Demyelinating disease of the brain and central nervous system: the causes of development and prognosis

To exclude an unfavorable prognosis, it is recommended to follow all the prescriptions of the doctor, to conduct a timely examination.

Demyelination

What is the demyelination of the brain will become clear if you briefly talk about the anatomy and physiology of the nerve cell.

Nerve impulses travel from one neuron to another, to organs through a long process called an axon. Many of them are covered by the myelin sheath (myelin), which provides a quick impulse transmission. At 30% it consists of proteins, the rest in its composition – lipids.

Development mechanism

In some situations, the destruction of the myelin sheath occurs, which indicates demyelination. Two major factors lead to the development of this process. The first is associated with a genetic predisposition. Without the influence of any obvious factors, some genes initiate the synthesis of antibodies and immune complexes. The antibodies produced are able to penetrate the hemato-encephalitic barrier and cause the destruction of myelin.

At the heart of another process is infection. The body begins to produce antibodies that destroy proteins of microorganisms. However, in some cases, proteins of pathological bacteria and nerve cells are perceived as identical. Confusion occurs and the body infects its own neurons.

At the initial stages of defeat, the process can be stopped and reversed. Over time, the destruction of the shell comes to such an extent that it completely disappears, baring axons. The substance for impulse transmission disappears.

The reasons

The basis of demyelinating pathologies are the following main reasons:

  • Genetic. In some patients, a mutation of genes responsible for proteins of the myelin sheath, immunoglobulins, and the sixth chromosome is noted.
  • Infectious processes. Launched by Lyme disease, rubella, cytomegalovirus infections.
  • Intoxication. Long-term use of , drugs, psychotropic substances, exposure to chemical elements.
  • Metabolic disease. Due to diabetes mellitus, there is a malfunction of the nerve cells, which leads to the death of the myelin sheath.
  • Neoplasm.
  • Stress.

Recently, more and more scientists believe that the mechanism of nucleation and development is combined. Against the background of hereditary conditionality under the influence of the environment and pathologies, demyelinating diseases of the brain develop.

It is noted that most often they occur in Europeans, some parts of the United States, the center of Russia and Siberia. They are less common among the population of Asia, Africa, and Australia.

Classification

Describing demyelination, doctors talk about myelinoclasia, the disruption of the neuronal membrane due to gene factors.

A lesion that occurs due to diseases of other organs indicates myelinopathy.

Pathological foci occur in the brain, spinal, peripheral parts of the . They may have a generalized character – in this case, the damage affects the membranes in different parts of the body. With an isolated lesion observed in a limited area.

Diseases

In medical practice, there are several diseases characterized by demyelinating processes of the brain.

These include multiple sclerosis – in this form they occur most often. Other manifestations include pathologies of Marburg, Devik, progressive multifocal leukoencephalopathy, Guillain-BarrĂ© syndrome.

Multiple sclerosis

Approximately 2 million people suffer from multiple sclerosis. In more than half of the cases, pathology develops in people from 20 to 40 years old. It progresses slowly, so the first signs are found only after a few years. Recently, it is diagnosed in from 10 to 12 years. It occurs more often in women, residents of cities. More people are living away from the equator.

Diagnosis of the disease is carried out using magnetic resonance imaging. At the same time multiple foci of demyelination are detected. Mostly they are found in the white matter of the brain. At the same time, fresh and old plaques coexist. This is a sign of an ongoing process that explains the progressive nature of the pathology.

Patients develop paralysis, tendon reflexes increase, local seizures occur. Fine motor skills suffer, swallowing, voice, speech, sensitivity, ability to keep balance are disturbed. If the optic nerve is affected, vision is reduced, the color perception is reduced.

A person becomes irritable, prone to , apathy. Sometimes, on the contrary, there are bouts of euphoria. Disorders of the cognitive sphere are gradually increasing.

The prognosis is considered relatively favorable if the disease in the initial stages affects only the sensory and optic nerves. The defeat of motor neurons leads to a breakdown of the functions of the brain and its departments: the cerebellum, pyramidal and extrapyramidal system.

Marburg’s Disease

The acute course of demyelination, leading to death, characterizes Marburg disease. According to some researchers, pathology is a variant of multiple sclerosis. In the first stages, damage occurs predominantly to the brain stem, as well as to the conduction paths, this is due to its transience.

The disease begins to manifest itself as a normal , accompanied by fever. Seizures may occur. In a short time in the affected area is a myelin substance. Movement disorders appear, sensitivity suffers, intracranial pressure increases, the process is accompanied by vomiting. The patient complains of severe headaches.

Devika’s Disease

Pathology is associated with demyelination of the spinal cord and visual nerve processes. Paralysis develops rapidly, sensitivity disorders are noted, and organs located in the pelvic area are disturbed. Damage to the optic nerve leads to blindness.

The prognosis is poor, especially for adults.

Progressive multifocal leukoencephalopathy

Mostly, older people show signs of a demyelinating disease of the central nervous system characterizing progressive multifocal leukoencephalopathy.

Damage to nerve fibers leads to paresis, the occurrence of involuntary movements, impaired coordination, reduced intelligence in a short time. In severe cases, dementia develops.

Guillain-Barre syndrome

Similar development has Guillain-Barre syndrome. More peripheral nerves are affected. Patients, mostly men, have paresis. They suffer from severe pain in muscles, bones and joints. Signs of arrhythmia, changes in pressure, excessive sweating speak of dysfunction of the vegetative system.

Symptoms

The main manifestations of demyelinating diseases of the nervous system include:

  • Movement disorders. There are paresis, tremor, involuntary movements, increased muscle rigidity. Observed coordination of coordination, swallowing.
  • Neurological. Epileptic seizures may occur.
  • Loss of sensation The patient perceives temperature, vibration, pressure incorrectly.
  • Internal organs. There is urinary incontinence, bowel movement.
  • Psycho-emotional disorders. Patients noted a narrowing of the intellect, hallucinations, increased forgetfulness.
  • Visuals Visual impairment is detected, the perception of colors and brightness is disturbed. They often appear first.
  • Overall well-being. The person quickly gets tired, becomes sleepy.

Diagnostics

The main method for detecting foci of demyelination of the brain is magnetic resonance imaging. It allows you to make an accurate picture of the disease. The introduction of a contrast agent outlines them more clearly, allowing you to isolate new lesions of the white matter of the brain.

Other methods of examination include blood samples, the study of cerebrospinal fluid.

Treatment

The main goal of treatment is to slow down the destruction of the myelin sheaths of nerve cells, normalize the functioning of the immune system, and combat antibodies that infect myelin.

Immunostimulating and antiviral effects have interferons. They activate phagocytosis, make the body more resistant to infections. Assigned to Copaxone, Betaferon. Intramuscular administration is indicated.

Immunoglobulins (ImBio, Sandoglobulin) compensate for the natural human antibodies, increase the patient’s ability to resist viruses. Appointed mainly in the period of exacerbation, administered intravenously.

Dexamethasone and Prednisalone are prescribed for hormone therapy. They lead to a decrease in the production of antimyelin proteins and prevent the development of inflammation.

In order to suppress the immune processes in severe cases, cytostatics are used. This group includes, for example, Cyclophosphamide.

In addition to drug treatment, plasmapheresis is used. The purpose of this procedure is blood purification, removal of antibodies and toxins from it.

Spinal fluid is being cleaned. During the procedure, liquor is passed through the filters in order to clean it of antibodies.

Modern researchers are treating stem cells, trying to apply the knowledge and experience of gene biology. However, in most cases, it is impossible to completely stop demyelination.

An important part of the treatment is symptomatic. Therapy is aimed at improving the quality and duration of life of the patient. Nootropic drugs are prescribed (Piracetam). Their action is associated with increased mental activity, improved attention and memory.

Anticonvulsant medications reduce muscle spasms and associated pain. These are Phenobarbital, Clonazepam, Amizepam, Valparin. To relieve rigidity prescribed muscle relaxants (Mydocalm).

The characteristic psycho-emotional symptoms are weakened when taking antidepressants, sedatives.

Forecast

Demyelinating diseases found in the initial stages of development can be stopped or slowed down. Processes that deeply affect the central nervous system have a less favorable course and prognosis. In the case of pathologies of Marburg and Devik, death occurs in a matter of months. In Guillain-Barre syndrome, the prognosis is quite good.

Hereditary predisposition, infections, and metabolic disorders cause the damage to the myelin sheath of the cells of the brain, spinal cord, and peripheral nerves. The process is accompanied by the development of serious diseases, many of which are fatal. To stop brain damage prescribed drugs, designed to reduce the speed and development of demyelination. The main goal of symptomatic treatment is to alleviate the symptoms, improve mental activity, reduce pain, involuntary movements.

The following sources were used to prepare the article:

Ponomarev V.V. Demyelinating diseases of the nervous system: clinic, diagnostics and modern treatment technologies // Journal Medical News – 2006.

Cherniy V. I., Shramenko E. K., Buvaylo I. V., Ostrovaya T. V. Demyelinating diseases of the nervous system and the possibilities of differential therapy in acute and subacute periods // International Neurological Journal – 3 (13) 2007.

Sineok E. V., Malov I. V., Vlasov Ya. V. Early diagnosis of demyelinating diseases of the central nervous system based on optical coherence tomography of the fundus // Journal Practical Medicine – 2013.

Demyelinating disease of the brain and central nervous system: the causes of development and prognosis

Totolyan N. A. Diagnostics and differential diagnosis of idiopathic inflammatory demyelinating diseases of the central nervous system // Electronic dissertations library – 2004.

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