I67.3 Progressive vascular leukoencephalopathy Progressive vascular leukoencephalopathy

Vascular subcortical encephalopathy is characterized by gait disturbances, dysfunction of the pelvic organs, mild or moderate neurological symptoms (usually transient), progressive dementia, volitional and emotional disturbances. The disease develops gradually. Both a continuous progressive course and slow progression with long periods of stabilization are possible. The aggravation of symptoms is usually directly related to a prolonged increase in blood pressure. The outcome is complete helplessness, inability to self-service and lack of control over the functions of the pelvic organs.
Dementia has a character typical of vascular diseases of the brain. There is a deterioration in memory, a decrease in the level of judgments, a slowdown and instability of mental processes. Often, "emotional incontinence" is revealed - a pronounced inability to restrain emotions with a predominance of asthenic reactions. Long periods of stabilization and even temporary regression of existing disorders are possible.
Depending on the prevailing symptoms, dysmnestic, amnestic and pseudo-paralytic dementia are distinguished. With dysmnestic dementia, there is a mildly pronounced decrease in memory and intelligence, a slowdown in physical and mental reactions. Criticism of one's own condition and behavior is slightly impaired. In the clinical picture of amnestic dementia, severe memory impairment for current events prevails, while past memories are preserved. Pseudo-paralytic dementia is characterized by a stable, monotonous good mood, minor memory disturbances, and a pronounced decrease in criticism.
The clinical picture in all forms of dementia is very variable, the predominance of both cortical and subcortical disorders can be detected, while cortical disorders are accompanied by a more noticeable decrease in intellectual-mnestic activity. In some cases, epileptic seizures are observed. Dementia is accompanied by disturbances in the emotional and volitional sphere. Neurosis-like phenomena, increased exhaustion and decreased mood are possible. In the later stages of the disease, there is a limitation of interests, emotional impoverishment and loss of spontaneity.
Gait disturbances, like dementia, progress gradually. At first the steps become smaller, the patient begins to shuffle his feet, lifts his feet heavily from the ground. Subsequently, the automatism of walking is disturbed, the gait becomes slow and cautious, all movements are controlled consciously, as if the patient were walking on slippery ice. The following signs of gait disturbances in Binswanger's disease are distinguished: a decrease in stride length, a slowing down of walking, a need for increased stability, difficulty in starting to walk, and a decrease in stability when turning.

The medical term "leukoencephalopathy" is used to define a group of diseases accompanied by damage to the white matter and a number of deep brain structures. Rapid progression leads to the formation of senile dementia.

In children, there are vascular varieties, congenital forms with a long chronic course. The survival time for this type is longer compared to the multifocal counterpart.

MR image of vascular leukoencephalopathy

To distinguish the pathology from a number of other neurodegenerative diseases with similar clinical symptoms, a classification according to ICD 10 was developed, where the forms of nosology are clearly distinguished.

What is leukoencephalopathy of the brain

The defeat of the white matter of the brain is caused in most cases by viruses. Vascular, dyscirculatory forms are caused by a violation of the blood supply to a certain area of ​​the brain. Chronic ischemia causes irreversible changes.

Clinical symptoms of the disease often occur when affected by papillomaviruses. The probability of nosology in patients with HIV is less than 6%.

Forms of vascular genesis progress slowly. The chronic course of the disease is characterized by gradual irreversible tissue damage. Mild ischemia provokes the formation of small necrotic areas. Diffuse arrangement leads to neurological disorders.

Types of leukoencephalopathy

The least dangerous form is focal. It is formed by chronic inflammatory processes of vascular origin. The lack of microcirculation of a certain part of the brain provokes hypoxia, a lack of oxygen. The death of white matter zones develops over several years.

Morphological changes proceed more aggressively in hypertension. An increase in intracranial pressure causes ruptures of small capillaries with areas of necrosis of the brain parenchyma. A variety of medical language is called "discircular encephalopathy". Appears in people over 55 years of age.

Progressive multifocal leukoencephalopathy has an aggressive course. People with pathology live no more than 5 years. Fatal outcomes are associated with extensive heart attacks, strokes.

Classification of types of leukoencephalopathy according to ICD 10

The progressive type of vascular genesis (Binswanger's disease) is encoded by the symbols "I67.3". Subcortical dementia with the code "F01.2" was excluded from the classification of diseases of the tenth revision.

Progressive multifocal (multifocal) leukoencephalopathy - "A81.2". The group of the same name includes phenylketonuria, Alexander's disease, Canavan. Pathologies of the category "IA" are distinguished for reasons, since they have an autoimmune origin - they are caused by tissue damage by the body's own immunoglobulins. Antibodies become aggressive when the structure of the membrane or the genetic information of the cell changes under the influence of viruses, chemical, physical factors.

Consider the complete classification algorithm:

• Diseases of the circulatory system – “IX. 100-199";
• Cerebrovascular diseases "I60-69";
• Other cerebrovascular diseases - "I67";
• Progressive vascular leukoencephalopathy - "I67.3";
• Other specified vascular lesions - "I67.8".

The international classification of the tenth revision is valid. When coding the diagnosis, dyscirculatory encephalopathy is often encountered, acute cerebrovascular insufficiency NOS cerebral ischemia (chronic).

Clinical symptoms of small focal leukoencephalopathy

Focal symptoms have a subacute course. The initial stages of the disease are identified by neurologists:

• Visual disturbances, speech;
• Pathology of the innervation of the muscles of one half of the body;
• Epilepsy attacks;
• Headaches, dizziness;
• Ataxia, anopsia.

Differential diagnosis of focal species is carried out to distinguish it from white matter changes in HIV, dementia. Spinal lesions proceed without disturbance of mental functions. Damage to the white matter is accompanied by cognitive impairment.

Progressive multifocal leukoencephalopathy

The cause of multifocal damage to the white matter is the JC virus, leading to widespread damage to the nervous system. The disease develops against the background of reduced activity of the immune system. Antiretroviral treatment is expensive, so most people die.

Progressive encephalopathy quickly leads to the destruction of the myelin of most nerve cells. The changes are irreversible, the symptoms gradually increase.

About 80% of the country's population is a carrier of human poliomavirus type 2, but encephalopathy does not occur. Only immunodeficiencies in AIDS create the possibility of rapid reproduction of the pathogen.

The immunity of the elderly cannot cope with the activity of the polyomavirus (JC) after immunomodulatory or immunosuppressive therapy after the treatment of oncological neoplasms, organ transplant operations.

In children, the appearance of pathology is observed after the start of therapy for chronic lymphocytic leukemia, Hodgkin's disease.

The 1C virus is transmitted by airborne or fecal-oral route. Most of the population is asymptomatic. Provoking factors:

• HIV infection;
• Taking immunosuppressants;
• Lymphogranulomatosis;
• Leukemias.

Magnetic resonance imaging is the only way to identify pathological foci inside the white matter. After the appearance of visual impairment, dysarthria, hemiparesis, aphasia, neurologists will be able to suggest a diagnosis. Final verification is possible only after a microscopic examination of brain biopsy specimens - tissue sections taken from the site of injury.

Dyscircular encephalopathy

Chronic progressive course of cerebrovascular pathology is accompanied by diffuse multifocal changes leading to hemiparesis, ischemic stroke, multiple neuropsychological and neurological disorders.

Progression of dyscirculatory encephalopathy is associated with tissue degeneration, accumulation of aggressive metabolites.

Before the use of neuroimaging methods, most of the causes of cognitive disorders were explained by experts as dyscirculatory encephalopathy. Practice shows overdiagnosis of cases of nosology. Nuclear magnetic resonance indicates only 20% of the incidence of white matter lesions in elderly patients with vascular disease.

The main difference of the discircular variety in comparison with a stroke is the damage not to large cerebral arteries, but to small penetrating vessels, arterioles. Diffuse damage to small branches causes a number of morphological changes:

1. Numerous heart attacks (lacunar);
2. Diffuse destruction of white matter;
3. mixed form.

Early identification of any category prevents progression after proper supportive therapy has been instituted.

Features of periventricular and residual leukoencephalopathy in children

Chronic lack of oxygen supply, prolonged ischemia of brain tissues leads to damage to subcortical structures, hemispheres, and brain stem. Pathological foci are found in the depths of the gray matter, accompanied by changes in the subcortical fibers.

Periventricular encephalopathy is characterized by predominant localization of pathological foci around the ventricles of the brain.

The residual view has congenital and acquired causes. The provoking factor in the child are traumatic injuries of the skull, inflammatory processes inside the cranium. A separate variety - encephalomyelopathy occurs due to anomalies in the structure of the vascular network of the brain.

Symptoms of residual encephalopathy in children:

• Cerebral paralysis;
• Oligophrenia;
• Epilepsy;
• Vegetative-vascular dystonia;
• Restless sleep.

Practice shows the presence of a latent course of nosology in newborns weighing about four kilograms. Clinical symptoms appear after the start of an active blood supply. Cases of dementia in preschoolers and schoolchildren are associated with injuries of the skull.

How long do people live with encephalopathy

Life expectancy is determined by the clinical form of the disease, the rate of progression, individual changes in the human body.

Progressive multifocal encephalopathy is accompanied by a fatal outcome 1-3 years after detection. Supportive care increases survival.

Varieties of vascular genesis have chronic progression. People with this variety, with proper organization of treatment, live for decades. Reduces the time of hypertension, pronounced ischemic foci of the brain structure, hemorrhages inside the brain.

Principles of diagnosis of leukoencephalopathy

The best way to verify changes in the white and gray matter of the brain is MRI. Leukoencephalopathy is localized in the brain and spinal cord. The images show areas of high intensity with a diameter of three millimeters to three centimeters of irregular round and oval shape. The location of the foci in the subcortical and periventricular regions determines the mode of T1 weighted sequences with additional contrast (MR angiography). The method diagnoses atrophy, expansion of ventricular spaces, fresh pathological zones.

Multifocal leukoencephalopathy

MRI of the brain with leukoencephalopathy reveals areas of varying intensity in the cerebellum, brainstem, parietal-occipital and frontal regions. Infratentorial localization is less common. A brain scan should be combined with an examination of the cerebrospinal fluid. The analysis allows to identify pathological agents that are the cause of the pathology.

Direct detection of the JC virus, HIV by PCR has a reliability close to 100%. If the test is negative, confirmation or exclusion of the diagnosis is possible by biopsy - taking material from the pathological area after the detection of MRI with three-dimensional modeling.

Diagnosis of autoimmune conditions, immunodeficiency is obligatory.

Electroneuromyography registers a decrease in signal conduction along auditory, visual, somatosensory nerve fibers. There are specific places of localization of pathology, the identification of which allows us to assume pathology with a high degree of probability.

Destruction of the myelin of the nerve sheaths, verification of axon degeneration are signs of neurodegenerative processes. Pathological waves occur at the subclinical stage before the first pronounced symptoms.

To identify pathologies at the beginning of development, an MRI should be done. The procedure qualitatively shows the soft tissue component saturated with water. The liquid after exposure to a magnetic field begins to resonate, the radio frequency signal changes. Registration of an impulse allows you to display a graphic image on the monitor screen after processing by the program.

Cerebrovascular disease in the early stages is manifested by a decrease in working capacity, increased fatigue, a decrease in mood background, sleep disturbances, when the patient wakes up in the middle of the night and then cannot fall asleep. Then the symptoms of cognitive impairments join, i.e. memory decreases, thinking slows down, mental counting becomes difficult, excessive fussiness appears. In the future, persistent headaches, tinnitus, dizziness join. Brain crises develop periodically, which proceed with a gross violation of the functions of the brain and are manifested by the development of weakness in the limbs on the one hand, speech, sensitivity, and vision disorders. If these symptoms disappear within 48 hours, then they speak of a transient cerebrovascular accident. If the symptoms persist for longer, then it is a stroke. In this case, gross dysfunctions of the nervous system can persist until the end of life, making the patient disabled. A stroke can be ischemic, in case of closure of the lumen of the vessel by an atherosclerotic plaque or thrombus, or hemorrhagic, when the integrity of the vascular wall is violated and a cerebral hemorrhage occurs.