Are you feeling weak too? Maybe this is a condition called myopathy!

Do you sometimes feel like your limbs are weak and tired? Do you find it difficult to do even simple things like get up from a chair, lift a heavy object, or comb your hair? If you feel like your muscles are getting weak for no apparent reason, don't just dismiss it as normal. Perhaps the cause of these symptoms is a condition called 'myopathy', which we are going to talk about today. Don't worry, we will talk about this in a simple way that you can understand.

What is myopathy? Simply put...

Myopathy, simply put, is a group of diseases that affect the muscles that are attached to our bones. These diseases damage our muscle fibers and weaken them. Just as a car's engine becomes weak, it becomes difficult to drive, when muscles become weak, it becomes difficult for our bodies to perform everyday tasks.

Are there different types of myopathy?

Yes, myopathies can be classified in different ways. They are mainly divided into two categories: inherited and acquired myopathies . Let's take a look at each of these types.

What are Inherited Myopathies?

These are types of myopathy that are caused by inheriting an abnormal gene mutation that is present in our body at birth, often from our mother or father.

• Congenital myopathies:

Symptoms usually begin at birth or in early childhood. However, they can sometimes appear in young adulthood or even later. A distinctive feature of this type is that the weakness is not limited to the muscles near the center of the body, but can affect the muscles of the entire body, and the disease is often non-progressive.

• Mitochondrial myopathies:

Our cells have little factories called mitochondria that make energy. This type of myopathy is caused by a defect in these mitochondria. In addition to muscle weakness, these conditions can also affect other organ systems, such as the heart, brain, and gastrointestinal system. They can develop due to genetic mutations, whether there is a family history or not.

• Metabolic myopathies:

This myopathy is caused by defects in genes that make enzymes that help muscles function and move properly. It can cause fatigue during exercise, pain in the shoulders and thighs when exercising, or a condition called rhabdomyolysis, in which muscle fibers break down without injury. Sometimes the symptoms can come and go, with muscle weakness and then return to normal.

• Muscular dystrophies:

In these cases, the muscles gradually weaken and atrophy due to an abnormality or deficiency of structural support proteins needed by muscle tissue. In each case, the arms and/or legs are affected to varying degrees. Sometimes the muscles of the eyes or face can also be affected.

What are acquired myopathies?

These are types of myopathies that develop later in life. They can be caused by a variety of factors, including other diseases, infections, exposure to certain medications, and electrolyte imbalances in the body.

• Autoimmune/inflammatory myopathy:

What happens in this is that our body's own immune system attacks our own muscles. As a result, muscle function is impaired. It's like our own defenses attacking themselves.

• Toxic myopathy:

This condition occurs when a toxin or drug disrupts the structure or function of muscles.

• Toxins: Alcohol, toluene (a volatile substance found in things like paint that some people inhale to get high).
• Medications: Checkpoint inhibitor immunotherapy drugs (e.g., pembrolizumab, nivolumab), corticosteroids (e.g., prednisone), cholesterol-lowering drugs (statins), amiodarone, colchicine, chloroquine, antivirals and protease inhibitors for HIV infection, omeprazole.

• Endocrine myopathies:

This condition occurs when the action of hormones in our body affects the muscles.

• Thyroid gland: Although low thyroid hormone levels (hypothyroidism) are more common, problems can also occur due to high thyroid hormone levels (hyperthyroidism).
• Parathyroid gland: Hyperparathyroidism causes increased calcium levels in the blood.
• Adrenal gland: `Addison's disease` and `Cushing's syndrome`.

• Infectious myopathies:

Various infections can affect muscle function.

• Viral infections: `HIV`, `influenza`, `Epstein-Barr`.
• Bacterial infections: `bacterial pyomyositis`.
• `Lyme disease`.
• Parasitic infections: `trichinosis`, `toxoplasmosis`, `cysticercosis`.
• Fungal infections: Candida, Coccidiomycosis.

• Electrolyte imbalances:

An increase or decrease in the levels of some important electrolytes in our body can affect muscle function.

• Potassium: decreased (`hypokalemia`), increased (`hyperkalemia`).
• Magnesium: Increased (`Hypermagnesemia`).

• Critical illness myopathy in the intensive care unit:

This is a condition that affects the limbs and respiratory muscles in people who are seriously ill and receiving treatment in an intensive care unit (ICU) . It can be caused by prolonged immobility or by the use of muscle relaxants, corticosteroids, and sleeping pills used during treatment.

Who is more likely to develop myopathy? How common is this condition?

Actually, anyone can develop myopathy, but some people are at a slightly higher risk.

Factors that may increase the risk:

• If someone in the family has myopathy, then there is a high chance of inheriting this disease genetically.
• Being male. Some types of myopathy are passed on through the X chromosome, so they may affect men more than women. However, congenital myopathy types that are passed on through other chromosomes affect everyone equally.
• Having an autoimmune, metabolic, or endocrine disease.
• Exposure to certain drugs or toxins (see the section on toxic myopathy we discussed earlier).

The prevalence of myopathy varies depending on the type. For example, myopathy that develops later in life:

• Inflammatory and endocrine myopathies are more common than other types, and they are more common in women than in men.
• Between 9 and 32 out of every 100,000 people suffer from inflammatory myopathies.
• Between 25% and 79% of adults with hypothyroidism may develop muscle-related symptoms, but the incidence of overt myopathy may be as low as 10%.

The most common congenital myopathies are the muscular dystrophies, which are usually more common in males.

• Duchenne's and Becker's muscular dystrophies are the most common types, affecting about 7 per 100,000 people worldwide.
• Mitochondrial diseases affect one in 5,000 people, and most of these affect skeletal muscle.
• Other types of congenital myopathies are very rare.

So, what are the symptoms of myopathy?

Many types of myopathy have several common symptoms. These are:

• Muscle weakness, especially in the shoulders, upper arms, and thighs (these are the muscles most affected and the ones that are most severely affected).
• Muscle cramps, stiffness, and spasms.
• Feeling tired quickly when exercising or doing even a small task.
• Feeling lifeless and lacking energy.

What does myopathy really feel like?

In many myopathies, muscles on both sides of the body (symmetric) weaken equally. Muscles closer to the middle of the body (proximal muscles), such as the shoulders, upper arms, hips, and thighs, are particularly affected. As a result, you may experience symptoms such as:

• Difficulty performing everyday tasks, such as bathing, dressing, and combing your hair.
• Difficulty getting up from a chair, climbing stairs, or doing tasks that require reaching above your head (e.g., changing a light bulb in the ceiling).
• Feeling like muscles are twitching and twitching.
• Muscles get tired quickly when engaging in an activity.
• Difficulty breathing during exercise.

It usually does not affect the muscles of the palms and soles.

Other symptoms vary depending on the type of myopathy.

• Muscle weakness can be either constant (non-progressive) or slowly progressive. In some diseases, muscle weakness can come and go, while at other times there may be normal strength.
• Delayed development of motor skills (e.g. walking, jumping, climbing stairs, holding a spoon or pencil) in young children.
• Inability to keep up with other children when playing sports or playing outside (e.g., playing catch).
• Problems with the muscles that help with swallowing and speaking. This can cause food to get stuck and speech to slur.

What should I do if I suspect I have myopathy?

If you have these symptoms, you should first talk to your primary care doctor. Depending on your symptoms, he or she may refer you to a specialist, such as a neurologist or a rheumatologist.

How is myopathy diagnosed?

Your doctor will ask you about your medical history, whether anyone in your family has had similar conditions, what medications you take, and your symptoms. Then they will do a physical exam. They will check your skin, reflexes, muscle strength, balance, and sensation.

Types of tests that the doctor may order:

• Blood tests:
• In some myopathies, muscle enzyme levels such as creatine kinase (CK) or aldolase may increase due to the breakdown of muscle fibers.
• Electrolyte levels such as sodium, magnesium, potassium, calcium, and phosphorus.
• Tests for autoimmune diseases: For example, `Antinuclear Antibodies (ANA), `Rheumatoid Factor', `Sedimentation Rate' and `C-reactive protein'.
• Endocrine gland tests: for example, thyroid hormone levels.
• Electromyography (EMG) and nerve conduction studies: These tests measure the type and extent of muscle damage by measuring how electricity travels through nerves and inserting small needles into muscles.
• A magnetic resonance imaging (MRI) test of the muscles .
• Genetic tests.
• Muscle biopsy: This involves surgically removing a small piece of muscle tissue for examination.

How is myopathy treated?

Once the exact type of myopathy you have is identified, your doctor will develop a treatment plan tailored to your symptoms.

Most treatments include physical therapy, occupational therapy, and some form of exercise . Other treatments are specific to the type of myopathy. In general, most acquired myopathies can be managed well, minimizing weakness and symptoms. Some congenital myopathies have specific treatments that can slow the progression of the disease. Currently, there is no specific treatment for most congenital myopathies, but physical therapy and some types of exercise can help.

Inflammatory and autoimmune-related myopathies

The goal of treatment for these is to reduce inflammation and the body's autoimmune response. Common treatments for these myopathies include:

• Immunomodulatory/immunosuppressant drugs: For example, methotrexate, cyclosporine, tacrolimus, azathioprine, mycophenolate, rituximab, and intravenous (IVIg) or subcutaneous (SubQIg) immunoglobulin.
• `Corticosteroids`: For example `prednisone` or `methylprednisolone`.

Inherited and genetic myopathies

Many congenital and genetic myopathies have no specific treatment or complete cure. Management is primarily through symptom control and a variety of therapies. A number of clinical trials are underway in various research areas for treatments and gene therapy.

Conditions called Duchenne muscular dystrophy and Pompe disease can be treated with specific medications.

Other acquired myopathies

Doctors treat myopathies caused by endocrine, toxic, and infectious causes by treating the underlying disease that caused the myopathy. Toxic myopathies are treated by stopping the offending substance (e.g., alcohol, toluene) or drug (e.g., statins). Muscle symptoms caused by bacteria, viruses, or other infectious agents usually improve when the infection is treated directly with antibiotics.

How do I take care of my health?

Whether myopathy is congenital, acquired, or a long-term (chronic) condition, there are things you can do to maintain your health and manage the condition. These include:

• Eat a good, balanced diet. Include lots of different fruits and vegetables in your diet.
• Stay active with mild cardiovascular exercise. Depending on your type of myopathy, you may be advised not to do certain types of weight lifting. So talk to your doctor before starting any exercise program.
• Maintain a healthy weight.
• If you have a skin rash caused by dermatomyositis, protect your skin from the sun. This can make the rash worse. Wear protective clothing and a hat whenever possible. Don't forget to apply sunscreen with at least SPF 30 before going outside.
• If you have difficulty swallowing, eat soft or semi-solid foods. You can also puree your food. If you are a bedridden person, eat while sitting up in bed.
• Take all prescribed medications on time.
• If treatments such as physical therapy, occupational therapy, or speech therapy are recommended, participate in them.

Remember, because there are many types of myopathy, your doctor will have to develop a treatment plan that is specific to your type of myopathy and your symptoms. It is therefore important to follow his or her instructions exactly. Also, pay attention to your body. Note any changes in your condition or the severity of your symptoms. See your doctor on a regular schedule (or sooner if you notice any changes). This way, you can make changes to your treatment plan as soon as symptoms appear.

Finally, the most important things to remember

Myopathy is not something to be afraid of, but it is not a condition to ignore either.

• If you experience persistent muscle weakness, pain, or fatigue, be sure to see a doctor.
• There are many types of myopathy, so an accurate diagnosis is very important.
• There are treatments and management methods for many types of myopathy.
• By following your doctor's instructions, taking the prescribed medication, and living a healthy lifestyle, you can live a good life with this condition.

I hope you find this information helpful. If you have any further questions, don't be afraid to talk to the doctor at any time.

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