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Myasthenia Gravis

Myasthenia Gravis

Myasthenia gravis is a chronic autoimmune disease in which muscle tone decreases. The exact causes of the onset of pathology have not yet been determined. Thymus hyperplasia, oncology, and systemic connective tissue diseases can provoke the development of MG. Asthenic bulbar palsy can be congenital or acquired.

 

Myasthenia gravis most often develops in women between the ages of 20 and 40 and men between 50 and 80 but can occur at any age.

 

MG develops as a result of an autoimmune attack on postsynaptic acetylcholine receptors, as a result of which neuromuscular transmission is disrupted. The cause of autoantibody formation is unknown, but the disease has been associated with thymic abnormalities, autoimmune thyroiditis, and other autoimmune disorders (e.g., rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), pernicious anemia).

 

The role of the thymus in MG is unclear, but 65% of patients have thymic hyperplasia, and 10% have thymoma. About half of thymic tumors are malignant.

 

Precipitating factors for myasthenia gravis include:

  • Infection;
  • Surgery;
  • Certain medicines (e.g., aminoglycosides, quinine, magnesium sulfate, procainamide, calcium channel blockers, immune checkpoint inhibitors).

 

Ocular MG is characterized by damage to the outer muscles of the eye. It occurs in 15% of patients.

 

Congenital myasthenia gravis is a rare autosomal recessive disorder with onset in childhood. This disease is not immune-mediated and is caused by presynaptic or postsynaptic abnormalities.

Ophthalmoplegia is common in patients with congenital MG.

 

Myasthenia gravis occurs in 12% of children whose mothers suffer from this disease. It is connected with immunoglobulins of the IgG class, which passively cross the placenta; this leads to general muscle weakness developing, which disappears over a few days or weeks as the antibody titer decreases. These patients require supportive care.

Symptoms and Causes of Myasthenia Gravis

Symptoms and causes of myasthenia gravis

Symptoms

Myasthenia gravis is a chronic, constantly progressive disease. Initially, it affects the muscle tissues of the face, then spreads to the trunk. The following types of MG are distinguished:

 

  1. Ocular – the nerve endings in the cranial region are affected, and the eyelids fall asymmetrically. The patient complains of double vision and deterioration in visual acuity. Gradually focusing on one subject becomes difficult.
  2. Bulbar – the lesion extends to the masticatory muscles and tissues of the larynx. The patient’s voice changes, speech becomes quieter and nasal. Some consonants are very difficult to pronounce, and stuttering develops. Due to the penetration of fluid into the respiratory tract, the risk of pneumonia increases.
  3. Lambert-Eaton – the muscles of the arms, legs, and neck do not receive nerve impulses. It is difficult for the patient to coordinate these areas of the body. This form is diagnosed in the elderly and is characterized by rapid progression.
  4. Generalized – the muscles of the eyes are immediately affected, then the process spreads to the larynx, arms, legs, and hips. The main danger of this form is that the respiratory muscles are affected over time.

The disease is characterized by constant progression. The patient may experience both the constant presence of disturbances and short-term episodes. The symptoms of MG can be recognized by variable muscle weakness, rapid fatigue, voice changes, gait, and shortness of breath.

 

Causes

MG is congenital and acquired. The first is associated with a gene mutation responsible for the formation of synapses – adapters through which a nerve impulse travels. As a result, the tissue cannot receive the task from the brain. The following factors can provoke the development of acquired MG:

  • neoplasms – benign and malignant;
  • other autoimmune diseases;
  • excessive physical activity;
  • consequences of viral and bacterial infections;
  • postponed injuries;
  • long-term medication;
  • hormonal disorders;
  • frequent stress, anxiety.

 

The following factors can contribute to the development of myasthenia gravis:

  • diseases of the central nervous system, hyperplasia;
  • thyrotoxicosis;
  • scleroderma;
  • encephalitis;
  • frequent colds;
  • systemic diseases;
  • genetic predisposition;
  • taking alcohol and drugs.

 

Diagnostics of myasthenia gravis

Diagnostics of Myasthenia Gravis

Diagnostics consists of the following stages:

  • patient’s medical treatment;
  • determination of the level of antibodies of the acetylcholine receptor, electromyography, or both.

Symptoms and signs of the disease, confirmed by test results, can be suspected of a diagnosis of myasthenia gravis.

 

Bedside Testing

The traditional bedside test using the short-acting (<5 minutes) drug edrophonium is not used in the United States and many other countries.

 

Since muscle weakness in MG decreases with a decrease in temperature, an ice test can be performed in patients with ptosis. 

  1. An ice pack is applied to the patient’s closed eye for 2 minutes. The test result is positive if the ptosis is completely or partially resolved. The ice test is usually not useful in patients with ophthalmoparesis.
  2. Patients with ophthalmic paresis can be evaluated with relaxation tests. For this test, patients are asked to lie quietly in a dark room for 5 minutes with their eyes closed. The test result is considered positive if, after resting, paresis of the oculomotor muscles is resolved.

 

Antibody Detection and Electromyography

If one of the bedside tests is positive, several of these items are needed to confirm the diagnosis:

  • Determination of serum levels of antibodies to acetylcholine receptors;
  • Electromyography (EMG).

With EMG using repetitive stimuli (2-3 per second), 60% of patients show a decrease in the amplitude of the electrically evoked muscle response by more than 10%. Single fiber EMG reveals neuromuscular conduction disorders in more than 95% of patients.

 

Further Research

  1. After the diagnosis of myasthenia gravis, CT or MRI of the chest organs should be performed to look for thymic hyperplasia and thymoma.
  2. Screening tests for autoimmune diseases that often accompany MG (e.g., pernicious anemia, autoimmune thyroiditis, rheumatoid arthritis, systemic lupus erythematosus) should be performed.
  3. Patients with a myasthenic crisis should receive an evaluation for the presence of an infectious trigger.
  4. Lung function assessments (such as forced vital capacity) help assess the risk of developing respiratory failure.

 

Etiology

Muscle weakness with MG develops due to damage to the postsynaptic membrane of the neuromuscular junction (the place where the nerve ending connects to myocytes). In this place, under the action of an electrical impulse, acetylcholine is released from the presynaptic membrane of the nerve ending.

  1. Acetylcholine attaches to its receptors on the surface of the muscle cell. 
  2. As a result, ion channels open, and the concentration of sodium in the muscle cell increases, which leads to membrane depolarization, causing muscle contraction.
  3. With MG, antibodies to acetylcholine receptors are formed on the postsynaptic membrane, and the transmission of impulses through the muscle cell is blocked.
  4. Residual tissues of the thymus play an important role in the disease’s development, which stimulates the body’s immune system to attack its tissues. 
  5. Thymus hyperplasia is observed in 65% of patients with MG and 15% thymoma. 
  6. Half of the patients with thymoma subsequently develop myasthenia gravis.

Myasthenia gravis treatment

Myasthenia Gravis Treatment

This disesease can’t be cured. But it can be treated with medications and in some cases with surgery. 

 

1. Anticholinesterase medications to relieve symptoms

To reduce the autoimmune response, use corticosteroids, immunomodulatory therapy (intravenous immunoglobulin, plasmapheresis), immunosuppressants, or thymectomy;

 

2. Supportive therapy

In patients with congenital myasthenia gravis, anticholinesterase drugs and immunomodulatory treatments are ineffective and should not be used. Patients with respiratory failure should be intubated and transferred to mechanical ventilation.

 

3. Symptomatic treatment

Anticholinesterase drugs form the basis of symptomatic treatment, but they do not affect the pathological process. They rarely eliminate all symptoms, and the disease may become refractory to their use.

  • pyridostigmine treatment starts with 60 mg orally every 3-4 hours and increases to a maximum dose of 120 mg based on symptoms;
  • if parenteral administration is necessary (for example, due to dysphagia), neostigmine can be used (1 mg corresponds to 60 mg of pyridostigmine);
  • anticholinesterase drugs can cause abdominal cramps and diarrhea, which are treated with atropine 0.4 to 0.6 mg orally (given with pyridostigmine or neostigmine) or propantheline 15 mg 3-4 times a day.

 

Patients who have responded well to treatment and then become worse require respiratory support because they may have a cholinergic crisis, and the use of anticholinesterase drugs should be discontinued for several days.

 

4. Immunomodulatory treatment

Immunosuppressants suppress the autoimmune response and slow the course of myasthenia gravis disease but do not lead to a rapid decrease in the severity of symptoms. 

  • Intravenous immunoglobulins at a dose of 400 mg/kg once a day for 5 days cause improvement in 70% of patients after 1-2 weeks. The effect can last from 1 to 2 months. 
  • Plasmapheresis (for example, 5 sessions with the removal of 3-5 liters of plasma for 7-14 days) has a similar effect.
  • In maintenance therapy, glucocorticoids are necessary; but in myasthenic crises, they do not have an immediate effect. In more than half of cases, after starting treatment with high-dose glucocorticoids, a sharp deterioration occurs. Treatment begins with prednisolone 10 mg orally once a day; each week, the dose is increased by 10 mg up to 60 mg, and the patient takes it for about 2 months, followed by a chalky decrease. Improvement occurs in a few months; then, the dose must be reduced to the required minimum to ensure symptom control.
  • Azathioprine 2.5–3.5 mg/kg once daily may be as effective as glucocorticoids, although there may be no improvement for many months. By taking cyclosporine 2.0–2.5 mg/kg orally 2 times a day, you can reduce the dose of glucocorticoids. These drugs require special care.
  • Other effective drugs include methotrexate, cyclophosphamide, and mycophenolate mofetil. In patients with resistant disease, monoclonal antibodies (rituximab, eculizumab) may be effective, but they are expensive.
  • Thymectomy may be indicated in patients with generalized myasthenia gravis if they are <80 years of age. Subsequently, in 80% of cases, remission occurs, or the amount of maintenance therapy can be reduced.

 

Before thymectomy for myasthenic crises and in resistant patients, plasmapheresis or intravenous immunoglobulins are effective.

 

 

FAQs

  • How many years do people live with a diagnosis of myasthenia gravis?

Patients can live until old age, subject to the doctor’s recommendations and regular examination. On average, myasthenia gravis appears at the age of 20-40, more often in women. After its manifestation, it is important to start treatment immediately and adhere to it until the patient gets better.

  • How serious is myasthenia gravis?

 Only the eye muscles are hit about 1 in 5 people. Therapy can help keep the symptoms under control. Very rarely, MG can resolve or get better on its own. If not treated in time, MG can be a threat to life, but it does not have a major influence on lifespan for most people.

  • Is exercise good for myasthenia gravis?

Physical training and exercise are safe in patients with MG; physical training programs reduce variable muscle weakness and daily functioning in patients with this disease. Respiratory muscle function improves with endurance training in patients with MG.

  • How to take prednisone in myasthenia gravis?

The dose of prednisone is based on an individual assessment of the severity of the patient’s condition. On average, the dosage is determined at the rate of 1 mg per 1 kg of body weight, but should not be less than 50 mg.

  • What diseases are similar to myasthenia gravis?

Many disorders may mimic MG, including generalized fatigue, amyotrophic lateral sclerosis (ALS), Lambert-Eaton myasthenic syndrome, botulism, penicillamine-induced myasthenia, and congenital myasthenic syndromes.

  • What should not be consumed in the presence of myasthenia gravis?

It is categorically contraindicated:

  1. Diuretics (lasix, furosemide), with the exception of veroshpiron; 
  2. сurariform muscle relaxants, GHB, antipsychotics, and tranquilizers; 
  3. sedative (sedative) drugs (except for Grandaxin, Adaptol, bromine, motherwort, valerian, Corvalol, valocordin).

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Lone Star Neurology
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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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