worldwide. It can cause muscle weakness by disrupting neuromuscular function. There are many treatments for Lambert-Eaton syndrome. However, their efficacy varies among patients.
Here, we will look closer at the treatment of Lambert-Eaton syndrome with different forms of therapy. From medications to surgery and IVIG infusions, we will introduce you to the most effective treatments for this disease to help you make an informed decision for your health.
What Is Lambert-Eaton Syndrome?
Lambert-Eaton syndrome is one of the many conditions IVIG can treat. It is also known as Lambert-Eaton myasthenic syndrome or LEMS.
In this autoimmune disorder, the immune system attacks the neuromuscular junctions between motor nerves and muscles. This area is the connection point between the neurons and muscles, where the nerve impulses are transmitted to the muscle fibers to cause contraction.
By affecting this area, LEMS disrupts the signals and weakens the muscles. Therefore, treatments for Lambert-Eaton syndrome focus on resolving this disruption and restoring connection.
People at Risk of Getting LEMS
Some groups of people are more susceptible to developing Lambert-Eaton syndrome than others.
One of these groups is older people (around 60 years old) with an underlying cancer like small-cell lung carcinoma. LEMS in this group is seen more often among men with a history of smoking. Symptoms may start before cancer diagnosis.
Another group more likely to seek the treatments of Lambert-Eaton syndrome is people with no underlying malignancies. In this group, LEMS peaks once around age 35 and another time at 60, affecting both sexes equally.
Symptoms of Lambert-Eaton Syndrome
The type of treatment for Lambert-Eaton syndrome will depend on the patient’s symptoms. Some of the most common symptoms of LEMS include:
- Fatigue
- Dry eyes
- Dry mouth
- Weight loss
- Muscle pain
- Double vision
- Drooping eyelids
- Trouble walking
- Muscle stiffness
- Trouble speaking
- Trouble breathing
- Muscle weakness
- Erectile dysfunction
- Difficulty swallowing
- Dizziness upon standing
- Bladder and bowel problems
- Tingling sensation in hands and feet
Causes of Lambert-Eaton Syndrome
As we already mentioned, Lambert-Eaton syndrome is an autoimmune condition where the body’s natural defenders mistake your healthy nerves as invaders and attack them.
Unfortunately, at this time, we do not fully know what triggers this response. It may be an effort by the body to fight an underlying cancer in people with small-cell lung carcinoma. However, it affects people without cancer as well.
Scientists working on treatments for Lambert-Eaton syndrome link this disease to genetics, but we still have a long way to go to understand the real cause of LEMS.
How Is Lambert-Eaton Syndrome Diagnosed?
Successful treatment of Lambert-Eaton syndrome depends on the correct diagnosis of the disease.
If you show any signs of this disease, your doctor will refer you to a neurologist. After a thorough physical examination and medical history evaluation, the neurologist will check your reflexes and perform the following tests:
- Blood tests – These tests will look for anti-calcium channel antibodies in your blood, which are common among people with LEMS.
- Electromyography – This test determines how well the signals from nerves reach your muscles.
- Scans – The doctor may perform imaging tests like CT or PET scans to see if you have small-cell lung cancer.
Treatments for Lambert-Eaton Syndrome
LEMS has no cure yet, but several treatments can alleviate its symptoms and help patients live a relatively normal life. Here are the most effective treatments for LEMS.
Cancer Treatment
Treatment of Lambert-Eaton syndrome depends on the patient’s underlying health conditions. Since 60% of LEMS patients have an associated small-cell lung cancer, the first approach is to treat the cancer.
Cancer treatment may include surgery, chemotherapy, radiation therapy, or a combination of all, based on your doctor’s opinion and the stage of your disease.
Chemotherapy targets cancer cells across the body, while radiation therapy targets specific areas, and surgery removes the cancerous tumor. By effectively treating the underlying cancer, LEMS symptoms can significantly improve.
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Nerve Signal Boosting Medications
Another line of treatment for Lambert-Eaton syndrome is medications that improve signal transmission from the nerves to the muscles. Some of the most common medications for this purpose include:- Amifampridine (3,4-diaminopyridine or 3,4-DAP) – This medication works by blocking the presynaptic channels and increasing calcium influx, which leads to increased acetylcholine (ACh) release (an impaired function in people with LEMS).
- Pyridostigmine – This medication increases ACh signaling by inhibiting its breakdown at neuromuscular junctions.
- Guanidine – This drug enhances ACh release by binding to voltage-dependent calcium channels at nerve endings. It increases calcium influx and triggers ACh release by keeping the channels open longer.
Immunosuppressants
Effective treatment of Lambert-Eaton syndrome is also possible with immunosuppressants. Doctors usually turn to this type of treatment for Lambert-Eaton syndrome when the above approaches did not work effectively. As the name suggests, immunosuppressants suppress the immune system’s defensive mechanisms. By dampening these immune responses, immunosuppressants restore muscle strength and alleviate other symptoms of LEMS. Some of the most common immunosuppressants used to treat LEMS include:- Prednisone – This medication is a corticosteroid that is also a treatment option for myasthenia gravis.
- Azathioprine – This immunomodulator is an effective option that is also useful for treating rheumatoid arthritis.
- Methotrexate – This antimetabolite immunosuppressant is also a chemotherapy agent used to treat cancer.
Plasma Exchange
Another type of treatment for Lambert-Eaton syndrome is plasma exchange or plasmapheresis. This procedure involves replacing plasma containing harmful antibodies with healthy plasma. A specialist will pass your blood through a machine to separate the plasma from the blood cells. After filtering out the harmful antibodies attacking your neuromuscular junctions, they will return the healthy blood to your body. Plasmapheresis alleviates LEMS symptoms by getting rid of the antibodies that are contributing to the condition. Your healthcare provider may consider plasma exchange if your symptoms are too severe. For effective treatment of Lambert-Eaton syndrome, you should undergo this procedure several times regularly based on the severity of your condition to keep your antibody levels in check.Immunoglobulin Therapy
Finally, intravenous immune globulin, or IVIG therapy, can be an effective treatment for Lambert-Eaton syndrome. IVIG is the primary form of therapy for refractory cases of LEMS that have not responded to other medications. IVIG treatment involves infusing patients with antibody solutions derived from the blood of healthy donors. The goal is to replenish the patient’s immune system with enough healthy antibodies to combat the attack on neuromuscular junctions and improve connections. Depending on a patient’s severity of symptoms and response to treatment, they may receive IVIG on a short or long-term basis. The typical regimen involves administering 2 g/kg over 2-5 days.What Is the Best Treatment for Lambert-Eaton Syndrome?
Treatment of Lambert-Eaton syndrome varies based on the patient and the severity of their disease. However, the first line of treatment for most patients is nerve signal-boosting medications, specifically 3,4-diaminopyridine. Some consider this the best and most effective form of treatment for LEMS, as it is generally well-tolerated by most patients and can significantly improve muscle strength. However, responses to this medication will vary, and patients with more severe symptoms may require more intensive treatments. In these cases, the best treatment for Lambert-Eaton syndrome may be a combination of medications, IVIG, and plasmapheresis. Patients with LEMS must work closely with their healthcare providers to determine the best treatment for their condition that meets their health needs.Speak to a Specialist About Copay Assistance
Living with Lambert-Eaton Syndrome
Muscle weakness caused by Lambert-Eaton syndrome can be so severe that it can make routine activities such as walking or climbing the stairs difficult. However, you can still maintain a good quality of life using the following tips:- Stay hydrated and follow a healthy diet
- Exercise regularly to maintain strength and mobility
- Avoid strenuous activities that can cause muscle fatigue
- Wear comfortable clothes that fit well to reduce the risk of falling
- Use assistive devices like walkers, canes, or wheelchairs to move around more easily
- Learn all you can about different forms of treatment for Lambert-Eaton syndrome and discuss them with your doctor
- Seek help from family, friends, and support groups to maintain a positive outlook
- Identify and avoid triggers that worsen your symptoms (stress, certain medications, etc.)
Can You Prevent Lambert-Eaton Myasthenic Syndrome?
Since scientists do not know the exact cause of Lambert-Eaton syndrome, there are no sure ways of preventing it. However, this disease is associated with small-cell lung cancer in most people. You can potentially lower your chances of developing LEMS by following these tips:- Quit smoking
- Avoid second-hand smoke
- Adopt healthier lifestyle habits
- Check your home routinely for radon