ENTsEFALOPATYYa (encephalopa-thia; Greek enkephalos brain + Greek pathos suffering, disease; synonym: a pseudoencephalitis, a tserebropatiya, entsefaloz) — the diffusion melkoochagovy damage of a brain of dystrophic character caused by various diseases and morbid conditions.
To development E. can give various factors during certain time influencing a brain. Such factors are intoxications, brain injuries, ionizing radiation, disturbances of metabolism and blood circulation of a brain. They act separately or combined and cause irreversible morfol. changes in a brain.
Distinguish inborn and acquired E. Inborn E. at children can be caused by anomalies of development of a brain, be result of impact on a brain of various disturbing factors in the perinatal period, such as pre-natal hypoxia, asphyxia in labor, a birth intracranial trauma (see. Children's paralyzes, Perinatal pathology). The pre-natal hypoxia caused Littl's disease (an inborn anoksemichesky embryopathy) — the form of cerebral palsy which is characterized by dominance of the lower spastic paraparesis. Inborn character can be had and genetically caused metabolic E., napr, family miogammaglobulinemiya (hereditary forms inborn E.). Acquired E. arise owing to impact on a brain of disturbing factors in the post-natal period. Distinguish traumatic (posttraumatic) E., developing as later or the remote complication of a craniocereberal injury, and toxic E., arising at systematic influence of neurotropic toxins, in particular various chemical substances, napr, alcohol, chloroform, cyclopropane, barbiturates, Novarsenolum, lead (see. Alcoholic encephalopathies, Poisonings, Lead), and also bacterial toxins (see Botulism, Diphtheria, Measles, Tetanus). As manifestation of a radial illness beam encephalopathies are described (see. Radial illness).
In recent years much attention is paid to studying metabolic E. Distinguish from them hepatic E., arising at various diseases of a liver, a thicket at cirrhosis, a virus, toxic or medicinal necrosis of a liver. It is described portosistemny E., developing at patients with cirrhosis after imposing of a porto-caval anastomosis (see). At the same time the toxicants which are contained in the blood of a portal vein which are not neutralized by a liver come to a big circle of blood circulation and have direct toxic effect on a brain (see. Liver failure). To metabolic E. carry group E., the kidneys tied with pathology, including uraemic E. (see. A renal failure, Uraemia), dialysis dementia and a syndrome of a dialysis imbalance (see Dialysis). Are connected with pathology of a pancreas pancreatic E., more often arising as a complication of acute pancreatitis (see Pancreatitis), endocrine E. — hypoglycemic and gipergli-kemichesky, giperosmolyarny, neke-tonemichesky E. (see a diabetes mellitus, the Hyperglycemia, the Hypoglycemia). To metabolic E. belong and
AA., connected with long parenteral food, napr, hypo-fosfatemichesky E., and also Gayet's syndrome — Vernike who is marked out at hron. alcoholism (see. Alcoholic encephalopathies).
Version metabolic E. E is., developing at the syndrome of inadequate secretion of antidiuretic hormone (ADG) arising at various diseases and patol. states, napr, at malignant tumors, bleedings, injuries, endocrinopathies, diseases of lungs. In these cases the osmotic pressure of a blood plasma decreases and the hyponatremia develops that is connected with the changed, inadequate allocation of ADG. At the same time reduction of osmotic pressure of a blood plasma cannot stop allocation of ADG a hypothalamus or other sources of this hormone. Water intoxication of an organism is caused.
Development E. it can be caused also by a hyperthermia (see the Giperter-michesky syndrome). At the same time dysfunction of a hypothalamus with the subsequent metabolic changes is noted.
The separate group is made distsirkulyatorny (vascular) E., caused hron. disturbance of blood supply of a brain at diffusion atherosclerosis — arteriosclerotic E., at an idiopathic hypertensia — hypertensive E., at disturbances of venous outflow from a brain — venous E. (see Atherosclerosis, the Idiopathic hypertensia).
As a result of the hypoxia caused by frustration of lung ventilation, blood circulation, fabric exchange, napr at long decrease in the ABP during an operative measure on heart and large vessels, can arise so-called anoxic, or hypoxemic, AA. (see the Hypoxia). Example anoxic E. is resuscitation caused E. In this case character and extent of damage of a brain are defined by duration of the period of clinical death.
Nek-ry researchers carry to group E. the diseases caused by slow viruses, napr, Kreyttsfeldt's disease — Jacoba, the Kura (see. Slow viral infections), to-rye, however, are nosologically independent with the identified activator.
At the heart of development E. most often lie: a hypoxia
of a brain, deterioration in its blood supply, a venous plethora, increase in permeability of vessels of a brain, it is frequent — microhemorrhages. In some cases changes of different types of a metabolism come to light (especially considerable — proteins and lipids), accumulation of products of their disintegration is noted that toxic affects a brain and aggravates disturbances of metabolism.
Presence of the general (along with specific) pathogenetic mechanisms E. causes emergence at all forms E. similar morfol. changes, but expressed in different degree. The diffusion atrophy of tissue of brain, an illegibility of borders between gray and white matter, wet brain, separate dot hemorrhages in substance of a brain, deepening of brain furrows, increase in volume of ventricles, subarachnoid space and its tanks, puffiness and a plethora of a meninx concern to them.
At microscopic examination in covers of a brain against the background of their sharp plethora the phenomena of a staz in small veins are noted. Vessels of gray and white matter of a brain are expanded, overflowed with blood, in them signs of a staz come to light, small hemorrhages are sometimes visible. Are considerably expanded also perivascular spaces. Reduction of number of neurons, degenerative changes in them with the phenomena of the central tigroliz (a chromatolysis — reduction or disappearance of basphilic substance of cytoplasm of neurons), swellings is characteristic.
Quite often swelling and fragmentation of myelin covers of nerve fibrils comes to light. In various sites of a brain the centers of demyelination are found. In white matter the small centers of a necrosis surrounded with proliferating glial cells come to light. Thus, microscopic changes are defined in all links of system a glia — a capillary — neuron.
ENTSEFALOTRIGEMINALNY the ANGIOMATOSIS 259
in the presence of similar patol. changes in a brain to each form E. idiosyncrasies are inherent: preferential localization of defeat, expressiveness of dystrophic processes in neurons, reactions of glial cells, to microcircus of la rny changes.
Clinically at E. depending on its stage come to light various nevrol. symptoms and symptoms of a disease or intoxication, against the background of to-rykh E develops. From the general manifestations most changes of the highest mental functions early are found: intellectual working capacity decreases, transition from one occupation to another is at a loss, memory, especially on recent events decreases, sleeplessness, slackness and block of patients with day are noted. Complaints to headaches, noise in the head, the general weakness, unstable mood are frequent. In nevrol. the status weakening of reaction of pupils to light, and also accommodation with convergence, asymmetry of a front innervation, sometimes — a nystagmus, decrease in visual acuity and hearing, pseudobulbar symptoms comes to light. At many patients change of a muscle tone is noted, increase is more often than it on extrapyramidal type, separate pyramidal symptoms, a lack of coordination, various vegetative frustration. At dynamic overseeing by patients these disturbances remain rather stable or, in process of progressing of a basic disease, increase. In such cases in later stages of a disease come to light accurately outlined nevrol. syndromes: parkinsonicheskiya
(see Parkinsonism), pseudobulbar (see Psevdobuljbarny paralysis), dementia (see Weak-mindedness). At a part of patients epileptic seizures (see Epilepsy), mental disorders are observed.
In a wedge, practice along with E., developing gradually, with podostry or chronic expansion the wedge, pictures, are observed acute E. with crushing generalized damage of a brain, considerable disturbances of microcirculation, hypostasis and swelling of substance of a brain. The acute current can be had hypertensive E., resulting from heavy cerebral crisis, uraemic and hepatic E., pancreatic E., and also group of tiaminzavisimy damages of a brain. In these cases in a wedge, a picture signs of wet brain prevail, coma, generalized convulsive reactions quickly develops. Current of these forms E. usually extremely heavy.
Additional information on features E. it can be received at an electroencephalography (see): disorganization of the main rhythms, urezheniye or absence and - a rhythm, existence patol comes to light. fluctuations in 0-and A-range, signs of epileptic activity; change of biopotentials of a brain at photostimulation and acoustic stimulation. The analysis of EEG finds decrease in total bioelectric activity of a brain. The computer tomography of a brain (see the Tomography computer) establishes its diffusion atrophy, deepening of brain furrows, expansion of cerebral cavities and subarachnoid spaces; these changes on a surface of hemicerebrums, in a cerebellum are especially considerable. Laboratory researches allow to reveal disturbances of biochemical indicators of blood, cerebrospinal liquid, urine and fabrics, in some cases — existence of toxic products. These data promote specification of an origin and pathogeny E. Features of a functional condition of a brain can be specified by means of such methods of a research as nuclear magnetic resonance (see) and positron emission tomography.
For diagnosis E. and definitions of its form matter data of the anamnesis: instructions on intoxication, a craniocereberal injury, the hereditary and acquired diseases of metabolism (a diabetes mellitus, disturbances of exchange of amino acids, lipids, mucopolysaccharides, etc.), the expressed atherosclerosis, a hypertension, impact of ionizing radiation, perinatal pathology.
Treatment is complex, directed to the basic disease which caused development E. At sharply developed E. use various life support systems — dialysis, artificial ventilation of the lungs, long parenteral food, hemoperfusion; hold also the events directed to cerebral decompression, elimination of a convulsive syndrome. At E. appoint the drugs improving metabolism of a brain: drugs of a nootropic row (Encephabolum, nootropil, piracetam), amino acids (Cerebrolysinum, methionine, glutaminic to - that), lipotropic connections (Essentiale, lipocerebrinum, lecithin), vitamins of group B, A, E, ascorbic and folic to - that, according to indications — vasoprotectives (Prodectinum, Stugeronum, Cavintonum, komplamin), biostimulators (an aloe, a vitreous). Medicamentous means apply is combined, using drugs, various on the nature of action, courses 1 — 3 month.
The forecast is defined by a basic disease, character and expressiveness nevrol. symptoms, possibility of use of specific therapy; in some cases it is possible to provide only stabilization of a condition of patients.
Bibliogra in c y A. P N. Clumsy encephalopathy and clumsy encephalitis, Neuropath. and psikhiat., t. And, century 5, page 41, 1942; Kalinin L. V. and Gusev E. I. Hereditary diseases of metabolism and a fakomatoza with defeat of a nervous system, M., 1981; Treatment of nervous diseases, under the editorship of V. K. Viderkholt, the lane with English, M., 1984; Martynov Yu. S. Is also small E. V is new. Changes of a nervous system and disease of a liver, M., 1980; Nikiforov A. S., etc. Encephalopathy at the patients with a terminal renal failure who are on treatment by a regular hemodialysis Rubbed. arkh., t. 49, No. 7, page 67, 1977; Pathogeny and clinic of alcoholic diseases, under the editorship of I. I. Lukomsky, page 261, M., 1970; Vascular diseases of a nervous system, under the editorship of E. V. Schmidt, M., 1975; Gifford R. W. a. Westbrook E. Hypertensive encephalopathy, mechanisms, clinical features and treatment, Progr. cardiovasc. Dis., v. 17, p. 115, 1974; Gut-gemann A. u. a. Porto-kavale Anastomose und sogenannte Enzephalopathie, Dtsch. med. Wschr., S. 2370, 1961.
E. I. Gusev.