HOUSE-KEEPER LETHARGIC ENCEPHALITIS (Page of Economo, Austrian neuropathologist, 1876 — 1931; Greek lethe oblivion + argia inaction; encephalitis; synonym: epidemic lethargic encephalitis, Ekonomo's encephalitis, encephalitis epidemic, encephalitis lethargic, encephalitis sleepy) — an infectious disease not clear (possibly, virus) etiologies, characterized in an acute stage by fever, the catarral phenomena, drowsiness, oculomotor disturbances, and in chronic — defeat of various departments of the central nervous system with development of parkinsonism and change of the personality.
The first descriptions of a disease similar on a wedge, a picture with E. l. aa., are made in 1673 — 1675 T. Sydenham who reported about cases of long drowsiness and nonsense against the background of repeated temperature increase (febris comatosa). In the subsequent sporadic cases and small outbreaks of diseases, similar E. l. aa., were repeatedly described under various names. In 20 century the first cases were registered during World War I in 1915 in the French army near Verdun, in 1916 — 1917 flashes were noted on other sites of the French front and in a number of the countries of Europe. In 1917 K. Ekonomo described this disease as an independent chesky form of a nosology. In the next years the disease widely extended, having occupied almost all countries and continents. There are no exact data on quantity hurting around the world, but the maximum of incidence fell on 1918 — 1926. Since 1927 sporadic cases of a disease prevail.
Etiology to a crust. time is not determined. Distributional pattern of a disease, its wedge, manifestations and features morfol. changes are typical for primary virus defeats of c. N of page. The assumptions existing originally about etiol. roles of an influenza virus and a virus of a herpes simplex of confirmation did not receive. Thanks to achievements of virology the etiology inf is established. diseases, to-rye belonged to E earlier. l. aa. (vernal and mosquito encephalitis; the diseases caused by various enteroviruses, etc.).
Ways of distribution E. l. aa. are not found out. An estimated way of distribution — airborne. Obviously, the main source of infection are patients and virus carriers (contact infection is established no more than in 4 — 6% of cases). The maximum of incidence falls on the winter period though sporadic cases are observed at all seasons of the year. Generally persons at the age of 20 — 40 years, men and women equally get sick. Residents of the cities are ill 5 — 6 times more often. Because the disease, as a rule, proceeds in the form of an easy respiratory syndrome, and symptoms of encephalitis develop rather seldom, the opinion was created that epidemiol. communication between these displays of a disease is absent.
Pathogeny it is not studied. Initially hematogenous and lymphogenous spread of a virus is supposed, to-ry further perinevralno reaches c. N of page. The chronic stage of a disease is probably caused by long finding of a virus in formations of a nervous system, and also autoimmune processes.
Macroscopic changes of a brain are observed changeably, preferential in the form of a hyperemia and sometimes small hemorrhages. The vascular and infiltrative process which is localized generally on average a brain, a thalamus and a hypothalamus is characteristic of an acute stage of a disease. At E. l. aa. most often black substance of a mesencephalon, the central gray matter of a tire of a mesencephalon surrounding silviyev a water supply system are surprised (a water supply system of a brain, T.) and including kernels of third cranial nerves. Less often changes come to light in gray matter of a myelencephalon and varoliyev of the bridge, and also in kernels of a cerebellum. The inflammatory centers and in gray matter of a spinal cord can be observed. Changes of nervous cells are expressed unsharply (except for black substance of a mesencephalon); in affected areas of a brain a large number of cells with normal structure remains. Dystrophic changes of cells of a brain are expressed more weakly, than at other primary viral encephalitis. Perivascular lymphoid infiltrates come to light, in to-rykh plasmocytes and polymorphonuclear leukocytes quite often are found. In cases hron. courses of a disease are also defined considerable changes in black substance of a mesencephalon, kernels of a diencephalon, including a hypothalamus, to a lesser extent in a pale sphere (pallidum) of a striate body and in a cerebral cortex. Along with old also the fresh inflammatory centers meet, is more often in the field of black substance of a mesencephalon.
The clinical picture
averages the Incubation interval 10 days with fluctuations from 4 to 20 days. The wedge is characteristic polymorphism. manifestations, noted still To. The house-keeper and other researchers both during separate flash, and at epidemics in the different countries, and also at sporadic cases. Allocate an acute and chronic stage of a disease.
The disease in most cases begins subacutely. Standard or subfebrile temperature, headaches, an indisposition, the catarral phenomena are noted. The acute beginning of a disease from rise in temperature to 38 ° is less often observed above, the wrong or remittiruyushchy character has edges. High temperature keeps up to 14 days and more. Drowsiness is observed more than at a half of patients. By the ABP it is more often lowered, in early stages of a disease pulse is slowed down. In an acute stage the symptom of Zilberlast — Zand (a symptom of trembling a century — trembling a century is quite often observed at approach of a finger of the patient investigating to eyes), the pristupoobrazny or continuous increase of breath which is combined with tachycardia. An asthma often is followed by psychomotor excitement. In some cases there is short-term apnoea. Violent avtomatizm like cough, sneezing, puffing, yawning, spitting, sometimes a hiccups are noted. In the period of epidemic E. l. aa. at patients different polymorphic rashes were observed.
In an acute stage sometimes there is a yellowness of skin, the liver increases. Hard cases can be complicated by damage of kidneys. Vegetative disturbances — increase in sweating out of the feverish period, vasculomotor disturbances, the sialosis sometimes raised are characteristic. Perhaps bystry weight loss or increase in body weight.
Signs of focal defeat of a nervous system at E. l. aa. are very various as on degree of manifestation, and but prevalence. The most typically so-called kernel of a disease distinguishing E. l. aa. from other neuroinfections — the okuloletargichesky syndrome (an okulotsefalichesky form), to-ry sometimes already in the acute period is combined with an amyostatic syndrome (see. Amyostatic symptom complex ), and a progreduated current frequent further with the phenomena parkinsonism (see). Are characteristic of an okuloletargichesky syndrome in the acute period the increased drowsiness which is often reaching degree lethargy (see) or, more rare, perversion of a dream (night wakefulness and day dream). Also damage of muscles of eyeglobes is noted, it is frequent — the III couples of cranial nerves (third cranial nerve): appear diplopia (see), dispersing squint (see), ptosis (see), mydriasis (see), ophthalmoplegia (see), disturbances convergences of eyes (see). Are frequent nystagmus (see), decrease or loss of pupillary tests (see. Pupillary reflexes ). These disturbances are often combined with signs of defeat of other cranial nerves — facial nerve (see), trifacial (see), hypoglossal nerve (see). Emergence of hyperkinesias of type is possible myoclonias (see), choreiform twitchings, tics (see), hiccups (see). Are quite often noted paresthesias (see), neuralgia (see), an acoustical and visual hyperesthesia, emergence of the symptoms reminding Menyer's disease — a hearing disorder, a sonitus, pristupoobrazny dizziness is possible (see. Menyera disease ). Also greasiness of skin, the raised sialosis, a glucosuria, a polyuria are characteristic. Pyramidal symptoms meet seldom (in particular, patol. Babinski's reflex).
Also grippopodobny forms of a disease proceeding in the form of the isolated all-infectious syndrome without the expressed pathology of a nervous system are described: vestibular — with the leading symptom complex in the form of a vestibular syndrome; thecosis in the form of a so-called epidemic hiccups when the hiccups is the only symptom of a disease. Also so-called peripheral form of a disease proceeding in the form of a polyradiculoneuritis is described (see. Radiculitis ), is more rare with the isolated defeats, generally facial nerve.
In some cases E. l. aa. begins with mental disturbances, to-rye can be the only symptom of a disease. In a prodromal stage there is an adynamy (see. Asthenic syndrome ) with feeling of weakness, weakness, hypersensitivity to sounds, light, headaches. The most characteristic mental disorder in an acute stage is frustration of a dream (patol. drowsiness or persistent sleeplessness). Often observed form of mental disorders in an acute stage is the delirium (see. Delirious syndrome ). In some cases it develops even before emergence characteristic of encephalitis nevrol. symptoms (see below) can be also followed by verbal and tactile hallucinations (see. Hallucinations ), horeopodobny and myoclonic twitchings or spasms. Emergence delirium acutum (see) always confirms extreme weight of a state and possibility of bystry death. In one cases after a delirium as an intermediate syndrome (see. Symptomatic psychoses ), in others — from the very beginning of a disease there is a sharp slackness, slowness of all mental functions, indifference, detachment from surrounding, i.e. the picture close to an apathetic stupor (see. Struporous states ). In an interval between an acute and chronic stage at middle-aged persons is also more senior fatigue, weakness, slowness and decrease in mental activity, a bad dream, irritability, in depression - dysphoric mood — a postencephalitic adynamy is noted (see. Dysphoria ). At children and teenagers after an acute stage motive and speech disinhibition, frustration of inclinations (obych but at teenagers), first of all — sexual is quite often observed.
In most cases signs of defeat of a nervous system within several weeks gradually regress. Palindromias are in some cases possible. Also rather resistant residual phenomena in the form of paresis of separate muscles of eyeglobes, insufficiency of convergence, sharp weight loss or sharp obesity on adiposogenital type can be observed (see. Adiposogenital dystrophy ), early puberty (see). The long adynamy, fatigue, headaches, sleeplessness is characteristic. Mental disorders as the residual phenomena are more often observed at children and teenagers.
Hron. stage E. l. aa. it is characterized by a syndrome of parkinsonism and neuroendocrinal frustration. Development of the progressing syndrome parkinsonism (see) both in the acute period, and in different terms after postponed E. l. aa., is not a complication, but a further course of a disease that is caused, obviously, a persistent viral infection with preferential defeat of pallidarny system (a pale sphere, a striate body). In sporadic cases E. l. aa. sometimes there is no expressed acute stage, and the disease is shown only by a syndrome of the progressing parkinsonism.
In a chronic stage in the presence of parkinsonism mental disturbances are characterized most often by decrease in level of motives that is shown by lack of initiative, indifference, indifference, depletion and slowness of thinking, attention, the speech — a bradyphrenia or a bradypsychia (see. Speech ). At children and teenagers in hron. stages prevail characterologic disturbances: mental infantility (see), psychopatholike frustration (sexual disinhibition, vagrancy, theft, alcoholism). Excessive mobility, importunity, falsity, tendency to unmotivated aggressive acts is characteristic. In the subsequent there can be symptoms of parkinsonism which are followed by a bradyphrenia.
Among paroxysmal frustration «oculogyric spasms» are most often observed (see. Look paralysis, spasm ), accompanied with fear, attacks of violent assignment of eyeglobes, as a rule, up, with their fixing in this situation within several seconds or minutes. Paroxysmal frustration can be shown by also sudden shout (klozomaniye), a polylogia — repeated repetition of the same word or phrase, attacks of unmotivated motive or speech excitement, in time to-rykh patients run to exhaustion, suddenly make aggressive acts, talk without a stop then again constraint appears. In the form of paroxysms there can be persistence, e.g. an intellectual chewing gum, persuasive inclinations (see. Persuasive states ) and short-term conditions of oneiric stupefaction (see. Oneiric syndrome ). At unsharply expressed parkinsonism paroksizmalno arise psychosensorial frustration (see), frustration of the scheme bodies (see), derealization (see), at a cut surrounding is perceived by the dead who stiffened, others, time — stopped, depersonalization (see) with feeling of an izmenennost mental and physical «I». The listed frustration are followed often by dizziness, instability at the closed eyes, a nystagmus, an okulostatichesky phenomenon — instability and even falling at test on convergence; they arise usually in the evening, during the backfilling, more rare in the afternoon.
Endoformny states are usually observed at unsharply expressed symptoms of parkinsonism. Crazy psychoses are heterogeneous. In one cases initially developing crazy ideas of prosecution, the relation, poisoning prevail, in others — similar contents the nonsense is residual, appearing after disappearance of a delirium or oneiroid (see. Nonsense ). Long hallucinatory-dilision states can meet (see. Paranoid syndrome ). From gallyutsinoz (see. Hallucinations ) arise tactile, in some cases more often followed by painful pain.
the Diagnosis in typical cases during epidemic flash does not present considerable difficulties. Sporadic cases, even proceeding with typical a wedge, a picture, always raise doubts in the relation of accessory to E. l. aa.
At E. l. aa. in blood decrease in amount of hemoglobin and reduction of number of erythrocytes is noted, the moderate neutrocytosis which is replaced then a relative lymphocytosis, ROE it is often accelerated, in later stages — it is slowed down. Changes in cerebrospinal liquid are observed not constantly: pressure it more often normal, is occasionally increased; in the acute period the insignificant lymphocytic pleocytosis which is seldom exceeding 40 cells in 1 mkl sometimes comes to light (but about 100 cells and more can reach). It accrues during 2 weeks and then is quickly normalized. Protein content (to 0,66 — 1,65 g/l) and glucose is sometimes slightly increased (to 50 — 100 mg / 100 ml).
Differential diagnosis carry out with tumors of a nervous system (see. Brain , tumors), intoxications (see. Intoxication ), poisonings (see) and other neuroinfections. AA. l. aa. in an acute stage it is necessary to differentiate with acute symptomatic psychoses of other etiology (see. Symptomatic psychoses ), in a chronic stage with psychopathies (see), schizophrenia (see). For this purpose the wedge, inspections, and also virologic and immunological researches use data (see. Virologic researches , Immunology ).
Specific treatment E. l. aa. does not exist. The complex to lay down. actions includes dehydrational therapy, use of glucocorticoids, and in later periods — and anabolic hormones, use of a wide complex of symptomatic means. At emergence of symptoms of parkinsonism both in the early period of a disease, and in the remote terms, apply the means compensating insufficiency of a dopamine (a levodopa, Midantanum), and also the drugs possessing anticholinergic action (Cyclodolum, Tropacinum, Norakinum, etc.). In the absence of effect of medicamentous therapy sometimes make stereotaxic destruction of a ventrolateralny kernel of a thalamus (see. Stereotaxic neurosurgery ). It is also necessary to maintain sufficient physical activity of the patient, including by means of LFK.
the Forecast in an acute stage in most cases rather favorable. At development of a syndrome of parkinsonism motive disturbances can result in disability. By means of the corresponding therapy the patient can keep working capacity for considerable term, but completely it is not possible to stop development of postencephalitic parkinsonism.
Prevention it is not developed.
Bibliography: Crawl M. B., Margulies M. S. and Propper-Grashchenkov N. I. The textbook of nervous diseases, page 271, M. — L., 1939; Margulies M. S. Acute encephalitis epidemic and sporadic, M. — Pg., 1923; The Multivolume guide to neurology, under the editorship of S. N. Davidenkon, t. 3, book 1, and. 386, M., 1962; V. M. K Frosts to a question of crazy options of epidemic encephalitis. Works psikhiat. clinics, century 5, page 163, M., 1934; Ekonomo C. Die Encephalitis lethargica, Lpz. — Wien, 1918; Ey H., Bernard P. et Brisset Ch. Manuel de psychiatrie, P., 1970; Handbuch der Neurologie, hrsg. v. O. Bumke u. Lake of Foerster, V., 1935 — 1936; Ravenholt R. T. Foege W. H. 1918 Influenza, encephalitis lethargica, parkinsonism, Lancet, v. 2, p. 860, 1982; Stern F. Die epidemische Encephalitis» B., 1928.
K. G. Umansky; H. G. Shumsky (psikhiat.).