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What to Know About Lambert-Eaton Myasthenic Syndrome?

Medically reviewed by Sandeep Dhanyamraju

Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune disorder affecting signals transmitted from the nerves to the muscles. The syndrome is caused by a mutation in the genes of the proteins responsible for muscle contractions. The syndrome occurs in the presence of malignant tumors (stomach, bronchi, ovaries, prostate, colon (colorectal cancer)) and autoimmune processes in the body (autoimmune thyroiditis, rheumatoid arthritis, Sjögren’s disease, systemic lupus erythematosus, etc.). The pathogenetic substrate of the syndrome is autoimmune mechanisms.

Generation and sex distribution Lambert-Eaton disease is close to myasthenia (M), said to the genetic association with HLA-B8-DR3. Increased risk of autoimmune diseases was detected in patients with Lambert-Eaton myasthenic syndrome and their family members. The first description of Lambert-Eaton syndrome dates from 1953. The condition was discussed in detail in 1957 by the American neurophysiologist Lambert and neurologist Eaton. It was later named after them.

 

What is Lambert-Eaton Syndrome?  

The disease is a myasthenic syndrome associated with autoimmune and malignant tumor processes.  It is characterized by variable muscle weakness and autonomic nervous system dysfunction, meaning that the muscles cannot contract properly, leading to muscle weakness and other symptoms often associated with small cell lung cancer (SCLC).

What is lambert-eaton syndrome

Its approximate frequency is 0,48 cases per 1 million population. However, for example, in the Netherlands, this figure is 0,78 cases and correlates with the prevalence of 3,42 cases per 1 million in the total population. It affects people between the ages of 20 and 70. Most often, their age exceeds 40 years. Initially, the syndrome was detected in males.

 

According to various experts, the disease occurs as a paraneoplastic syndrome in most affected people (50 to 75% of the total). 

  • Most of them are men (about 70%). 
  • In women, it is rare (up to 20%). 
  • Most of the tumors (80%) detected in Lambert-Eaton syndrome are caused by small cell lung cancer. 

In this case, myasthenic syndrome can occur several years before the tumor is formed. Occasionally, Lambert-Eaton syndrome is accompanied by other paraneoplastic syndromes, particularly paraneoplastic polyneuropathy.

 

What are the Main Causes of Lambert-Eaton Syndrome?

The main factors in developing LEMS may be malignant neoplasia or an autoimmune process. In autoimmune diseases, the immune system mistakes your body for a foreign object. Your immune system produces antibodies that attack your body.

  • Your body attacks the nerve endings that control the amount of acetylcholine. Acetylcholine is a neurotransmitter that causes muscle contractions. Muscle contractions allow you to make voluntary movements such as: walking, wiggling your fingers, and shrugging.
  • Specifically, your body attacks the voltage-gated calcium channel protein (VGCC). VGCC is required for the release of acetylcholine. You do not produce enough acetylcholine with VGCC injection, so your muscles cannot work properly.
  • Many cases of Lambert-Eaton myasthenic syndrome (LEMS)  are linked to lung cancer. Researchers believe that cancer cells produce the protein VGCC. This causes your immune system to produce antibodies against VGCC. These antibodies then attack both cancer cells and muscle cells. Anyone can develop LEMS  in life, but lung cancer may increase the risk of developing this disease. You may be at higher risk if your family has a history of autoimmunity.

 

What are the Lambert-Eaton Myasthenic Syndrome Symptoms?

What are the lambert-eaton myasthenic syndrome symptoms

Symptoms may include:

  • dry mouth; 
  • excessive fatigue and muscle weakness;
  • soreness in the upper arms and thighs;
  • leg weakness and instability of movement;
  • a paralytic “duck-like” walk;
  • reduced tendon reflexes;
  • drooping eyelids;
  • erectile dysfunction may occur in men.

When examining patients with Lambert-Eaton disease, the majority (90%) have antibodies to calcium channels, which consist in the structure of motor nerve fiber endings and tumor cells. The autoimmune attack targets the presynaptic membrane of the neuromuscular synapse. Its disruption results in a decrease in the release of the neuromuscular transmission mediator acetylcholine, which disrupts the passage of excitation from the nerve fiber to the muscle. This process manifests in the body the main symptoms of the syndrome: excessive fatigue and muscle weakness. 

 

As the myasthenic disease progresses, you will also experience:

  • When the patient is interviewed, they complain of leg weakness and instability of movement, which is more pronounced when walking and climbing stairs for long periods of time;
  • Patients with Lambert-Eaton disease tend to have a paralytic “duck-like” walk;
  • Tendon reflexes are reduced;
  • Lambert-Eaton syndrome has a definite increase in muscle strength during movement, which distinguishes it from myasthenia gravis, where muscle weakness increases with movement.

 

Accompanying symptoms may also include:

  • discomfort in the back and neck;
  • paresthesias in the distal limbs;
  • myalgia;
  • decreased vision acuity;
  • various autonomic disorders, such as decreased lacrimation, dry mouth, orthostatic arterial hypotension, and distal hyperhidrosis.

In some cases, upper eyelid drooping is found. Rarely impaired swallowing, double vision, and myasthenia gravis movement disorders have been noticed.

 

How to Treat Lambert-Eaton Syndrome?

How to treat lambert-eaton syndrome

Anticholinesterase drugs, corticosteroids and cytostatics are used to treat the disease. Therapeutic plasmapheresis is carried out. Lambert-Eaton myasthenic syndrome is a rather complex disorder with many inherent symptoms. For this reason, it is treated as an inpatient condition. The treatment of Lambert-Eaton syndrome depends on what caused it:

  • If the origin of Lambert-Eaton syndrome is due to the manifestation of a malignant tumor, therapy is primarily aimed at eliminating it. When the cancer is treated positively, the symptoms of Lambert-Eaton syndrome are also reduced.
  • If the origin of the disease is autoimmune, symptomatic treatment is carried out. For this purpose, drugs (most often glucocorticosteroids) that suppress the immune system are used. Plasmapheresis procedures filter the autoantibodies circulating in the blood.
  • Experts suggest taking a medication called acetylcholinesterase inhibitors, which promote this through the accumulation of acetylcholine in the synapse. It is needed to restore nerve impulse conduction along the neuromuscular synapse. These include pyridostigmine and ipidacrine. 
  • Lambert-Eaton syndrome therapy often uses healthy human immunoglobulin, administered intravenously to the patient. Potassium preparations are prescribed to improve muscle contraction, which also positively influences the passage of the nerve impulse. Cytostatics and hormones are used in the treatment process to reduce the severity of the destructive processes in the human body.

 

Schedule a Consultation with Lone Star Neurology 

At Lone Star Neurology, we have highly qualified doctors with years of experience helping patients facing neurological issues, particularly with LEMS diagnosis. We have a highly experienced neurologist staff who are both professional and compassionate, and empathetic at the same time. So, the patient’s state of health can be made close to normal by removing antibodies from the blood after treatment. 

Our doctors recommend that patients exercise and take walks outdoors. It is essential to follow the treatment regimen prescribed by the doctor, take the medication on time, and observe the doses recommended by the neurologist. 

 

FAQ

  • What is the difference between myasthenia gravis and Lambert-Eaton syndrome?

Eaton-Lambert syndrome is caused by antibodies that interfere with the release of the neurotransmitter acetylcholine. While myasthenia gravis leads to muscle weakness and myasthenic crises. This distinguishes Eaton-Lambert syndrome from myasthenia gravis. 

  • How long can you live with Lambert-Eaton syndrome?

It is vital for myasthenia gravis patients to monitor their health and to treat comorbid conditions in good time and prevent their progression. If all conditions are fulfilled, living to a fairly advanced age is possible.

  • How is Lambert-Eaton myasthenic syndrome diagnosed?

The LEMS diagnosis is made by a neurologist based on the examination of the patient, a neurological examination and the results of an electroneuromyography.

  • Is Lambert-Eaton myasthenic syndrome progressive?

The Lambert Eaton disease can be progressive without proper treatment and care. To maintain a normal body, a person with myasthenia gravis should maintain a healthy lifestyle, a proper diet, regular physical exercise, adequate rest, and try to avoid stress.

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Edward Medina
Edward Medina
15:34 30 Jun 22
Just such an amazing staff that makes you feel like part of their family. I’ve been going there for over 5 years now... and each visit I get the very best care and treatments that I have ever received in the 20+ years that I’ve been dealing with severe debilitating migraines. Since i started seeing them the number of my migraines has dropped from 15-20 a month to 2-3 every 3 month. I highly recommend them …they will change your life!read more
Daneisha Johnson
Daneisha Johnson
22:20 19 May 22
Dr. Askari was very kind and explained everything so I could understand. The other staff were nice as well. I would... have gave 5 stars but I was a little taken aback when I checked in and had to pay 600.00 upfront. I think that should have been discussed in a appointment confirmation call or email just so I could have been prepared.read more
Jean Cooper
Jean Cooper
16:54 29 Apr 22
I love the office staff they are friendly and very helpful. Dr. JODIE is very caring and understanding to your needs... and wants to help you. I will go back. would recommend Dr. Dr. Jodie to other Patients in a heart beat. The team works well together.read more
Linda M
Linda M
19:40 02 Apr 22
I was obviously stressed, needing to see a neurologist. The staff was so patient and Dr. Ansari was so kind. At one... point he told me to relax, we have time, when I was relaying my history of my condition. That helped ease my stress. I have seen 3 other neurologists and he was the only one who performed any assessment tests on my cognitive and physical skills. At one point I couldn't complete two assessments and got upset and cried. I was told, it's OK. That's why you're here. I was truly impressed, and super pleased with the whole experience!read more
Leslie Durham
Leslie Durham
15:05 01 Apr 22
I've been coming here for about 5 years. The staff are ALWAYS friendly and knowledgeable. The Doctors are the absolute... best!! Jodie Moore is always in such a great mood which is a plus when you are already stressed. Highly recommendedread more
Monica Del Bosque
Monica Del Bosque
14:13 25 Mar 22
Since my first post my thoughts have changed here. It's unfortunate. My doctor and PA were great, but the office staff... is horrible. They never call you back when they say they will, they misinform you, they cause you too much stress wondering what's going on, they don't keep you posted. They never answer the phone. At this point I've left four messages in the last week, and I have sent three messages. Twice from their portal and one direct email. No response. My appointment is on Monday morning at 8:30am, no confirmation on my insurance and what's going on. What the heck is going on, this is ridiculous!I've given up... the stress her office staff has put me through is just not worth it. You can do so much better, please clean house, either change out your office staff, or find a way for them to be more efficient please. You have to do something. This is not how you want to run your practice. It leaves a very bad impression on your business.read more
Ron Buckholz
Ron Buckholz
23:32 23 Mar 22
I was actually pleasantly surprised with this visit! It took me a long time to get the appointment scheduled because no... one answers your phones EVER! After a month, I finally got in, and your staff was warm, friendly, and I was totally impressed! I feel like you will take care of my needs!read more
Steve Nabavi
Steve Nabavi
16:28 16 Mar 22
It was a nice visit. Happy staff doing all they can do to comfort the patients in a very calming environment. You ask... me they are earned a big gold star on the fridge. My only complaint they didn't give me any cookies.read more
Katie Lewis
Katie Lewis
16:10 10 Feb 22
Had very positive appointments with Jodie and Dr. Sheth for my migraine care. Jodie was so fast with the injections and... has so much valuable info. I started to feel light headed during checkout and the staff was SO helpful—giving me a chair, water, and taking me into a private room until I felt better. Highly recommend this practice for migraine patients, they know what they’re doing!!read more
Joshua Martinez
Joshua Martinez
16:02 10 Dec 21
I was scheduled to be checked and just want to say that the staff was fantastic. They were kind and helpful. I was... asked many questions related to what was going on and not once did I feel as though I was being brushed off. The front desk staff was especially great in assisting me. I'm scheduled to go back for a mri and am glad that I'll be going there.read more
Isabel Ivy
Isabel Ivy
21:42 03 Nov 21
I had such a good experience with Lone Star Neurology, Brent my MRI Tech was so awesome and made sure I was very... comfortable during the appointment. He gave me ear plugs, a pillow, leg support and blanket, easiest MRI ever lol 🤣 My 72 hour EEG nurse Amanda was also so awesome. She made sure I was take care of over the 3 days and took her time with the electrodes to make sure it was comfortable for me! Paige was also a huge help in answering all my questions when it came to my test results, and letting me know her honest opinions about how I should go forth with my treatment.read more
Leslie Luce
Leslie Luce
17:37 20 Oct 21
The professionalism and want to help attitude of this office was present from the moment I contacted them. The follow... up and follow through as well as their willingness to find a way to schedule my dad was above and beyond. We visited two offices in the same day with the same experience. I am appreciative of this—we spend a lot of time with doctors and this was top notch start to finish.read more
robert Parker
robert Parker
16:38 16 Apr 21
I love going to this office. The staff is friendly and helpful. The doctor is great. I am getting the best... neurological tests and treatment I have ever had. The only reason I did not give them a 5 star rating is because it is impossible to reach a live person at the office to reschedule appointments. Every time I have tried to get through to the office it says all people are busy and I am sent to a voicemail. If they could get their phone answering fixed, I would give them a strong 5 stars.read more
MaryAnn Hornbaker
MaryAnn Hornbaker
00:26 25 Feb 21
Dr. Harney is an excellent Dr. I found him friendly , personable and thorough. I evidently am an unusual case. ... Therefore he spent a Hugh amount of time educating me. He even gave me literature to further explain my condition and how to follow up. This is something you rarely get from your doctors. So I am more than please with my doctor and his staff.read more
Roger Arguello
Roger Arguello
03:05 29 Jan 21
Always courteous, professional. The staff is very friendly and always work with you to find the best appointment time.... The care team has been great. Always taking the time to listen to your concerns and to find the best treatment.read more
Margaret Rowland
Margaret Rowland
01:12 27 Jan 21
I have been a patient at Lone Star Neurology for several years. Now both my adult daughters also are patients there. I... love Jodie. She is always so prompt whether it is a teleamed call are a visit in the office. She takes the time to explain everything to me and answers all my questions. I am so blessed to have Jodie as my doctor.read more
Susan Miller
Susan Miller
03:01 13 Jan 21
My husband had an accident 5 years ago and Lone Star Neurology has been such a blessing to us with my husbands care.... Jodie Moore is his provider and she is amazing! Jodie is very knowledgeable, caring, and thorough. She takes her time with you, making sure your needs are met and she is happy to answer any questions you may have. Lone Star Neurology’s patients are very lucky to have Jodie providing their care. Thank you Lone Star Neurology and especially Jodie for everything you have done for us. Jodie, you are the best!read more
Windalyn C
Windalyn C
01:32 09 Jan 21
Jodie is wonderful. She is very caring and knowledgeable. I have been to over a dozen neurologists, and none were able... to help me as much as they have here. Thanks!read more
Katie Kordel
Katie Kordel
00:40 09 Jan 21
Jodi Moore, nurse practitioner, is amazing. I have suffered from frequent, debilitating headaches for almost 20 years.... She has provided the best proactive and responsive care I have ever received. My quality of life has been greatly improved by her caring approach and tenacity in finding solutions.read more
Ellie Natsis
Ellie Natsis
15:41 07 Jan 21
I have had the best experience at this neurologist's office! For over a year I have been receiving iv treatments here... each month and my nurse, Bobbie is beyond wonderful!! She's so attentive, knowledgeable, caring, and detail oriented. She makes an otherwise uncomfortable experience much more pleasant and definitely puts me at ease! She also helps me with my insurance,ordering this specialty medication and dealing with the ordering process which is no easy feat.Needless to say, she goes above a beyond in every way and I'm so grateful to this office and to Bobbie for all they do for me!read more
Matt Morris
Matt Morris
15:39 07 Jan 21
Let me start by saying that I have been coming here for years. Due to my autoimmune disease, I am in this office... once every three weeks for multiple hours at a time. The office is very clean and the staff very friendly. My only complaint would be there communication via phone. They aren't the best at responding if you leave a voicemail and expect a call back. I understand that this is prob just due to the sheer number of alls they receive daily. What I can say I like the best about the office are the people. Bobby who handles my infusions is great. I never have any issues with her setting up my infusions. She is very quick to reply to messages sent via text and if she were to leave then my whole opinion of the office may change. I also enjoy people like Matt, Lauren, and Jodi. I appreciate all that they do for me and without this team I'm not sure I would be as happy as I am to visit the office as frequently as I have to. Please ensure that these folks are recognized as they are what makes my visit to this office so tolerable :).read more
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