Leukoencephalitis - symptoms and treatment methods. Treatment of inflammatory diseases of the central nervous system in dogs Hearst's encephalitis

Leukoencephalitis is a special form that is of viral origin. The white matter of the brain is mainly affected and there are dystrophic changes in neurons.

However, it is not possible to understand exactly which viruses provoke the development of the disease. It is assumed that these may be measles, rabies, and herpes viruses of the third type.

Three types

There are three known types of the disease. The first is called acute hemorrhagic leukoencephalitis, and is extremely difficult, while there is a pronounced process of demyelination of nerve endings and white medulla. The disease is considered one of the most unfavorable and death occurs literally two days after the onset of the first signs.

Schilder's leukoencephalitis is a degenerative-demyelinating disease of the brain, which is characterized by the formation of large areas of demyelination that tend to merge. It has a constantly progressive course and non-specific symptoms.

Acute hemorrhagic leukoencephalitis of Hurst resembles the first type in its course, but has minor differences from it, which can only be determined after a thorough examination.

Why does it appear

The causes of leukoencephalitis remain in question. There is some evidence that measles viruses are to blame here, so anyone who has had this disease should be more attentive to their health.

Also among the reasons is called such a condition, known as post-vaccination complications. However, it is not yet possible to say for sure that this is so.

Only one thing is clear - a virus that enters the body or begins its vigorous activity is considered a trigger. But that's why this process occurs, so far it has not been possible to find out.

Clinical picture

Leukoencephalitis of the brain does not have symptoms that would make it possible to make a correct diagnosis without difficulty after the first visit to the doctor. Basically, the disease is detected in childhood, although sometimes symptoms can appear at an older age.

The main symptoms appear after vaccination in children who have certain immune diseases or abnormalities in neurological health.

The disease progresses very quickly, and despite its causes, after a year or a year and a half, the patient becomes deeply disabled, and then death occurs due to respiratory failure or a long-term epileptic seizure.

The peculiarity of the disease is that all the symptoms begin to appear quite suddenly and progress very quickly. Literally before our eyes, a healthy child becomes unable to move or perform other previously habitual actions.

Hemorrhagic leukoencephalitis can also be subacute. In this case, the symptoms will not be so acute, and the disease will not progress so quickly. Patients experience impaired consciousness, often developing coma, which can also cause death.

Neurological symptoms become noticeable already in the first month of illness. It should be noted here:

1. hallucinations.
2. Increased muscle tone.
3. Agnosia.
4. Apraxia.
5. Hyperkinesis.
6. Paresis.
7. Paralysis.
8. Violation of coordination of movement.

Despite its causes, acute hemorrhagic leukoencephalitis is easily confused with other neurological pathologies, so often death occurs even without a correct diagnosis.

How to recognize

The diagnosis is made mainly in accordance with research methods such as CT or MRI. They allow you to understand the severity of the disease and what is the state of the brain at the moment.

Treatment

Acute leukoencephalitis has no specific treatment, since it has not yet been possible to understand what caused the disease. Drugs such as corticosteroids are used. Basically, it is prednisolone at a dose of 1 - 1.5 mg per kilogram of body weight. The duration of treatment is from 2 to 3 weeks.

If there is a positive result, the drug is administered further in order to maintain normal health. Antibiotics may also be prescribed, most often penicillin, erythromycin, tetracycline.

A general increase in the intake of potassium preparations is mandatory, but foods with a high content of sodium should be discarded. If necessary, as an additional treatment - vitamin therapy and psychotropic drugs.

Forecast

The prognosis is always unfavorable. Death occurs one and a half to two years after the onset of the first symptoms. Sometimes there is a chronic course, while during the period of remission there are no symptoms, but they occur during the period of exacerbation.

Leukoencephalitis (LE) Schilder- diffuse sclerosis was described by the author in 1912. The disease is characterized by severe bilateral demyelination of the cerebral hemispheres and the brain stem (with fairly intact axial cylinders), a pronounced glial and aocular inflammatory reaction. The morphological picture and features of the course of the disease allow us to attribute it to the group multiple sclerosis.
Schilder's leukoencephalitis is equally common in both childhood and adulthood. The onset of the disease is usually gradual, very rarely stroke-like. The first main manifestations of leukoencephalitis may be behavioral changes, progressive disorders of higher mental functions(visual and auditory gnosis, praxis, speech, intellect), epileptic seizures, psychotic states, pyramidal paresis. There is no "typical" picture of LE. In some cases, the disease begins under the guise of a brain tumor, in others it proceeds as a mental illness, in others it resembles multiple sclerosis. Such a variety of clinical manifestations is due to the diffuse nature of the demyelinating process in the cerebral hemispheres, the size of the foci of demyelination, and the severity of perivascular edema.
The most common and typical form of Schilder's leukoencephalitis is pseudotumorous. With it, signs of intracranial hypertension (headaches with vomiting, progressive congestive changes in the optic discs) accompany increasing, predominantly unifocal symptoms (Jacksonian seizures, pyramidal hemiparesis, central homonymous hemianopsia).
Often, focal pathological activity on the EEG, a slight increase in cerebrospinal fluid pressure, and protein-cell dissociation (protein from 0.7 to 3.3%o with normal cytosis) are detected.
The features of the pseudotumorous form of LE in comparison with the tumor process are: the presence of signs of multifocal and bilateral lesions; a tendency to fluctuations in the severity of symptoms and remissions, typical of the demyelinating process; dissociation between congestive changes in the optic discs and the absence of an increase in the pressure of the cerebrospinal fluid and hypertensive changes on the graph; in the liquid, significant hypergammaglobulinorahia, frequent pathological changes in the Lange reaction; on the EEG - early gross diffuse changes; lack of M-echo shift on echoEG; in remission - a decrease in protein-cell dissociation in the cerebrospinal fluid, focal changes in the EEG.
Complex therapy should be directed, as in the treatment of multiple sclerosis, primarily to suppress autoallergic reactions and include hormones of the adrenal cortex, as well as desensitizing agents. Among the symptomatic agents, the most important are anticonvulsants and myotonolytics.

Leukoencephalitis is a special form of encephalitis that is caused by a viral infection and has an autoimmune nature. This disease is distinguished by one characteristic feature in which the white and gray matter of the brain is affected. Most often, the disease takes the form of sclerosing encephalitis, usually found in childhood and is always accompanied by destruction of the myelin sheath.

Disease history

The first cases that were reported in medical practice regarding leukoencephalitis are inflammations of the brain caused by Japanese mosquito bites or infected ticks. Some clinical studies have linked the disease to the herpessymplex family. And, nevertheless, the analysis of blood and cerebrospinal fluid did not show the activity of the above viruses. The same applies to serological studies.

In the future, many doctors made attempts to identify other etiological factors, but they also did not give the desired results. Some forms of leukoencephalitis have been associated with Kuru disease and the rabies virus. In this case, the disease could develop as a form of vaccinal encephalitis and, at the same time, was of an autoimmune nature.

Most of the first studies in the field of virology nevertheless came to the conclusion that various viruses cause leukoencephalitis. As such, a single causative agent has not been identified, so the main theory regarding the disease is based on an incorrect response of the immune system to an external agent.

In this case, the virus can provoke both an acute course of the disease and a chronic one.

Chronology of studies on the etiology of leukoencephalitis:

Leukoencephalitis: general information

The disease is based on a number of pathomorphological changes that lead to white matter demyelination. This affects the cerebral hemispheres, in which the axial cylinders are predominantly affected.

The disease is characterized by a rapid course, while mental disorders can develop at the very beginning. Such patients, even in the early stages, notice a sharp weakness, they gradually lose interest in everything that happens around.

Against this background, speech disorders are already noted.

As the disease progresses and the degree of brain damage, many skills are lost, motor skills are weakened. This becomes noticeable by the pronounced stiffness and awkwardness. Behavior at the same time begins to differ from social norms and can reach an extreme degree of dementia, a violation of self-service joins.

If at this stage the progression of the disease is not stopped, then numerous neurological manifestations and hyperkinesis develop. The further condition can be critical, since cardiac activity is disturbed. Cachexia and rigidity of the limbs appear, as a result of which a lethal outcome can occur quite quickly.

Characteristic features of the disease

A typical manifestation of panencephalitis is focal or diffuse demyelination, which is noted in all parts of the brain, there is also a pronounced expansion of the furrows and complete atrophy of the convolutions. In those patients who underwent histological studies, inflammatory and destructive processes were most pronounced in the gray and white matter of the brain tissues.

The myelin that is destroyed during the course of the disease is usually already fully formed. This type of demyelination can also be observed in leukodystrophy and neurotic idiocy. In addition, the proliferative reaction of glia due to the action of the virus is pronounced. As a result, there is a compaction of the cortical substance of the brain.

If the disease is acute, then cerebral edema is almost always present. Gliosis in this case can be both large-focal and small-focal. Hemorrhages in the brain are usually punctate due to fibrinoid necrosis of the walls of the vascular plexuses.

What is neuroinfection

With leukoencephalitis, the cause of the disease is always the penetration of an external agent: first into the bloodstream, and then into the brain. Various forms of CNS damage can be caused by viruses, bacteria and fungi. In some cases, the disease can manifest itself against the background of an extensive craniocerebral injury, when the path for infection is open.

The severe course of leukoencephalitis and meningitis is often mixed, then the bone marrow is also involved in the pathological process. Depending on the prevailing signs, treatment tactics are selected.

If the disease is acute, in addition to the main signs of leukoencephalitis, other reactions from the body are also observed. The patient may have a high temperature, migraine-like conditions, general weakness, muscle rigidity. When conducting laboratory studies, there is an increase in the erythrocyte sedimentation rate, neutropenia, an increase in the concentration of lymphocytes.

Schilder's leukoencephalitis

For the first time the disease was considered as an independent pathology at the beginning of the last century. The scientist Schilder then introduced the term diffuse leukoencephalitis. This form of the disease was characterized by extensive areas of myelin disturbance. The doctor suggested that the basis of leukoencephalitis is a cerebral lesion of the brain.

The disease manifests itself in childhood, so some experts also gave it the name of childhood multiple sclerosis. Other scientists are of the opinion that pathology has no age restrictions and absolutely everyone can be at risk.

A characteristic feature of the disease is that it has a slow course.

Demyelination occurs in the same way as viral diseases, such as herpes infection and the measles virus. Immunity to the invasion of a pathological agent begins to secrete antibodies that are autoimmune in nature, and the cerebral inflammatory process covers the entire brain.

With Schilder's leukoencephalitis, there is a violation of the regulatory systems of lipid metabolism, respectively, metabolic processes begin to work incorrectly. The areas of damage to the white and gray matter of the brain have clear outlines, but are formed randomly. This feature is also characteristic of hereditary leukodystrophies.

In some cases, foci of demyelination can form in the brain stem and cerebellum. If the disease manifested at a later age, then zones of pathological foci in the form of plaques, which are characteristic of multiple sclerosis, can be observed.

Symptoms of the disease

The clinical picture in this form of leukoencephalitis can be unstable and is marked by polymorphic symptoms. The first signs of the disease may be associated with the appearance of mental disorders and loss of skills. Patients have bouts of apathy, behavioral disorders, intellectual abilities are significantly reduced.

Against the background of damage to the central nervous system and regulatory centers, optic neuritis, strabismus, hearing loss, bulbar disorders are possible. Speech skills are significantly reduced, cerebellar ataxia and tremor appear. This occurs if the inflammatory process affects the cerebellum.

Extrapyramidal disorders are manifested in the form of torsion dystonia and weakness, as well as hyperkinesis. A convulsive syndrome often joins this condition, while epileptic seizures have a severe course. In the later stages of the disease, tetra- and hemipaesis are noted, which are related to pyramidal disorders.

Diagnostics

Schilder's leukoencephalitis includes a whole symptom complex that is characteristic of this particular form of the disease, so it is not difficult to determine the disease even at the very first stage of development. In isolated cases, the clinical picture may be similar to some variants of multiple sclerosis or resemble a psychiatric pathology.

To identify pathological changes, a neurologist must make an accurate history of the disease, as well as collect a maximum of diagnostic and clinical data.

To diagnose visual and hearing impairments, consultation with other specialists will also be required. This is necessary in order to differentiate Schilder's leukoencephalitis from similar diseases.

The most informative and minimally invasive diagnostic method is MRI of the brain. This procedure allows you to identify all lesions in the structures. When conducting electroencephalography, a decrease in alpha activity is diagnosed, and in some cases, epileptic signs can be recorded.

If a study of the cerebrospinal fluid is carried out, then an increase in the concentration of gamma globulin is detected against the background of a decrease in the total weight of the protein component. In all patients, demyelination zones usually reach 3 cm in diameter. With Schilder's leukoencephalitis, disseminated encephalomyelitis or stroke cannot additionally develop.

Other laboratory studies are aimed at excluding the pathology of the adrenal glands, which is typical for many neuroinfections. The level of fatty acids in the blood in such patients usually remains unchanged, so it is important not to confuse this disease with leukodystrophy.

In some cases, additional examination of brain tissue may be required to determine the exact type of cerebral lesion.

Therapy and further prognosis

To date, the pathogenesis of leukoencephalitis has not been fully understood. This greatly complicates the development of techniques aimed at improving the patient's condition and delaying the death. As complex therapy glucocorticoid drugs may be prescribed against the background of correction of the immune status. For this purpose, patients are prescribed methylprednisolone at the maximum dosage.

To maintain the function of the central nervous system, various antioxidants and neuroprotectors are prescribed. On the initial stage diseases, vascular drugs can give a good effect. To reduce cerebral edema, a course of diuretics is prescribed. If pronounced mental deviations are noted, then psychotropic drugs are indicated.

To improve the prognosis, therapy should be aimed at maximum containment of the demyelination process and prevention of the formation of new necrotizing foci in the brain tissue. Treatment, however, cannot stop the progressive course of leukoencephalitis and is palliative. A lethal outcome can occur both in six months and in 5 years.

Measles virus and allergic reactions as the cause of the disease

Some experts, taking into account all previous studies, rely on the fact that the measles virus is found in most patients in the blood and biopsy. This is due to the fact that this pathogen can be in the blood for a long time, as well as in the cerebrospinal fluid.

At the same time, the concentration of virus antibodies is several times higher than the permissible values, which may indicate a connection between measles and leukonecephalitis.

In some patients, during the electron microscopic method, rather large microviruses are found in the brain, which can also be associated with measles infection. All these research data provide for further study of the disease, especially in people at risk.

Against this background, an analysis is also carried out in the field of immunology regarding the allergic reaction of the body to the ingress of a pathogen from the outside. It was taken into account that the disease is viral in nature, and the main reason lies in the wrong, autoallergic response of the body. In this case, the virus is just one of the links in a complex pathological process.

The theory of the allergic nature of the disease is also confirmed by the fact that patients have morphological changes that are characteristic of autoimmune encephalitis. In many patients, regardless of age, dystrophic changes in the adrenal cortex are diagnosed, which indicates a malfunction of the immune system and leads to the fact that encephalitis acquires a progressive course.

AT this case a noticeable clear relationship in the violation of the pituitary-hypothalamus-adrenal cortex system and the development of allergic and autoimmune reactions. This feature allows leukoencephalitis and multiple sclerosis to be included in one group and to develop a common mechanism for minimizing negative manifestations on the brain.

Some studies point to the fact that the virus, penetrating into all physiological fluids, can “mask” well. This leads to the fact that infectious encephalitis becomes chronic. In this case, the clinical picture will already be blurred, the central nervous system suffers not from the virus itself, but from the waste products of the pathogen in the brain tissues.

The entry gate for viral infection is the upper respiratory tract. After ingestion, the pathogen begins to multiply in epithelial cells, after which it spreads through the bloodstream throughout the body. The incubation period is about two days.

The measles virus is quite active in all epithelial tissues. During the diagnosis, it is easy to detect in the histological material. After the end of the incubation period, a new wave of viremia is noted. This disease is manifested by damage to the conjunctiva, skin and epithelium of internal cells.

With a sharp decrease in immunity, the virus penetrates into the brain tissue, causing damage to its structures. If infection of the lymphatic tissue is observed, then the pathogen can settle in the thymus, spleen, tonsils. When diagnosing the respiratory tract, foci of necrosis are often diagnosed.

After a measles infection, a specific allergic restructuring may persist in the body for a long time. Also in the blood at this stage, antibodies to the virus can be detected.

In some cases, the disease may be accompanied by the addition of secondary bacterial infections. Then the patient's condition deteriorates sharply.

Acute hemorrhagic leukoencephalitis

This form of brain damage is the least common. When conducting a series of studies in all patients, cerebral edema is detected. The affected areas of the connective tissue acquire a soft pinkish-gray or yellowish color. During subsequent histology, extensive areas of fibrinous necrosis of the walls of small vascular plexuses are noted.

Foci of demyelination in the perivascular zones are characterized by significant destruction of axons. At the very beginning of the disease, infiltrates in the white matter are represented by neutrophils, but accumulations of plasma cells, macrophages and lymphocytes are also present.

Acute hemorrhagic leukoencephalitis is characterized by extensive damage to the central nervous system. Almost all patients have vasculitis, and hemorrhages affect not only the cerebral hemispheres, but also the spinal column. If the foci merge, then extensive necrotic areas are formed.

All patients must undergo a series of laboratory tests that allow us to differentiate the disease from acute form disseminated encephalomyelitis. In the blood, there is a sharp increase in ESR and leukocytosis. In the cerebrospinal fluid, cytosis is pronounced, and there is also a significant increase in protein concentration.

The presence of an autoimmune process confirms that with this form of leukoencephalitis, antibodies to their own protein, myelin, are detected.

Some scientists suggest that the disease can be attributed to one of the varieties of acute multiple sclerosis. In both cases, an important characteristic is that demylinization is accompanied by significant infiltration in different zones.

A similar morphological picture can also be with cerebral purpura, which is accompanied by severe anaphylactic lesions. nervous system. This is especially true with the introduction of large doses of penicillin. Perivenous encephalitis can also have a similar clinical presentation and is the result of a serum complication.

Acute hemorrhagic leukoencephalitis progresses quite rapidly. Death can occur as early as 2 weeks after infection. If the disease has taken a chronic course, then the central nervous system receives irreversible organic changes.

For this brain lesion, edema is primarily characteristic. On a section of the hemispheres, pronounced foci of pink-gray color can be found, which can have many small and heterogeneous hemorrhages. When conducting a histological examination, necrotization of the walls of small vessels is noted, which can be combined with destruction of axons.

Van Bogart leukoencephalitis is most commonly diagnosed in boys under the age of 12. In adolescence, the disease almost never occurs. The very first signs are pronounced irritability, lethargy, attacks of aggression. Gradually, children lose their speech, it becomes more and more scarce.

Against the background of gradual dementia, apraxia develops, to which various hallucinations join. Leukoencephalitis is characterized by extensive neurological symptoms.

When carrying out diagnostics by the EEG method, paroxysmal periodic activity is recorded with the registration of slow high-voltage waves.

The defeat of the flexor muscles leads to the fact that stiffness of movements appears. The defeat of the vestibular apparatus is accompanied by a tremor of the limbs and a shaky gait. In the later stages of the development of the disease, the function of swallowing, swallowing reflexes are disturbed. Accession of bulbar paralysis is noted extremely seldom.

Van Bogart's leukoencephalitis can proceed in a delayed phase for a long time. Also, this form of brain damage is characterized by long periods of remission, but recovery does not occur. As the function of the central nervous system is impaired, other organs join the pathological process, and as a result, the patient dies.

Diagnosis and treatment

Such a diagnosis is not difficult, since a number of studies confirm the development of the disease. First of all, it also takes into account the fact that clinical manifestations are progressive. Special attention is paid to the defeat of the cerebellar and pyramidal systems.

If the analysis of cerebrovascular fluid is carried out, in which high titers to the measles virus are often found. Computed tomography reveals various foci of atrophy of the cerebral hemispheres.

With an increase in intracranial pressure, ventricular expansion is often noted, while the concentration of white matter, including its density, is significantly reduced.

As such, there is no treatment that will cure the patient. If the disease is acute, then the patient should be in the intensive care unit. The therapeutic course includes necessarily anticonvulsant drugs, muscle relaxants, B-group vitamins and vascular drugs.

Arachnoiditis as a complication of leukoencephalitis

If the disease is intense, then against the background of a sharp drop in immunity, concomitant secondary infections may join. In this case, in addition to leukoencephalitis, the pia mater and spinal cord may be involved in the pathological process.

Arachnoiditis is not associated with purulent processes; in the diagnosis, its characteristic feature is that there is a noticeable tendency to form numerous adhesions in the subarachnoid space. This leads to the fact that the full movement of the cerebrospinal fluid (cerebrospinal fluid) is disturbed, against this background, the cranial pressure rises significantly. This complication can also be due to measles infection.

When arachnoiditis of the posterior cranial fossa has developed, all signs of the disease can occur suddenly. In this case, the entire brain stem and cerebellum are always affected.

The headache is so severe that it is usually accompanied by vomiting. Sometimes a change in the position of the head helps to alleviate the condition a little.

Symptoms infectious disease difficult to confuse with any others. The first thing the patient encounters is a sharp rise in temperature. All this is accompanied by vomiting, a sharp headache, weakness and dizziness. After the disease takes an active form, the general condition worsens even more. The entire CNS is involved in the destructive process, while neurological symptoms often depend on the location of the site of inflammation.

If the infectious process most of all affected the arachnoid membrane, then convexital arachnoiditis is diagnosed. Against the background of leukoencephalitis, convulsive seizures begin to occur in patients, in addition, paresis of the limbs develops and their general sensitivity decreases significantly.

Optic-chiasmal arachnoiditis most negatively affects the organs of vision. The disease progresses quite rapidly, leading to complete blindness. At the initial stage, only a change in the field of view, as well as a decrease in acuity, is noted. Patients experience prolapse of the temporal and nasal concentration fields. In addition to everything, insomnia, a violation of carbohydrate and water-salt metabolism can become.

If the inflammatory process touches the cerebellopontine angle, then the appearance of tinnitus, dizziness is characteristic. Tics of the facial muscles and sharp pains may develop. The tone of hearing drops significantly, which often leads to complete deafness. All these signs develop against the background of improper coordination of movement.

Most often, leukoencephalitis can be complicated by arachnoiditis of the internal auditory canal. The inflammatory process in this case does not capture the cerebellum, but the disease itself takes a protracted course. Patients in this situation may complain of tinnitus, dizziness.

When there is a lesion (necrotization) of the cranial nerves, the signs of the disease may not appear so clearly. In addition to brain damage, the facial and trigeminal nerves are affected. If the cerebellum is infected, then the patient has serious violations in the coordination of movements.

When cerebrospinal fluid accumulates in excess in the brain and intracranial pressure melts, tonic convulsions develop, accompanied by intermittent breathing.

To identify the disease, first of all, the fundus is examined, this allows you to determine the increase in intracranial pressure. Pneumoencephalography reveals the adhesive process, as well as an increase in the size of the cerebellum. All this indicates an acute inflammatory process and significantly worsens the prognosis for leukoencephalitis.

Research on cerebral leukoencephalitis

To date, there are many hypothetical models that are aimed at studying the causes that provoke the disease. Particular importance is given to infectious agents that penetrate into the bloodstream and lymphatic system and cause damage to immunocompetent cells.

Practically in all patients, when conducting a detailed diagnosis of the immune status, there is a deficiency of IL-2. This leads to the fact that the protective function becomes untenable due to an incorrect response of the intrathecal T-cell link, all this becomes the cause of hyperactivation of another link - the B-cell. The consequence of all this is the development of an autoimmune process.

The persistence of pathogens in the glial cells of the brain causes the development of an acute demyelination process. Gradually, this leads to the destruction of white and gray matter and irreversible processes in the defeat of the central nervous system.

A number of studies conducted among children of the same age made it possible to identify criteria that can give a negative prognosis for the course of leukoencephalitis. Most often, patients had a multifocal lesion of the cerebral cortex, while the pathological process captured precisely the white matter.

Multiple sclerosis was diagnosed in 90% of children against the background of leukoencephalitis. Closer to adolescence, due to improper functioning of the adrenal glands, retrobulbar neuritis and abnormal development of sexual function due to hormone deficiency developed. In girls, during laboratory tests, the level of progesterone dropped significantly.

Clinical studies in the field of immunology are a range of studies that include the analysis of clinical manifestations, blood biochemical parameters, neuroendocrine abnormalities. In some cases, timely diagnosis may slightly improve the course of the disease, but the prognosis will still be unfavorable.

Therapy for leukoencephalitis includes immunocorrective tactics, which reduces the activity of the autoimmune response. Also, other pathogenetic factors are taken into account, the elimination of which makes it possible to reduce the rate of neurological deficit.

Timely extracorporeal detoxification reduces the frequency of exacerbations of epileptic seizures by several times against the background of a slowdown in the development of dementia.

Numerous immunological studies have been carried out regarding the viral origin of leukoencephalitis. So, tests on hamsters have shown that the disappearance of the measles virus in the brain is fatal. At the initial stage, local areas are noted, after which they already become extensive.

The gradual spread of a viral infection in the cerebral cortex of animals led to damage to the myelin fibers between the subcortex of the white matter and the cortex. At the same time, specific lymphatic infiltrates are formed around the vessels, subcortex and neurons, which are a kind of antigen-antibody response.

A complete lesion of the white matter is called "nodular panecephalitis". Other researchers call this condition "subacute encephalitis". This vague classification is due to the fact that many encephalitis have a similar clinical picture.

In neurological practice, a number of procedures have been developed that are aimed at the timely detection of the disease.

The first thing to consider here is an integrated approach.

What should include diagnostics:

Treatment of leukoencephalitis

At the first stage, corticosteroids are necessarily prescribed. The dosage is calculated taking into account the weight of the patient, so that it does not exceed 1 mg per 1 kg. To maintain the therapeutic effect, the hormonal course can be quite long. If the period of remission is replaced by an exacerbation, then the daily dose may be increased.

Since prednisolone therapy is accompanied by a decrease in general immunity, it is imperative to prescribe antibacterial agents for the duration of treatment. It is also important to observe the water-salt balance, potassium preparations can be prescribed in parallel.

As the pathological process progresses, therapy becomes ineffective. In patients, various negative symptoms are increasingly noted - lethargy increases, skills and interest in the outside world are lost. In some cases, antioxidants and B-group vitamins help maintain activity.

Later phases of the disease are accompanied by various mental disorders. With the development of psychoses and hallucinations, tranquilizers and psychotropic drugs are used. The use of anticonvulsants is indicated for epileptic seizures.

Except drug treatment it is recommended to conduct dispensary monitoring of the activity of leukoencephalitis. The duration of observations may be different depending on the focal lesions of the brain. If the disease has become protracted, then clinical and neurological control will be carried out less frequently.

In addition to observation by a neurologist, patients are shown a mandatory periodic examination by an endocrinologist, psychiatrist and ophthalmologist. To identify vascular disorders, dopplerography is performed. In the future, the program of surveys and monitoring is carried out according to the standard scheme.

Leukoencephalitis is one of the variants of brain damage, which is destructive. The disease has a progressive course and is accompanied by a gradual destruction of the white matter of the brain. Leukoencephalitis is based on either an infectious-allergic reaction or an autoimmune process. In both cases, the consequence of extensive pathology is demyelination.

Schilder's leukoencephalitis: the use of MRI diagnostics - on video.

Symptoms appear gradually and steadily progress or remit. The earliest include mental disorders, which in young children are manifested by apathy, lethargy or irritability, capriciousness, disobedience, unmotivated aggressive behavior. Children lose their neatness skills, speech becomes more scarce, the logic of thinking disappears. Against the background of mental disorders gradually, over several weeks or months, focal neurological symptoms develop (apraxia, agnosia, visual and auditory hallucinations, polymorphic hyperkinesis, increased muscle tone according to the plastic type evenly in the flexion and extensor muscle groups, which leads to stiffness of movements) . Later, spastic mono-, hemi- or tetraparesis and paralysis develop. There are also violations of coordination of movements and unsteady gait (of cerebellar or frontal origin). Phonation and swallowing disorders are usually associated with high palatal and pharyngeal reflexes. Bulbar palsy is rare.

Constant symptom leukoencephalitis convulsions are, more often in the form of small or abortive seizures, interspersed with rare extended seizures. In the late stage of the disease, vegetative and trophic disorders join: cachexia, profuse sweat, hiccups, thermoregulation disorders, bedsores, etc. In the terminal stage, patients are immobilized, states of decerebrate rigidity may occur.

The EEG registers periodic paroxysmal activity with an interval of 5-15 s simultaneously in most leads in the form of slow (1-2 s) high-voltage waves. As the disease progresses, these changes become more pronounced.

There may be in the blood leukocytosis, the fraction of alpha-globulin is increased. Patients usually have an elevated titer of antibodies to the measles virus or to myxoviruses - the JC virus or SV-40.
in the cerebrospinal fluid in most cases, there is no cytosis and no increase in protein content. In the electrophoretic study of proteins, however, it is found that Y-globulin makes up to 40 percent or more of the total protein, and the albumin fraction is reduced. Colloidal reactions give maximum flocculation in the first test tubes (paralytic type of Lange reaction).

When steadily progressive course patients die 2-12 months after the onset of the first symptoms. With a long course, life expectancy can be 3 years or more, and remissions last from several months to several years, during which the symptoms of the disease may decrease.

There are several klshshko-morphological variants leukoencephalitis, of which only subacute sclerosing leukoeicephalitis of Van Bogart and Dawson and acute hemorrhagic leukocephalitis are described in young children.

Van Bogart's leukoencephalitis is isolated from the group leukoencephalitis based on the regular detection of characteristic inclusions in the cells of the nervous system along with changes typical of leukoencephalitis. The severity of brain damage decreases in the direction from the cortex to phylogenetically more ancient formations, but more often than in other forms of the disease, the trunk and spinal cord are affected.

The clinical feature of this form leukoencephalitis- early manifestation and predominance of extrapyramidal disorders (hyperkinetic form), which are joined only at later stages by pyramidal symptoms. She does not have epileptic seizures.

Degenerative-demyelinating lesion of the brain, accompanied by the formation of large or confluent zones of demyelination. It has a steadily progressive course with a nonspecific and polymorphic clinical picture, which may include mental disorders, pyramidal and extrapyramidal syndromes, cognitive deficits, damage to the cranial nerves, episyndrome. Schilder's leukoencephalitis is diagnosed according to clinical criteria and MRI results after the exclusion of other pathologies with similar manifestations. Therapy is carried out with glucocorticosteroids, anticonvulsants, muscle relaxants and psychotropic drugs. However, the treatment is ineffective.

ICD-10

G31.0 Limited brain atrophy

General information

Schilder's leukoencephalitis was first considered as an independent nosology in 1912 by a psychoneurologist, whose name was firmly fixed in the name of the disease, although the author himself designated the pathology described by him with the term "periaxial diffuse leukoencephalitis". Later, descriptions of other clinical forms of leukoencephalitis were presented by various researchers: in 1941 - hemorrhagic leukoencephalitis, in 1945 - subacute sclerosing leukoencephalitis. Since the main pathomorphological substrate of the disease is diffuse zones of white matter demyelination, Schilder's leukoencephalitis is included in the group of demyelinating diseases.

The predominant age of manifestation of Schilder's disease is still a controversial issue. Foreign experts in the field of neurology consider the onset in the age period from 7 to 12 years to be characteristic, and some authors propose to classify the disease as a childhood form of multiple sclerosis. Observations of domestic neurologists, on the contrary, indicate an equal degree of damage to persons of different age categories.

Causes of Schilder's leukoencephalitis

The etiopathogenesis of Schilder's disease is under study. From the name of the disease, it can be seen that the inflammatory etiology of the cerebral lesion, i.e., encephalitis, was originally implied. A viral theory of the disease is assumed according to the type of slow infections. Among the possible infectious agents, the role of measles, herpes infection, myxoviruses, which may trigger the process of autoimmune cerebral inflammation, is discussed. However, unsuccessful attempts to isolate the pathogen led to the emergence of a different etiopathogenetic theory. The latter suggests a connection between Schilder's leukoencephalitis and dysfunction of the regulatory mechanisms of lipid metabolism, which brings the disease closer to hereditary leukodystrophy.

Morphological changes consist in the formation in the white cerebral substance of the hemispheres of significant zones of demyelination, which have clear pointed outlines and are often asymmetrically located. In some cases, such lesions form in the cerebellum and brain stem. In patients who fell ill in the pubertal period and in adulthood, cases are described when, along with areas of extensive demyelination, rounded plaque-like foci are observed, resembling plaques of multiple sclerosis.

Symptoms of Schilder's leukoencephalitis

The disease is characterized by the presence of nonspecific and polymorphic symptoms. It can manifest gradually developing mental disorders: mood lability, apathy, behavioral disorders, episodes of arousal with a hallucinatory syndrome. Intellectual decline progresses up to dementia. Agraphia, acalculia, alexia, agnosia, apraxia are observed. Due to demyelination of the cranial nerves, optic neuritis, ophthalmoplegia, hearing loss, decreased vision, and bulbar disorders occur. When the cerebellum is damaged, cerebellar ataxia, chanted speech, and intentional tremor appear. The defeat of the visual zone of the cortex leads to hemianopsia, cortical amaurosis. Extrapyramidal disorders are possible in the form of hyperkinesis, torsion dystonia, etc. Pyramidal disorders are usually observed in the later stages of leukoencephalitis in the form of mono-, hemi- and tetraparesis. Often there is a convulsive syndrome (like Jacksonian epilepsy or with generalized epileptic seizures), characterized by the absence of a specific EEG pattern.

The variability of combinations of various symptom complexes is so pronounced that it does not allow one to single out a typical variant of the course of Schilder's disease. In some cases, the clinic is similar to the progressive variant of multiple sclerosis, in others it has a pseudotumorous character, in the third it resembles a psychiatric pathology. In the latter case, patients may be treated by a psychiatrist until the development of overt neurological symptoms.

Diagnosis of Schilder's leukoencephalitis

It is very difficult to diagnose Schilder's leukoencephalitis in vivo. This task requires the neurologist to carefully compare the anamnestic, clinical and tomographic data, and carefully conduct differential diagnostics with similar diseases. In order to examine the visual and auditory analyzers, an ophthalmologist and an otolaryngologist may be involved in consultations.

Electroencephalography reveals signs of diffuse cerebral damage: a decrease in alpha activity and disorganization of the rhythm; epileptiform activity is often determined. In the study of cerebrospinal fluid, an increase in the level of gamma globulin is found against the background of a decrease in the specific gravity of the albumin fraction. The most informative method of instrumental diagnosis is MRI of the brain. Schilder's disease confirms the presence of at least one large or a pair of confluent foci of demyelination in the cerebral white matter.

To establish the final diagnosis, many neurologists are guided by the criteria of C.M. Poser 1985: according to MRI data, there are 1-2 rounded demyelination zones with a size of at least 2x3 cm; no pathology of the adrenal glands; exclusion of any other cerebral pathology (multiple encephalomyelitis, stroke, etc.); compliance with the norm of the level of fatty acids in the blood serum; identification of areas of diffuse chronic sclerosis at autopsy. In some cases, only histological studies of the cerebral tissues of the affected area allow to distinguish Schilder's leukoencephalitis from leukodystrophy.

Treatment and prognosis of Schilder's leukoencephalitis

The lack of clear ideas about the etiopathogenesis of Schilder's disease has not yet allowed the development of more or less effective methods her treatment. Some effect of glucocorticosteroid therapy was noted, in connection with which many patients are prescribed methylprednisolone, initially parenterally at a loading dose, and then orally with a gradual dose reduction. In parallel, a course of neuroprotective, antioxidant and vascular therapy is carried out, if necessary, anticonvulsant treatment (carbamazepine, diazepam), muscle relaxants (amantadine, tolperison, amidine), decongestants (furosemide, acetazolamide, magnesium sulfate), psychotropic pharmaceuticals are prescribed.

Timely treatment can only slightly delay the progression of the pathology. However, despite its implementation, all patients die. The time of onset of death varies from several months to 3 years from the onset of leukoencephalitis.