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Description Of Myopathy

Medically reviewed by Sandeep Dhanyamraju

What is Myopathy? Myopathy is a disease that occurs due to a disturbance in the metabolism and structure of muscle tissue, as a result of which a decrease in the strength of the affected muscles appears and a limitation of motor activity.

Myopathies belong to the group of neuromuscular diseases, characterized by dystrophic lesions of muscle tissue with atrophy of individual fibers (myofibrils) in some places. In addition, the atrophied myofibrils are replaced by connective or adipose tissue; this leads to the loss of the ability of the muscles to contract, which leads to the appearance in the clinic of the disease of muscle weakness and the limitation of motor activity.

There are two main forms of myopathy:

  • Primary myopathies, which are often hereditary. In turn, they are divided into the following types:
  1. congenital myopathies that develop during infancy
  2. early childhood myopathies that develop at the age of 5-10 years
  3. juvenile myopathies that develop during adolescence.
  • Secondary myopathies are arising from the background of other diseases.

For example, against the background of endocrine disorders (Itsenko-Cushing’s disease, hyperaldosteronism, hyperparathyroidism, hypo-, and hyperthyroidism), chronic intoxications (alcoholism, drug addiction, work at enterprises with occupational hazards), severe chronic diseases (chronic renal failure, chronic liver failure, chronic obstructive pulmonary disease, heart failure), and tumor processes.

Primary forms of myopathy are much more common than secondary ones. Therefore, it is essential during pregnancy planning to consult a geneticist to identify the risk of developing this pathology in an unborn child.

Based on which part of the body muscle weakness is more pronounced, the following types of myopathy are distinguished:

  • the proximal pathological process is located in those parts of the limbs that are as close as possible to the body (for example, in the case of the arms, these are the shoulders)
  • the distal muscles of the limbs are affected, which are at maximum distance from the body (for example, in the case of the legs, the calf muscles)
  • mixed has symptoms of proximal and distal forms of the disease.

Primary myopathies have a poor prognosis, especially if the first symptoms appeared in early childhood. The involvement of the heart muscle and respiratory muscles in the process is also essential. In this case, the prognosis of the disease worsens. The most favorable outcome is observed in secondary myopathies since, in this case, it is easier to achieve success in the treatment of the underlying disease that caused the development of myopathy.

Diagnosis of myopathy

Treatment of myopathy

The doctor may suspect the presence of myopathy even at the level of the initial examination since an appropriate appearance characterizes such people.

  • First, there is muscle atrophy (proximal extremities) and pseudohypertrophy of the distal extremities.
  • Secondly, over time, a duck gait is formed. Also, myopathy is characterized by drooping of the shoulders, pterygoid lagging of the shoulder blades, protrusion of the abdomen forward, and increased lumbar lordosis.

Then the doctor proceeds to a neurological examination, during which he reveals a weakening of tendon reflexes, a decrease in muscle strength. Unlike neuropathy, myopathy does not impair sensitivity.

After the examination, the patient is sent to the laboratory to pass the required list of tests. One of these is urinalysis, which looks for creatinine to confirm myopathy. The presence of creatinine in the urine may indicate muscle damage; therefore, this indicator is not devoid of informative value in diagnosing the disease in question. Next, a biochemical blood test is performed, in which the level of enzymes is assessed.

In addition, electrophysiological research methods are used in electroneurography and electromyography.

  • The results of electroneurography provide information about the state of the peripheral motor neuron, which is necessary if there is a suspicion of neuropathy.
  • Electromyography is performed to assess the condition of muscle tissue directly. With myopathy, there is a change in muscle potentials (their amplitude decreases and their duration decreases).

The most informative method is a morphological examination of muscle tissue; this is done with a muscle biopsy. Although the most informative, this method is rarely carried out, only in cases where the previous research methods did not provide adequate information to confirm a particular diagnosis. In myopathy, the morphological picture of muscle tissue samples is as follows: randomly scattered atrophied myofibrils (contractile elements of muscle fibers), hypertrophied muscle fibers, and replacement of muscle tissue with connective or adipose tissue is observed.

A consultation with a cardiologist is appointed; they will then send the patient for an ECG and ultrasound of the heart to assess the condition of the heart muscle. If pneumonia is suspected as a complication of the course of myopathy, an X-ray of the lungs is performed.

Treatment of myopathy

For the treatment of primary forms of myopathy, there are no drugs that can eliminate genetic defects that caused the development of the disease. In this case, treatment is only symptomatic. It aims to alleviate the symptoms of the disease and improve the quality of life.

For this purpose, anabolic hormones, anticholinesterase drugs, and vitamin complexes are prescribed. The effect of these drugs is aimed at inhibiting dystrophic and atrophic changes in muscle tissue.

Secondary forms of myopathy respond better to treatment since therapy aims to eliminate concomitant diseases that caused the development of myopathy. In the case of a competent selection of drug and non-drug methods of treatment, there is a gradual elimination of symptoms that bother people with myopathy against the background of endocrine disorders, chronic intoxication, severe chronic diseases, etc.

The appointment of physiotherapy complements the above treatment methods. For example, electrophoresis with neostigmine, iontophoresis with calcium, and ultrasound are used.

In addition, it is essential to prescribe massage and exercise therapy. The use of these treatment methods is aimed at slowing down the process of dystrophic changes in muscle tissue with subsequent atrophy. Also, due to constant physical activity, the degeneration of muscle tissue into connective and adipose tissue is reduced. However, it is essential not to overload the weakened muscles; therefore, the set of exercises is selected by a qualified specialist.

Much attention is paid to breathing exercises, which aim to improve the ventilation of the lungs; this is necessary to prevent the development of complications in the form of pneumonia, which can occur due to damage to the respiratory muscles.

Medicines to treat myopathy

The goal of myopathy treatment is to reduce muscle atrophy. Anabolic hormones (retabolil, nerobol) are of great help in this. Their action is expressed in stimulating the growth of muscle cells, which is necessary for maintaining muscle mass. Due to this, there is an increase in the indicator of strength, performance, and endurance. However, there is a downside to taking these drugs. With prolonged use, side effects may occur in the form of baldness or increased hair growth on the face and body. In addition, there is such a concept as masculinization, which means the appearance of secondary sexual characteristics of the male sex in women. In this case, there is a change in the female constitution; the voice deepens, the menstrual cycle is disrupted, the elasticity of the skin changes, and acne appears.

From anticholinesterase drugs, drugs such as proserin, neuromidin are used. Preference is given to neuromidin, as its spectrum of action is much wider than proserin. Also, with prolonged use of proserin, various unpleasant side effects are noted. Therefore, in most cases, the drug is prescribed in short courses.

In turn, neuromidin has a good stimulating effect on neuromuscular transmission and increases the contractile activity of muscles. In addition, neuromidin has sedative and analgesic (relieves pain) effects.

Vitamins of groups E, B, and C, and nicotinic acid, are used from vitamin preparations.

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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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