Erb's genetic disease. Erb-Roth progressive muscular dystrophy. Muscular dystrophy with Dreyfus contractures. Treatment of Erba-Roth myopathy with medicines

Erba-Roth juvenile myopathy is a genetic disease that children of perfectly healthy parents can suffer from. As a rule, the disease begins to manifest itself only in adolescence or youth, and almost all diagnoses occur at the age of 14-18 years. The second name for this disease is muscular dryness.

This pathology was first described in 1884 by the renowned German neurosurgeon Wilhelm Heinrich Erb. That is why it is most often called Erb's myopathy.

Causes

Erba-Roth myopathy is a primary disease that occurs on its own, and is most often hereditary or genetic in nature. This means that the main cause of all problems is a failure that occurs in the genes. And if this disease is not hereditary, then the main reason here is genetic.

Most often, the main cause of the disease is deviations in the process of pregnancy in the fetus, as well as bad habits of the mother - smoking, drug addiction, alcohol consumption, especially in the early stages of fetal development.

Also, factors such as working in a hazardous industry, living in an ecologically unfavorable area, working with chemicals during pregnancy, and much more can be to blame for the “breakdown” in the genes. All this can affect the chromosomes. It has also been proven that juvenile myopathy usually occurs when the mother becomes pregnant after 40 years of age.

Symptoms

The main symptom of Erb-Roth disease is weakness in the muscles. This weakness is permanent, but the person does not experience pain. Even after a long rest, weakness does not go away. Although at the very beginning of the disease a slight improvement may be observed after a night's sleep, but they quickly pass, and the weakness returns again.

First of all, the muscles of the pelvic girdle and hips suffer, and over time, the disease spreads to the shoulder girdle and to the muscles of the body. At the same time, their pseudohypertrophy, that is, an increase in volume due to the replacement of muscle tissue with adipose or connective tissue, is very, very rare.

With the further development of the disease, thinning or even atrophy of the muscles of the trunk and limbs occurs. They become inactive and very thin. With this type of disease, almost all human muscles suffer, but since the pathology progresses very slowly, the patient can retain the ability to walk up to 40 years, and sometimes longer.

Another symptom of the disease is a decrease in muscle tone. In this case, the muscles become sluggish and their flabbiness appears. Due to the fact that the weakness of the muscular corset of the human body is manifested, problems with the spine begin. In patients with myopathy, its curvature almost always occurs, scoliosis, lordosis, kyphosis and other pathologies of the spinal column appear. At the same time, it is impossible to cure scoliosis or kyphosis in this case, and these defects themselves begin to progress over time. And if at the very beginning the curvature of the spine is barely noticeable, then by the age of 40 they can be seen with the naked eye.

Complications

Juvenile myopathy of Erba-Roth has its own specific complications, from which the death of a person most often occurs. Among them, the most common are:

1. Respiratory failure.
2. Complete loss of the ability to move independently.
3. Congestive pneumonia.
4. Intervertebral hernias.
5. Rachiocampsis.
6. Paresis.
7. Paralysis.

It is impossible to prevent all these complications, but they can be delayed if all the recommendations given by the doctor are followed.

Treatment

To date, there is no cure for Erb's juvenile myopathy. Doctors recommend that the patient engage in physical therapy, visit the pool, spend more time in the fresh air and try to lead an active lifestyle. Be sure to take massage courses every 2 or 3 months, but all prescribed treatment is best taken in a hospital setting.

As for drugs, the treatment is usually used such means as:

1. Cerebrolysin.
2. Theonicol.
3. Vitamin E.
4. Potassium orate.
5. Prozerin.
6. Nootropil.
7. Cocarboxylase
8. Vitamin B12
9. Cavinton.

These drugs are prescribed only by the attending physician and only in strictly individual dosages.

The article will discuss the progressive Erb-Roth muscular dystrophy.

Muscle dystrophy is a chronic genetic disease that affects the human muscular system. The muscles affected by the pathological process cease to function normally, gradually become thinner, and in their place, over time, an increase in a layer of fat begins.

The main types of dystrophy

There are several most common types of muscular dystrophy:

1. otherwise called pseudohypertrophic or negative congenital dystrophy. Pathology manifests itself in childhood, between two and five years. At the initial stage of development, mainly the muscles of the lower body are affected. In children who lead a sedentary lifestyle, leg muscles begin to increase. This is due to the replacement of muscles with fat.

The gradual progression of the pathology leads to the defeat of the upper body, reaching the arms. With a standard course of the disease, by the age of 12, the child completely loses the ability to move. The probability of death is high, it is 85-90% before the child reaches the age of twenty.

2. Steinert's disease. This is a congenital pathology, also called myotonic disease. Myotonia refers to a too slow process of muscle relaxation after contraction. Pathology affects adults aged 20-40 years. There are also cases of morbidity in children in but this is an exception to the rule. The disease is not tied to gender, it affects both women and men equally. Steinert's pathology is accompanied by a weakening of the facial muscles responsible for facial expressions, as well as limbs. The course of the disease is not characterized by rapidity, progression can occur over a long period of time.

3. Pathology of Becker. This disease is also characterized by a long development. At the initial stage, it passes latently, and the patient does not experience any difficulties for a long time. The pathology acquires an aggravated form due to injury or diseases of the nervous system, which provoke the progression of the disease.

4. Landouzy-Dejerine disease. This pathology has no age limits and can affect both children from five years old and adults up to 55 years old. The humero-scapular-facial region of the body is mainly affected. A characteristic feature of the pathology is a long progression, which can allow the patient to remain able to work for several decades. The main signs of the pathological process is damage to the muscles of the face, which leads to impaired speech function. This is due to the inability to completely close the lips. This symptom also applies to the eyelids, which may not close completely. With the further development of the disease, atrophy of the muscles of the face, shoulders, limbs and torso occurs, which completely immobilizes a person.

5. Distal type of muscle dystrophy. It is a benign form of progressive dystrophy. Quite often, pathology in the diagnosis is confused with Marie-Charcot. To differentiate these pathologies, an electroencephalogram is prescribed, which allows you to clarify the diagnosis. Characteristic signs of the development of pathology is the loss of muscle function in the arms and legs with their further thinning. Against the background of the distal form of dystrophy, paresis of the hands and feet is observed.

6. Myodystrophy named after Emery-Dreyfus. Initially, this type of pathology was not distinguished into a separate disease. This was due to the fact that in external manifestations the disease resembles Duchenne dystrophy. But with a more detailed study of both pathologies, it was found that Emery-Dreyfus myodystrophy is a separate disease with characteristic symptoms.

This form of dystrophy is rare. It affects patients who are under 30 years old. At an older age, isolated cases have been reported. The main difference between this form of dystrophy and others is the damage to the heart muscles, which can lead to the death of the patient. All other signs of the disease are more benign than in Duchenne pathology.

Let's move on to the consideration of Erb-Roth muscular dystrophy.

Description

Erba-Roth juvenile myopathy is a disease of genetic origin that can affect the children of perfectly healthy parents. Most often, the pathology begins to progress in adolescence. The vast majority of diagnoses occur between the ages of 14 and 18. Erb's pathology is also called muscular tabes.

The disease was first mentioned in 1884 by the German neurosurgeon Heinrich Erb, which gave the name to the pathology he described.

Causes

Erba-Roth muscular dystrophy is a disease of the primary type that occurs as an independent pathology due to a hereditary or genetic factor. That is, the main reason for the development of this type of myopathy is a violation in the genes. Accordingly, if the disease is not caused by a hereditary predisposition, then its cause lies in a gene failure.

The most common prerequisite for the development of Erba-Roth's muscle-progressive pathology is intrauterine malformations of the fetus, caused, among other things, by the bad habits of the expectant mother: smoking, using drugs and alcohol. These factors are especially affected at an early stage of fetal development.

What else can cause a disorder in the genes?

Violation in the genes can also be caused by the work of a woman in hazardous production, permanent residence in an unfavorable environment, work with chemicals while carrying a child, etc. These factors can negatively affect chromosomal development. In addition, it is a proven fact that Erba-Roth juvenile muscular dystrophy develops as a result of the birth of a child in a woman over 40 years of age.

Signs of myopathy

The main symptom of the presence of Erb's pathology is muscle weakness. This symptom is constantly present, while it does not cause pain to a person. Weakness in the muscles does not go away even after a long stay in a state of rest. At the initial stage of the course of Erba-Roth's disease, there may be a slight improvement in the patient's condition after a night's sleep, but this quickly passes and weakness comes again.

The muscles located on the hips and in the pelvic girdle are primarily affected. In the future, the disease spreads to the muscles of the trunk and shoulder girdle. Pseudohypertrophy, accompanied by an increase in volume through the growth of adipose tissue and the death of muscle tissue, occurs extremely rarely with Erb-Roth muscular dystrophy.

In the future, the disease is characterized by thinning and subsequent atrophy of the muscles of the limbs and trunk. Muscle mobility decreases, and the tissues themselves become thinner. Almost all human muscles are affected by Erb's disease, but the progress of the pathology is slow and it is possible to maintain the ability to walk up to forty years.

Decreased muscle tone is another characteristic feature of Erb muscular dystrophy. Muscles at the same time become lethargic and sagging. As a result of such changes, a person begins to have problems with the region of the spine, its pronounced curvature occurs, and so on. These diseases are not treatable and in the future also begin to progress.

Diagnostics

There are quite a few diagnostic methods for identifying Erba-Roth dystrophy, as well as differentiating it from other pathological processes. At the first visit, the specialist will collect the patient's history, clarify the existing complaints and symptoms. Next, an examination plan is drawn up, which may include the following diagnostic procedures:

Electromyography.

Scraping of muscle tissue for research.

Genetic test.

Examination of blood and urine.

Consultation of a therapist, orthopedist, gynecologist and other specialists.

It must be remembered that the later the disease manifested itself, the better for the patient. This is due to the fact that with the manifestation of pathology at an early age, the probability of death is high.

Treatment of Erba-Roth myopathy with medicines

Therapy for muscle dystrophy is a complex and lengthy process. At the moment, no drugs have been developed that can completely cure the patient. All therapeutic techniques are aimed at improving the patient's condition and restoring some of the functions that have been inhibited by pathology.

To stop the progression of dystrophy, the following drugs are used:

1. Corticosteroid drugs.
2. Vitamin B1.
3. Adenosine triphosphate.

In addition, to slow down the development of the pathological process, implantation of fetal stem cells is used.

Other therapeutic methods

As a preventive measure, the following therapeutic methods are prescribed:

1. Physiotherapy procedures.
2. Massage.
3. Breathing exercises.

There are three main principles that should be followed in case of limb-girdle muscular dystrophy of Erb-Roth - physical activity of adequate intensity, adherence to proper nutrition, timely receipt of psychologist support.

Principles of supporting the body with muscle dystrophy

Often, patients with Erb's pathology have an unwillingness to fight the disease, which negatively affects the body as a whole. As a result of the lack of physical activity, the progression of the pathology is aggravated and accelerated. Muscles should receive the load in the required volume.

A very important stage of patient support is psychological support. Sometimes the support of relatives and friends is enough, but in some cases qualified assistance may be required. It is important for a person with a similar diagnosis to know that he is not alone and that there are people who support him, empathize and are always ready to help.

Diet

Equally important is the observance of a special diet for Erb-Roth myodystrophy. The diet should reduce inflammation, normalize the level of glucose in the blood, and also remove toxins from the body, while providing the tissues with the necessary nutrients. The main principles of therapeutic nutrition for muscular dystrophy are:

• Avoiding bad fats.
• Meat and fish should be "clean", without antibiotics and hormones.
• Lack of gluten and sugar in the diet.
• Supplement your diet with plenty of green vegetables and spices.
• No carbonated drinks or artificial colors.
• Milk should only be consumed from goats.

With Erba-Roth pathology, treatment should be comprehensive and timely.

Prevention

Predicting the appearance of muscle dystrophy, as well as detecting it at an early stage, is quite difficult. Therefore, the prevention of pathology involves the implementation of two rules:

1. A complete and detailed examination of a woman when planning a pregnancy. Particular attention should be paid to the presence of mutated genes.
2. During pregnancy (if it was not done before conception), a test is performed to detect chromosome mutations.

Forecast

The prognosis of survival depends on the type of disease, as well as the complications that pathology can lead to. Among the associated diseases and conditions of muscular dystrophy, the following stand out:

• Violation in the work of the heart.
• Curvature of the spinal column.
• Violation of motor activity.
• Decreased mental abilities of the patient.
• Violation of the respiratory function.

Conclusion

Progressive muscular dystrophy of Erba-Roth is a severe and incurable disease that can lead to complete immobilization of a person. When planning a child, parents should take a responsible approach to the issue of examination for genetic mutations.

Dystrophy of the muscular system is a chronic hereditary disease. Its main symptom is considered to be gradually increasing weakness in the muscles and their degeneration. There are several types of pathology. In today's article, we will take a closer look at the treatments, causes, and symptoms of Erba-Roth muscular dystrophy.

Medical certificate

The disease is a polymorphic variant of hereditary myodystrophy. It differs from other types of pathology in the clinical picture, course and time of onset. For the first time, the description of the disease was presented by the German neurologist W. Erb in 1882. At the same time, V. Roth dealt with this problem in Russia, which he later designated as “muscular dryness”. It was by the names of the two scientists that the disease was named. In modern neurology, several of its names are used - progressive Erba-Roth muscular dystrophy, limb-girdle muscular dystrophy.

Pathology begins its development, as a rule, in childhood. However, the age of onset of the first symptoms can range from 10 to 30 years. Men and women are equally affected by manifestations of muscular dystrophy. Neurologists note that the disease that began in childhood progresses rapidly, if we compare its course in adolescence and adulthood. In addition, in the second case, it proceeds in a mild form.

Disease pathogenesis

What is myodystrophy? It is advisable to consider the causes and effective methods of treatment after studying the pathogenesis of the disease. It begins its development with pathological changes in muscle tissues, which are metabolic and structural in nature. This is myopathy. They arise from mutations in genes. As a result, there is a deficiency or complete cessation of the synthesis of proteins, which are a necessary structural component of myocytes.

The disease can be descending, when weakness is observed in the proximal arms. However, most often it has an ascending type of distribution of muscle changes. As the disease progresses, there is a decrease in the volume of muscle fibers. They gradually cease to function fully and are destroyed. In their place, a fatty layer is formed. Over time, muscle tissue is completely replaced by fat. As a result, immobilization occurs, followed by disability.

Main reasons

Erba-Roth muscular dystrophy is an independent disease, the appearance of which is due to a hereditary or genetic factor. Its main reason is changes at the gene level in one of the parents or the patient himself. In 30% of cases, the violation occurs primarily. In all other situations, it is hereditary.

Intrauterine gene developmental disorders, due to the appearance of which pathology begins, are usually provoked by:

• bad habits of a pregnant woman;
• living in places with poor ecology;
• work in hazardous production after the conception of a child;
• late birth;
• uncontrolled use of antibiotics;
• prolonged contact with toxic substances.

With the development of complications, the disease becomes deadly for humans. Among the negative consequences, doctors include paralysis of the limbs, congestive pneumonia, various disorders in the respiratory / cardiac system.

Clinical picture

The main symptoms of Erba-Roth muscular dystrophy are the following disorders:

• "duck" gait, when the patient rolls over from one leg to another;
• imbalance and instability;
• difficulty getting out of bed, bending over;
• protrusion of the shoulder blades;
• reduction in waist circumference;
• pathological fatigue.

As the disease progresses, there is a weakening of the muscular corset of the back and shoulder girdle, which leads to lordosis. It becomes increasingly difficult for patients to hold objects in their hands. Mimic muscles on the face also lose their mobility. This is manifested by incomplete closing of the eyelids and protrusion of the lips.

Erba-Roth muscular dystrophy can develop over many years. In medical practice, there are cases when patients lived with muscle weakness for more than 20 years. Only after this time did they develop other symptoms.

Diagnostic methods

If symptoms suggestive of myodystrophy appear, it is necessary to visit a neurologist. First of all, the doctor pays attention to the patient's history, conducts a physical examination. Then a comprehensive examination is assigned, which consists of the following activities:

• genetic testing;
• electroneuromyography;
• biopsy and biochemical examination of muscle tissue;
• blood test for creatine phosphokinase;
• Analysis of urine.

Electroneuromyography is considered the most informative diagnostic method. It allows you to assess not only the degree of neuromuscular transmission, but also to determine the level of muscle excitability. The latter study is especially important for differential diagnosis. Manifestations of Erba-Roth muscular dystrophy are similar to ALS, toxic myopathy, polymyositis and a number of other pathologies.

Genetic testing helps to confirm the autosomal recessive nature of inheritance, the presence of mutations. However, a negative result does not invalidate the initial diagnosis. Not all varieties of mutations known to modern science can be detected through such testing. A negative analysis is an indication for a biopsy of muscle tissue. In the case of dystrophy, the study shows a reduced number of muscle nuclei, the presence of changes of a necrotic or sclerotic nature.

Features of therapy

How to identify Erb-Roth myopathy? The disease is not possible to overcome completely. Therefore, patients with a similar diagnosis are prescribed symptomatic therapy. Its main goal is to improve the quality of life of the patient and maintain full physical activity. In modern medical practice, drugs, exercise therapy and physiotherapy are used.

Medical therapy

Medical treatment of Erba-Roth muscular dystrophy includes the use of the following drugs:

1. Vitamin complexes (A, groups C, E, B and D).
2. ATP to normalize cell energy metabolism and activate membrane enzymes.
3. Alpha-lipoic acid ("Thiolipon", "Dialipon") helps to restore metabolic processes.
4. "Riboxin" has antiarrhythmic, anabolic and antihypoxic effects.
5. "Actovegin" improves the healing process of bedsores, normalizes arterial and venous circulation.

The duration of administration and dosage of drugs are determined by the doctor individually.

With damage to the heart muscle, "Inosine", glycosides and antiarrhythmics are prescribed. If contractures develop, orthopedic therapy may be required. A decrease in the vital capacity of the lungs against the background of atrophy of the respiratory muscles is a direct indication for connection to a ventilator.

exercise therapy

An obligatory component of the therapy of Erba-Roth muscular dystrophy is the exercise therapy complex. The main goals pursued by the exercises:

• development and maintenance of the muscular apparatus;
• proper relaxation;
• prevention of contractures, due to which the patient loses the ability to move;
• correct breathing;
• prevention of scoliosis and other similar deformities.

In the course of therapy, physical and breathing exercises, massages of different levels of activity are used. In case of pathologies of the musculoskeletal system, the exercise therapy complex is always selected individually by a specialist. The exception is minor loads in the pool. In this case, the instructor first shows the exercises, and after several sessions the patient can already repeat them independently.

Features of physiotherapy

With muscular dystrophy (myodystrophy) of Erba-Roth, physiotherapeutic effects are possible in two directions: body wraps, electrophoresis with enzymatic agents. In rare cases, patients are prescribed electrical stimulation. This procedure causes the muscles to contract. It is recommended in situations where the patient's muscles are so weak that simple movements are accompanied by severe discomfort.

Diet

With Erb-Roth myodystrophy, it is important to adhere to a special diet. A properly selected diet allows you to stop inflammatory processes in the body, remove toxins and provide tissues with the necessary nutrients. It implies following the following principles:

• complete rejection of fatty, fried, salty and smoked;
• the use of fresh vegetables and fruits, lean fish in the diet;
• lack of foods high in gluten and sugar;
• only goat milk is allowed;
• carbonated drinks and alcohol are prohibited.

In general, such a diet contains the principles of proper nutrition. Therefore, it can be adhered to throughout life, without fear of causing significant harm to health.

Recovery prognosis

In clinical medicine, this pathology does not belong to the group of deadly. However, the prognosis is poor in most cases. The disease progresses quite quickly. Approximately 20-25 years after the onset of the initial symptoms, it leads to a complete loss of mobility and a wheelchair.

Muscle atrophy eventually extends to the cardiac and respiratory systems. This leads to secondary disorders, such as heart failure, lung infections. It is these pathologies that lead to death. Mild forms of muscular dystrophy do not affect the life expectancy of patients.

In the United States, the causes and treatment of Erba-Roth dystrophy are being actively studied today. Dystrophy, according to local scientists, may soon be curable. The positive results of a study on the use of gene therapy have recently been published. It involves the introduction of a modification of the adeno-associated AAV1 virus of the Parvoviridae family into the affected cellular elements of the muscle tissue. This virus provokes an immune response, as a result of which the process of alpha-sarcoglycan synthesis is normalized.

Prevention methods

There is no specific prevention of this disease, since in most cases it is hereditary. However, doctors offer several methods to minimize the risk of its occurrence.

First of all, at the planning stage, both future parents must undergo a comprehensive examination of the body. It, as a rule, also implies genetic testing for the detection of pathological genes. If necessary, consultation with narrow specialists may be required.

If a pathology is suspected, a study of cellular elements and blood in the fetus is prescribed to detect gene mutations. The procedure is performed early. According to its results, the doctor offers parents several options for solving the problem.

If the disease manifested itself already at a conscious age, it is necessary to take measures to alleviate the patient's condition and to prevent the development of possible complications. In this matter, each case is individual. Therefore, there are no universal recommendations.

Conclusion

What is Limb Girdle Muscular Dystrophy? This is a serious disease, accompanied by weakness in the muscles. This diagnosis is not a sentence, but causes irreversible damage to the body. However, its timely detection and competent treatment can minimize the negative consequences, significantly facilitate the patient's life.

Erb-Roth myopathy (generally accepted scientific name for the disease Erb-Roth limb-girdle myodystrophy) belongs to a group of hereditary neuromuscular diseases. What does this mean? This group of diseases is characterized by a genetically determined lesion of nerve fibers, skeletal striated muscles or anterior horns in.

The term "or muscular dystrophy" is used to refer to a large number of diseases with a different clinical picture, but they are based on a primary lesion of the structure of the muscle fiber, and not motor neurons. Myodystrophies differ in symptomatic manifestations, the timing of the onset of the disease, the rate of progression of symptoms, the genetic nature and type of inheritance.

Erba-Roth's muscular dystrophy is characterized by an autosomal recessive type of inheritance, so the disease manifests itself in children whose parents are carriers of the mutation. Currently, 15 genes have been identified, the mutations of which lead to the development of this pathology. The gene anomaly leads to a disruption in the synthesis of proteins that are part of the structure of muscle cells (myocytes), causing the death of myocytes.

The disease was first described in 1885 by the German neurologist W. Erb. At the same time, V. Roth was engaged in the study of pathology in Russia.

Signs of Erb-Roth myopathy

It is difficult for a person suffering from Erb-Roth myotonia to raise his arms up and perform actions associated with this movement.

Women and men get sick with the same frequency. In the population, myodystrophy occurs with a frequency of 1-3 cases per 100,000 population.

The debut of the disease falls on the 2nd or 3rd decade of life, but it can also be in childhood. Muscle damage begins with the pelvic or shoulder girdle. The first symptoms of the disease include a change in gait as a result of the development of weakness in the legs (hips). A person's gait resembles a "duck gait", waddling. Difficulty climbing stairs, getting out of a chair or bed.

The defeat of the shoulder girdle is manifested by the occurrence of weakness in the hands. It becomes difficult for a person with Erba-Roth's muscular dystrophy to raise his arms above his head, and in severe cases it is impossible. Weakness in the hands does not allow the patient to comb his hair, spin a light bulb over his head, lift things to a certain height. As the disease progresses, independent eating, doing housework brings a person significant difficulties up to the deprivation of the opportunity to perform them. Muscle hypotrophy gradually develops, which is manifested by the lagging of the shoulder blades from the back or (“pterygoid shoulder blades”) by protrusion of the abdomen forward, a decrease in the volume of the waist (“wasp waist”). In the advanced stage of the disease, the muscles of the anterior abdominal wall and back are involved, and a curvature of the spine (lumbar hyperlordosis) is formed. Deep disability, which is manifested by the complete immobility of a person, occurs 15-20 years after the onset of the disease. A decrease in the contractility of the respiratory muscles leads to the development of frequent pneumonia, respiratory failure. Over time, heart failure develops.

Mimic muscles are not involved in the pathological process. Intelligence remains intact.

Diagnosis of the disease

To establish the diagnosis, a comprehensive examination of the patient is used, which includes a neurological examination, conduction (ENMG), taking biopsy material from muscle tissue. A genetic study is required.

A neurological examination reveals a decrease or complete loss of reflexes from the arms and legs, muscle hypotrophy while maintaining a sensitive area, as well as a decrease in muscle strength. With the help of ENMG, primary changes in the skeletal muscles are detected against the background of maintaining the conduction of impulses along the nerve fibers. Histological examination of the biopsy material reveals degenerative changes in the muscles.

Treatment of Erb-Roth myodystrophy

Specific treatment has not been developed. Symptomatic therapy means are used to maintain a person's motor skills.

Used (neurobion, vitagamma), thioctic acid (octolipen), ATP, actovegin.

Mandatory exercise therapy and massage courses.

Disease prognosis

Important components of the treatment of this disease are physiotherapy exercises and massage.

The rate of progression of Erba-Roth myopathy can vary greatly from person to person. Typically, death occurs from respiratory (congestive pneumonia) or cardiac complications (heart failure, arrhythmia, cardiomyopathy). With a mild course of Erba-Roth myopathy, a person can retain the ability to move and self-service for many years.

For preventive purposes, genetic counseling of future parents is used, the exclusion of closely related marriages.