Lambert- Eaton Myasthenic Syndrome
Lambert-Eaton myasthenic syndrome (LEMS) is a very rare condition that affects the signals sent from the nerves to the muscles.
It means the muscles are unable to tighten (contract) properly, resulting in muscle weakness and a range of other symptoms.
About half of LEMS cases occur in middle-aged or older people with lung cancer. The remaining cases are not associated with cancer and can start at any age.
LEMS is also known as myasthenic syndrome or Eaton-Lambert syndrome.
Key Facts
- The worldwide prevalence is estimated to be between 1/250,000-1/333,300.
- It affects males more than females but more recent reports show almost equal occurrence in both sex.
- It is usually a condition of later adulthood but does present occasionally in childhood.
- LEMS is not inherited.
Symptoms
The symptoms of LEMS develop gradually over weeks or months.
The main symptoms are weakness in the legs, arms, neck and face, as well as problems with automatic body functions, such as controlling blood pressure.
Common symptoms include:
- aching muscles
- difficulty walking and climbing stairs
- difficulty lifting objects or raising the arms
- drooping eyelids, dry eyes and blurred vision
- swallowing problems
- dizziness upon standing
- a dry mouth
- constipation
- erectile dysfunction
- strength that temporarily improves when exercising, only to reduce as exercise continues
Diagnosis
The Doctor will first check your medical history, ask about your symptoms, carry out a physical examination, and test your reflexes.
If they think you have a problem with your nerves, they may refer you to a specialist called a neurologist for further tests to determine the cause.
Tests you may have include:
- blood tests – a blood test can detect substances in the blood (antibodies) resulting from the immune system attacking the nerves
- nerve studies – a needle may be inserted into your skin to check how well signals are reaching the muscles from the nerves
- scans – you may have a CT scan or PET scan to check for lung cancer
If initial scans do not find cancer, you may be advised to have regular scans every few months for a few years to check that it does not develop later on.
Causes
LEMS is caused by the body’s natural defences (the immune system) mistakenly attacking and damaging the nerves.
Normally, nerve signals travel down the nerves and stimulate the nerve endings to release a chemical called acetylcholine. This chemical then helps activate the muscles.
If the nerve endings are damaged, the amount of acetylcholine they produce decreases, which means nerve signals do not reach the muscles properly.
It is not known what triggers the immune system to attack the nerves. It is often associated with lung cancer but can occur in people without cancer.
There is currently no cure for LEMS, but a number of treatments can help reduce the symptoms.
These include:
- Treatment for lung cancer – if you have lung cancer, treating it can significantly improve the symptoms of LEMS
- Medicine to help nerve signals reach the muscles – commonly used medicines include 3,4-diaminopyridine and pyridostigmine
- Medicine to reduce the activity of the immune system (immunosuppressants) – commonly used medicines include steroid tablets (prednisolone, azathioprine and methotrexate
- Immunoglobulin therapy – injections of antibodies from donated blood that temporarily stop your immune system from attacking your nerves
- Plasmapheresis – a procedure to redirect your blood through a machine that filters out the antibodies attacking your nerves
Medicine is the main treatment, although immunoglobulin therapy and plasmapheresis may be recommended in the short term, or if muscle weakness is severe and other treatments have not helped.
Some people respond well to treatment and are eventually able to stop taking medicine, although this may not be for several years.
Others respond not as well and find the condition affects their everyday activities and quality of life.
LEMS does not affect life expectancy if it is not associated with cancer. But people with lung cancer and LEMS tend to have a shorter life expectancy because it is often not diagnosed until the cancer has spread, and by this point, it is very difficult to treat.
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Lambert- Eaton Myasthenic Syndrome
Lambert-Eaton myasthenic syndrome (LEMS) is a very rare condition that affects the signals sent from the nerves to the muscles.
It means the muscles are unable to tighten (contract) properly, resulting in muscle weakness and a range of other symptoms.
About half of LEMS cases occur in middle-aged or older people with lung cancer. The remaining cases are not associated with cancer and can start at any age.
LEMS is also known as myasthenic syndrome or Eaton-Lambert syndrome.
Key Facts
- The worldwide prevalence is estimated to be between 1/250,000-1/333,300.
- It affects males more than females but more recent reports show almost equal occurrence in both sex.
- It is usually a condition of later adulthood but does present occasionally in childhood.
- LEMS is not inherited.
Symptoms
The symptoms of LEMS develop gradually over weeks or months.
The main symptoms are weakness in the legs, arms, neck and face, as well as problems with automatic body functions, such as controlling blood pressure.
Common symptoms include:
- aching muscles
- difficulty walking and climbing stairs
- difficulty lifting objects or raising the arms
- drooping eyelids, dry eyes and blurred vision
- swallowing problems
- dizziness upon standing
- a dry mouth
- constipation
- erectile dysfunction
- strength that temporarily improves when exercising, only to reduce as exercise continues
Diagnosis
The Doctor will first check your medical history, ask about your symptoms, carry out a physical examination, and test your reflexes.
If they think you have a problem with your nerves, they may refer you to a specialist called a neurologist for further tests to determine the cause.
Tests you may have include:
- blood tests – a blood test can detect substances in the blood (antibodies) resulting from the immune system attacking the nerves
- nerve studies – a needle may be inserted into your skin to check how well signals are reaching the muscles from the nerves
- scans – you may have a CT scan or PET scan to check for lung cancer
If initial scans do not find cancer, you may be advised to have regular scans every few months for a few years to check that it does not develop later on.
Causes
LEMS is caused by the body’s natural defences (the immune system) mistakenly attacking and damaging the nerves.
Normally, nerve signals travel down the nerves and stimulate the nerve endings to release a chemical called acetylcholine. This chemical then helps activate the muscles.
If the nerve endings are damaged, the amount of acetylcholine they produce decreases, which means nerve signals do not reach the muscles properly.
It is not known what triggers the immune system to attack the nerves. It is often associated with lung cancer but can occur in people without cancer.
There is currently no cure for LEMS, but a number of treatments can help reduce the symptoms.
These include:
- Treatment for lung cancer – if you have lung cancer, treating it can significantly improve the symptoms of LEMS
- Medicine to help nerve signals reach the muscles – commonly used medicines include 3,4-diaminopyridine and pyridostigmine
- Medicine to reduce the activity of the immune system (immunosuppressants) – commonly used medicines include steroid tablets (prednisolone, azathioprine and methotrexate
- Immunoglobulin therapy – injections of antibodies from donated blood that temporarily stop your immune system from attacking your nerves
- Plasmapheresis – a procedure to redirect your blood through a machine that filters out the antibodies attacking your nerves
Medicine is the main treatment, although immunoglobulin therapy and plasmapheresis may be recommended in the short term, or if muscle weakness is severe and other treatments have not helped.
Some people respond well to treatment and are eventually able to stop taking medicine, although this may not be for several years.
Others respond not as well and find the condition affects their everyday activities and quality of life.
LEMS does not affect life expectancy if it is not associated with cancer. But people with lung cancer and LEMS tend to have a shorter life expectancy because it is often not diagnosed until the cancer has spread, and by this point, it is very difficult to treat.
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