OLIGOPHRENIAS (oligophrenia, singular; grech, oligos small + phren mind; synonym: inborn weak-mindedness, mental retardation) — the group various on an etiology, a pathogeny and clinical manifestations of not progreduated morbid conditions, the general sign to-rykh is existence inborn or acquired in the early childhood (up to 3 years) the general underdevelopment of mentality with dominance of intellectual insufficiency.
One of the first Dufour in 1770 described inborn weak-mindedness with allocation of its versions on degree of depth (an idiocy and a deficiency of intellect). At the beginning of 19 century. Zh. Eskirol separated inborn weak-mindedness (idiocy) from acquired (dementias), and also entered division of inborn weak-mindedness into three options according to degree of manifestation of disturbances of the speech into practice. In the 2nd half of 19 century V. Manyan described easy degree of inborn weak-mindedness — the moron a nost. The term «oligophrenias» for designation of all group of the inborn and acquired in the early childhood weak-mindedness was offered by E. Krepelin.
The indicator of prevalence of O., according to WHO materials (1968), based on generalization of these many foreign researches, fluctuates within 1 — 3% of the population. However these data need amendments taking into account that in many countries of the West carry conditions of weak-mindedness at the progressing psychological diseases which arose at early children's age to ukhmstvenny backwardness. It is also necessary to mean that the majority epidekhmiol. researches had selective character. In the second half of 20 century the tendency to increase in quantity mentally retarded is around the world noted that it is generally connected with increase in the general life expectancy, decline in mortality of children with anomalies of development of c. N of page thanks to achievements of medicine. Considerably O.'s identification aged from 7 up to 19 years increases that the hl speaks. obr. influence of social factors (the beginning school and the prof. of training, survey at a conscription). Occur among O. men approximately by 1,5 times more often than among women. The ratio of persons with different degree of mental retardation (moronity, a deficiency of intellect, an idiocy), according to a number of researchers, makes respectively 75, 20 and 5%.
The clinical picture
At all variety a wedge, manifestations by the general for various forms O. is a total underdevelopment of mentality, including an underdevelopment of cognitive abilities and persons in general. The leading role in structure of mental defect belongs to insufficiency of the highest forms of cognitive activity, first of all abstract thinking, at rather sufficient level of development of evolutionarily more ancient components of the personality, such as the requirements connected with instincts, the lowest affectivity. However the specified features of mental defect at O. are shown in an ontogeny not at once; they become clearer by the end preschool — to the beginning of school age of the child. At early children's and preschool age insufficiency of cognitive activity is shown preferential in an underdevelopment of mental functions, to-rye treat the initial stage of development of abstract thinking: lag in terms of development of mental activity and motor activity; distortion and slow rate of formation of visual and acoustical conditioned reflexes; insufficiency of emotsionalnovolevy reactions; delay of terms of emergence of emotional reactions on surrounding on the first year of life; lag in development of the speech, imitative «manipulative» character of a game; long absence of skills of self-service; poorly expressed cognitive interest to surrounding; not differentiation or lack of the highest emotions (sympathy, feeling of attachment, etc.) at early children's and preschool age. At children of school age on the foreground the specific and situational nature of thinking, weakness or impossibility of generalization, inability of allocation * essential signs of objects and the phenomena act more clearly. At deep mental retardation assimilation of any abstract knowledge and school training can be impossible in general.
Teenagers and adult persons with O. along with insufficiency of abstract thinking have more noticeable an immaturity of the personality (the increased suggestibility, a nekritichiost, inability to make the independent decision in difficult everyday situations, the expressed dependence of behavior on an external situation, impulsiveness of acts).
Intellectual insufficiency at O. in a varying degree affects all mental processes, first of all cognitive that is expressed in insufficiency of perception, disturbances of active attention, delay and fragility of storing, low level of logical memory. The underdevelopment and weak differentiation of intellectual and moral emotions, inadequacy of affects to the endured events, weakness of an initiative and motives, lack of fight of motives, insufficient focus of action are characteristic. The speech at O. differs in the poverty of a vocabulary, abundance of stamps which are not developed by phrases frequent in agrammatisms and defects of a pronunciation. Signs of an underdevelopment are available also in mental activity, and in motor activity (delay in development of static and kinetic functions, insufficient focus and co-ordination of movements, their angularity, insufficiency of thin manual motility, a mimicry and pantomimika).
At O. frustration — disturbances of a skull-but-brain innervation and functions of pyramidal system (paresis, disturbances of a muscle tone), diencephalic frustration, in particular tserebroendokrinny insufficiency are often observed various nonspecific nevrol. In the somatic status at O. connected with disturbances of pre-natal development various anomalies of development and a dysplasia in the form of deformations and changes of the sizes of a skull, anomalies of a structure and arrangement of auricles, eyes, jaws, teeth, shortenings of phalanxes of fingers, dizrafichesky symptoms (a labium leporium, splitting of the sky, spina bifida, an union of fingers — a syndactylia, etc.), malformations of internals — hearts, easy, urinogenital bodies are noted, as a rule, and also went. - kish. path. Quite often there are lag in physical development, disturbances of proportions of a body, a rachiocampsis, an underdevelopment of generative organs.
The loudspeaker O. carries the neprogrediyent-ny, so-called evolutive character connected with age maturing of c. N of page, and also with processes of a reparation and compensation. Expression of positive dynamics of O. is gradual, slower, than at healthy children, increase in level of mental capacities, strengthening is mobility of mental processes, improvement of the phrase speech, emergence of more correct self-assessment and critical relation to surrounding, reduction of motor insufficiency, replenishment of a stock of knowledge, acquisition of household data, simple labor and the prof. of skills. Such dynamics is inherent generally to persons with uncomplicated O. in degree of moronity. Due to more or less expressed positive evolutive dynamics and social adaptation, according to D. E. Melekhova (1970), 77% of persons with O. in degree of moronity become systematically able-bodied, and part of them completely adapts socially and does not need observation of the psychiatrist. Favorable evolutive dynamics of O. depends on depth of a mental underdevelopment, a kliniko-pathogenetic form O., additional pathogenic influences, and also timeliness and completeness of medical and rehabilitation actions. Certain distinctions in dynamics are available at mentally retarded with different temperament — eretich-ny (with strong and more mobile temperament) and torpid (with weaker and inert temperament). So, eretichny oligophrenic persons have a rate of mental development higher, household skills are fixed easier.
Evolutive dynamics of mental development at O. can be broken by conditions of a decompensation, to-rye most often arise during the transition age periods at complicated by the Lake. At the same time the main a wedge, manifestations are tserebrastenichesky and psychopatholike states (counterinhibition of inclinations, especially sexual, tendency to goings and vagrancy, aggression, impulsiveness). Much less often the decompensation is expressed in the form of incidental and recurrent psychoses preferential at pubertal age. Most of domestic and foreign researchers regard these psychoses as specific to sick O. in this connection they are designated as «psychoses of oligophrenic persons», «psychoses at moronity». Their wedge, a picture differs from a wedge, pictures of the endogenous and exogenous psychoses which are also arising at the Lake. «Psychoses of oligophrenic persons» are generally shown by affective frustration (conditions of a dysphoria and alarming depression with fears), katatonopodobny conditions of motive excitement or a stupor, and also episodes of the rudimentary, not developed, hallucinatory and crazy frustration on an alarming and depressive affective background. Also short-term episodes are frequent devocalizations (see) and twilight stupefaction (see). Psychotic states lasting from 1 to 6 weeks have character of single or recurrent attacks. Both during attacks, and in intervals between them tsereb-rastenichesky symptoms and strengthening of vegetative frustration are usually observed. Incidental psychoses at O., as a rule, come to an end with unset. After several attacks of recurrent psychosis deepening of changes of the personality on organic type is possible. In a pathogeny of conditions of a decompensation, including their psychotic forms, strengthening of residual and organic cerebral insufficiency with emergence of liquorodynamic and cerebrovascular frustration is important. Besides, the additional pathogenetic role of en-dokrinno-humoral shifts of the pubertal period, especially disgarmonichesk proceeding is supposed. Various exogenous factors — injuries, infections, overloads connected with school training — promote emergence of conditions of a decompensation.
Classification and the short characteristic of oligophrenias at various morbid conditions
the Most widespread classification of O. is classification by degree of intellectual defect, important practical value for the solution of questions of social adaptation, in particular for definition of an opportunity and type of training mentally retarded has edges. Usually allocate three degrees of mental retardation: moronity, deficiency of intellect and idiocy. For definition of degree of mental retardation use intellectual coefficient (IQ), to-ry is quantitative assessment of intelligence and is defined on the basis of performance of standard psychological tests.
Moronity — easy degree of mental retardation (IQ is equal to 50 — 70), is characterized by the broad range of intellectual insufficiency, sufficient development of the everyday speech, ability to assimilation of the special programs based on specific visual instruction, a cut also the prof. by skills, relative adequacy and independence of behavior in a usual situation is carried out at slower speed, and also ability to mastering simple labor. In comparison with other degrees of O. at moronity of line of the personality and character differ bigger differentsirovannostyo and identity; thanks to rather higher mental development morons in many cases well adapt in usual conditions of life. Due to the broad range of intellectual insufficiency at moronity quite often in the practical purposes allocate the it expressed, average and easy degrees.
Deficiency of intellect — average degree of mental retardation (IQ is equal to 20 — 49), differs in the expressed concreteness of thinking, its situational character, the inability to formation of abstract concepts which is insufficiently developed by the speech with agrammatisms and tongue-tie, the general motor insufficiency. Imbeciles cannot study according to the program of auxiliary schools for mentally retarded. At the same time skills of self-service are available to them, and it is frequent also elementary labor skills. Rate of mental development at a deficiency of intellect is slowed sharply down. In the International classification of diseases the deficiency of intellect is subdivided into the mental retardation of average degree and sharp mental retardation differing in poor development of the speech and a possibility of acquisition only of skills of self-service.
Idiocy (deep mental retardation on terminology of the International classification of diseases) — the deepest degree of a mental underdevelopment (IQ less than 20), at a cut the thinking and the speech are almost not developed, perceptions are poorly differentiated, reaction to surrounding is sharply reduced and often inadequate, emotions are limited to feelings of pleasure and discontent, any intelligent activity, including skills of self-service, are unavailable, patients are often slovenly. Static and locomotory functions are roughly underdeveloped in this connection many patients are not able to stand and go independently. The behavior in one cases differs in slackness, low-mobility, and in others — tendency to uniform motive excitement with the stereotypic movements (rocking of a trunk, waves of hands, a clap in palms), and nek-ry patients with periodic manifestations have aggressions and autoaggressions (can suddenly strike, bite any of people around, scratch and strike itself blows). Increase and perversion of inclinations is often observed — patients persistently frig, eat sewage. In most cases the expressed nevrol, disturbances and somatic anomalies take place. Life of such patients passes at the instinctive level. They need permanent care and supervision.
Criteria of learnability, a possibility of social adaptation mentally retarded are put in a basis of classification of oligophrenias of D.E. Melekhov (1970). Proceeding from kliniko-psikhopa-tologicheskoy structures of defect and opportunities of social adaptation, it allocates O. with a primary type of defect and O., at to - ry the mental underdevelopment is combined with emotional and strong-willed disturbances, a tserebra-sthenic syndrome and various nevrol, disturbances.
A number of classifications of O. based on the principle of klinikopatogenetichesky correlation is developed. So, M. S. Pevzner (1966) allocates uncomplicated O. without the expressed disturbances of the emotional and strong-willed sphere and without rough losses of functions of analyzers; The lake, complicated by hydrocephaly; The Lake, combined with a local hearing disorder, speeches, space synthesis, motive systems; The Lake, combined with an underdevelopment of perednelobny departments of a brain; The Lake, combined with defeat of subcrustal structures of a brain. S. S. Mukhin (1961) allocated, proceeding from a condition of «a physiological tone» of a brain, sthenic, asthenic and atonic forms O. Many researchers subdivide O. on primary (hereditarily caused) and secondary (exogenous).
In the classification offered by Jervis (G. A. Jervis, 1959), one of the most detailed etiol, classifications of O. including more than 40 separate forms all O. share on physiological (caused by «sociocultural» factors) and pathological, to-rye also exogenous forms are subdivided on endogenous (hereditarily caused). The lack of many foreign classifications of O. should be considered inclusion of dementias in them (see. Weak-mindedness ), the progreduated hereditary and degenerative diseases which began at early children's age as a result of a row (a tuberous sclerosis, a neurofibromatosis, a disease of Sterdzha — Weber), schizophrenia and epilepsies.
Two criteria — degree of intellectual insufficiency and etiol, a factor are the basis for a systematics of O. in the International classification of diseases.
One of the most widespread in the USSR is O.'s classification offered G. E. Sukhareva (1965, 1972), edges relies on the following criteria: time of influence etiol, factor, etiology and features wedge, pictures. Proceeding from the first criterion O. of the endogenous nature are allocated (in connection with defeat of sex cells of parents); The lakes caused embrio-by both fetopathies, and O., arising in connection with the vrednost operating in prenatal and early post-natal the periods. Allocate also atypical forms O. connected with hydrocephaly, an underdevelopment of analyzers, primary endocrine disturbances, napr, a hypothyroidism. Lakes differentiate on features a wedge, pictures, including degree of mental retardation (moronity, a deficiency of intellect, an idiocy).
Proceeding from features of psychopathological structure of defect a number of researchers, in particular G. E. Sukhareva (1965), allocate the simple and complicated forms O. At simple forms O. defect is generally expressed by the phenomena of a mental underdevelopment; at the complicated forms the mental underdevelopment is combined with the additional psychopathological syndromes not specific to O., such as tserebrastenichesky, neurosis-like, psychopatholike, epileptiform, etc., to-rye are connected with effects of early organic lesion of a brain.
Unlike O. with the obscure etiology of a form O. with the established etiology and often with certain somatoneurologic signs received the name of the differentiated Lakes. They can be divided into a number of groups: The lake at chromosomal diseases; hereditary O.; forms O. with the mixed (hereditary and exogenous) etiology; the exogenous caused forms, at to-rykh depending on the period of ontogenesis there is a damage of a brain, subdivided into O. connected with pre-natal exogenous defeats, O. caused by perinatal pathology and O. caused by early post-natal exogenous organic lesion of a brain.
Lakes at chromosomal diseases are connected with disturbances of quantitative structure or structure of chromosomes. With rare exception they do not submit to Mendelian patterns of inheritance (see. Mendel laws ) also are observed only within one generation. Mental retardation in this group O. is, as a rule, combined, with the expressed disturbances of physical development, various displaziya and rough defects of a constitution. Among O. caused by anomalies of autosomes, the most common form is the Down syndrome (see. Down disease ). More rare, but clinically accurately outlined form connected with structural anomaly of the 5th couple of chromosomes in group B is represented by a syndrome of «cat's shout» (see. Le Jeune syndrome ), to-ry a thicket occurs at women and it is characterized by a combination of the expressed mental retardation (more often a deficiency of intellect or an idiocy) with a delay of physical development and a number of dysplastic signs (a «anti-Mongoloid» section of eyes, a hypertelorism, an epikant, a low arrangement of auricles, a cross fold of palms). A pathognomonic symptom is the peculiar mewing timbre of a voice connected with anomaly of a structure of a throat.
The lake with superficial mental retardation, or boundary intellectual insufficiency, is observed in large part cases at anomalies of gonosomes. Treat such O. Shereshevsky's syndrome — Turner, a syndrome of Triplo-X, Klaynfelter's syndrome, a syndrome of XYY and nek-ry others.
Shereshevsky's syndrome — Turner (see. Turner syndrome ), meeting only at women, it is connected with lack of one X-chromosome — a formula of chromosomal complement 45/Х0, sex chromatin (see) in kernels of cells in most cases is absent. Sexual infantility, in particular an underdevelopment of internal generative organs, absence or fibrous regeneration of ovaries, primary amenorrhea, infertility is characteristic of it. Mental retardation (moronity) takes place only at a small part of patients, however in some cases the deficiency of intellect and even an idiocy meets. At most of patients mental infantility is noted. At pubertal and mature age the secondary psychogenic reactions connected with awareness of own inferiority are possible.
The syndrome triplo-H, caused by existence of additional X-chromosome (47/XXX), occurs only at female persons. The cytogenetic research reveals 47 chromosomes and the doubled quantity of a sex chromatin. A wedge, manifestations of a polimorfna, often are found various a dysplasia, a delay of physical development, an amenorrhea. However quite often somatic anomalies and disturbances of sexual differentiation are absent. Superficial mental retardation occurs more often than at Turner's syndrome (approximately at 75% of patients). Besides, note the increased risk of developing of schizophrenia.
Klaynfelter's syndrome (see. Klaynfeltera syndrome ) it is caused by existence of one or several additional X-chromosomes, occurs at men. More often the formula of a karyotype 47/XXY takes place. In kernels of cells the sex chromatin inherent to female type is found. The wedge, a picture is characterized by a hypogenitalism, an underdevelopment of testicles, high growth with eunuchoid proportions, frequent a gynecomastia, an adiposity and pilosis on female type, poor development of men's secondary sexual characteristics, infertility. The intellectual underdevelopment varies from boundary intellectual insufficiency to the expressed moronity. The normal intelligence is in rare instances possible. Experience of inferiority at teenagers and adult patients becomes frequent a source secondary psychogenic, including neurotic, reactions and psychogenic patol, development of the personality.
The syndrome of XYY which is observed at men is connected with an additional Y-chromosome, edges comes to light by detection of Y-chromatin in buccal swabs of a mucous membrane of a mouth by means of luminescent microscopy. A wedge, manifestations are not specific. In the majority of observations at patients high growth is noted (on average 186 cm). Part of them has an eunuchoid constitution and a number of dysplastic signs (the wrong growth of teeth, an abnormal bite, increase in a mandible, a radioulnarny synostosis, etc.). Sexual function is not broken, kept an opportunity to have healthy posterity. Superficial mental retardation (moronity) with uneven structure of defect is quite often noted. The main psychopathological feature of a syndrome is the psychopatholike state which is shown tendency to dysphorias, an affective explosibility, aggression, impulsiveness, counterinhibition of inclinations, to-rye are quite often combined with asocial behavior (aggressive acts, vagrancy, theft, alcoholism). Diagnosis of a syndrome demands a cytogenetic research.
Among the known hereditary forms O. the main place is taken metabolic, or enzymopathic, by O. caused by hereditary diseases of a metabolism with an autosomal and recessive mode of inheritance. According to B. V. Lebedev (1975), apprx. 50 hereditary diseases of a metabolism is followed by disturbance of intellectual development and a condition of weak-mindedness. In view of the progreduated nature of the majority of these diseases the question of legitimacy of reference of conditions of weak-mindedness at them to O. is disputable. However suspension of disease process at a number of hereditary diseases of exchange at early age, the stable nature of the mental defect at them submitting natural-tyam evolutive dynamics, inherent O. allows to carry, according to most of the Soviet psychiatrists, weak-mindedness at such diseases as Fenilketonuriya, a gomo-tsisteinuriya, a galactosemia and nek-ry others, to mental retardation. Among metabolic O. O. caused by disturbances of exchange of amino acids and carbohydrates meet more often. O. is most widespread at fenilketonuriya (see), so-called phenyl-pyruvic O., a cut is the cornerstone anomaly of exchange of amino acid — phenylalanine. The second place on frequency is taken by O. at homocystinurias (see), caused by defect of exchange of methionine. This form phenotypical is similar to Marfan's syndrome. Mental retardation (moronity or a superficial deficiency of intellect) is characteristic of it in large part cases, convulsive attacks, the raised muscle tone are quite often noted; in blood, urine and cerebrospinal liquid the increased content of methionine and homocystine comes to light. O. belongs to hereditary forms at histidinemias (see) — a combination of mental retardation to the general underdevelopment of the speech (alalia), convulsive attacks, an ataxy, the increased maintenance of a histidine (to 10 mg / 100 ml of blood); at an argini-nyantarny aminoaciduria — mental retardation, convulsive attacks, the increased fragility of hair and nails, increase in a liver (see. Aminoatsiduriya arginineamber ); at a tsitrullinuriya — mental retardation, a hyperreflexia, the increased content of citrulline in blood and urine owing to disturbance of synthesis of urea.
O. belong to more studied forms O. connected with inherited disorders of exchange of carbohydrates at galactosemia (see), fruktozuriya (see) and sakharozuriya. These diseases of exchange genetically caused insufficiency of the enzymes providing normal metabolism of carbohydrates — galactoses is the cornerstone, of fructose and sucrose. A wedge, manifestations at the same time are similar: the expressed dispeptic frustration (vomiting, a diarrhea) after meal, jaundice are observed at the birth, a hepatomegalia and cirrhosis, quite often convulsive attacks, slackness, a growth inhibition; in blood and urine the increased content of the corresponding carbohydrates, nek-ry amino acids. At a galactosemia heavier current is noted, it is frequent with a lethal outcome on the first year of life. Mental retardation is characteristic of all three forms, its depth is less at fructose at a riya.
Carry to O. caused by disturbance of exchange of carbohydrates also O. at gargoilizm (see), the basis of a pathogeny to-rogo is made by excess adjournment of glikozaminoglikan in various bodies and fabrics, including a brain. Gargoilizm meets twice more often at men. The progressing nature of weak-mindedness (always deep weak-mindedness) at early age does disputable reference of this form
metabolic O. carry nek-ry forms O. with the obscure type of disturbance of metabolism to O. K, in particular Laurence's syndrome — Muna — Bidlya (see. Laurence — Muna — Bidlya a syndrome ) and progeria (see). — Muna — Bidlya the combination of obesity on adiposagenital type, a hypogenitalism, a degeneration of a retina, a polydactylia (shestipalost), displaziya of a constitution and mental retardation of various degree is characteristic of Laurence's syndrome.
A progeria — the rare hereditary disease which is characterized by symptoms of presenilation. Sexual infantility is usually observed. Intellectual insufficiency is expressed in different degree. Characteristics mentalities inherent to senile age.
The special place among the hereditary differentiated O. belongs to forms, to-rye skin (xerothermic forms), etc. are shown by a combination of mental retardation to anomalies of development of separate systems, in particular connecting fabric, bone system (dizostozichesky forms). The pathogeny of these forms is a little studied. Carry Marfan's syndrome to their number (see. Marfana syndrome ), Kruzon's syndrome (see. Dysostosis ), Aper's syndrome (see. Apera syndrome ), and also a syndrome Ore (see. Keratoza, table ). The combination of inborn weak-mindedness to skin anomalies (an erythema, bubbles), a keratitis, helcomas, is frequent with a convulsive syndrome, paresis takes place at de Sanctis's syndrome — Kakkyona described in 1932.
The exogenous caused O. include the following forms. Rubeolyarny O. is caused by the embryopathy caused by a virus of a clumsy rubella in the first months of pregnancy. The wedge, a picture is characterized by the expressed mental retardation, anomalies of development of eyes (a cataract, mikroftalm, inborn glaucoma), inborn heart diseases (defects of partitions are more often), and also symptoms of an underdevelopment of an inner ear (deafness, relative deafness). At the same time a nanocephalia, anomalies of skeleton structure, urinogenital bodies, and also a hypomyotonia, hyperkinesias, disturbances of swallowing are frequent.
The lake, connected with listeriosis of c. the N of page, is a consequence of a pre-natal encephalomeningitis. The wedge, a picture of a malospetsifichn is also shown by the expressed mental retardation which is combined with a psychoorganic syndrome. The diagnosis is established on the basis epidemiol. the anamnesis, a wedge, pictures, immunol, Paul's reactions — Bunnellya (see. Paul — Bunnellya reaction ) and detection of listeriya in cerebrospinal liquid (see).
The combination of mental retardation (moronity, a deficiency of intellect) to the residual phenomena of a pre-natal encephalomeningitis is characteristic of O. at inborn syphilis — Argayll Robertson syndrome (see), paralyzes, paresis, decrease or lack of tendon jerks, hyperkinesias, convulsive attacks at early children's age, and also various anomalies — deformations of a skull, a saddle nose, the high sky, Getchinson's triad (a parenchymatous keratitis, deafness, defeat of the central upper constant cutters), an underdevelopment of a xiphoidal shoot. For diagnosis, in addition to listed a wedge, signs, positive specific reactions in blood and cerebrospinal liquid, e.g. Wassermann reaction matter, RIBT (reaction of an immobilization of pale treponemas).
The lake, caused by a pre-natal toxoplasmosis, is subdivided into two versions. The first of them is connected with the embryopathy caused by defeat of a germ in the first months of pregnancy. At the same time the expressed mental retardation (a deficiency of intellect or an idiocy) is combined with rough somatic anomalies (a nanocephalia, a microphthalmia, various displaziya of a structure of a skull, anomalies of internals). The second version — result of the encephalomeningitis postponed in the second half of pregnancy; it is characterized by a combination of less deep mental retardation to a chorioretinitis, hydrocephaly, convulsive attacks, hyperkinesias, spastic paralyzes and paresis of extremities, pseudobulbar frustration, disturbances of a craniocereberal innervation, availability of intracerebral calcificats. The diagnosis is established on the basis by a wedge, pictures, a lab. data (positive serol, reactions, intracutaneous test), X-ray analysis of a skull, and also ophthalmologic inspection, epidemiological (contact with animals) and obstetric anamnesis (misbirths and still births).
The lake, caused by a hemolitic disease of newborns, it is connected with immunol, the conflict between the fruit and an organism of mother caused by incompatibility of their blood on a Rhesus factor or factors of AB0. Damage of a brain of the child, in particular bark and basal gangliyev big hemispheres, is a consequence of accumulation in blood of an indirect bilirubin and other toxic decomposition products of erythrocytes (bilirubinovy encephalopathy). The wedge, picture is characterized by a combination of mental retardation of different degree and a psychoorganic syndrome to defects of hearing (in connection with defeat of the acoustic analyzer and the appropriate conduction paths of a brain) and choreoathetoid hyperkinesias. Besides, oculomotor frustration, squint, a subcrustal dysarthtia, and regarding cases — paresis, paralyzes, disturbances of the space analysis and synthesis are noted.
The lake, caused by asphyxia and a mechanical birth trauma, it is connected with damage of a brain of a fruit (newborn) as a result of the cerebral hypoxia caused by disturbances of cerebral circulation. In a wedge, a picture along with intellectual insufficiency of different degree of manifestation (from easy moronity to an idiocy) there are various manifestations of a psychoorganic syndrome: general motive disinhibition, affective lability, explosiveness, tserebrastenichesky symptoms, insufficiency of motives. The arrest of development of static and locomotory functions, speeches, quite often erased phenomena of cerebral palsy, convulsive attacks, disturbances of a cherepnomozgovy innervation are noted. At O. caused by hl. obr. asphyxia, the wedge, a picture differs in dominance of a psychasthenia, is frequent with weakness of motives, slackness; dynamics at the same time is more favorable. At O. caused by a mechanical birth trauma explosiveness, disinhibition of inclinations, more expressed dysmnesias and speeches, focal nevrol, symptoms and convulsive attacks are observed.
The lakes caused by early post-natal infectious, infectious and allergic and traumatic damages of a brain carry, as a rule, to the complicated O., the pathogeny to-rykh includes, on the one hand, anomalies of development not created funkts. systems of a brain, and with another — the phenomena of disturbance or loss of the created functions. Wedge, features depend not so much on an etiology how many from severity, prevalence and preferential localization of organic lesion of a brain. As a rule, there are no anomalies of development and a dysplasia inherent to chromosomal and hereditary forms O. Mental retardation of different degree (from easy moronity to an idiocy) can be combined with various options of a psychoorganic syndrome (tserebrasteniche-sky, neurosis-like, psychopatholike). Disturbances of development of the speech, dyslexia, dysgraphia, various affective frustration (euphoric background of mood, dysphoria, apathy, affective lability), and also disturbances of inclinations are often observed. Manifestations are various nevrol: hydrocephaly, it is frequent with the phenomena of intracranial hypertensia, diencephalic frustration, disturbances of a craniocereberal innervation, relative deafness or deafness, paresis and paralyzes, hyperkinesias, convulsive attacks. The loudspeaker O. it is characterized by tendency to a decompensation during the transition age periods, especially in pubertal.
The lake at hydrocephaly (see) is among atypical. Degree of a mental underdevelopment can fluctuate from moronity to an idiocy. Tendency to reasoning, quite good mechanical memory, an euphoric background of mood are characteristic good development of the speech with a large supply of words and abundance of speech stamps. At nek-ry patients lability of affect, irritability, tendency to fears are noted. During the strengthening of intracranial hypertensia there are a psychasthenia, convulsive attacks, accrues nevrol, the symptomatology, worsens the general mental state.
Etiology and pathogeny
. The etiology in some cases is not found out. Etiol, factors of the differentiated O. are subdivided on endogenous on - investigative and exogenous (exogenous and biological and mikrosotsialno-psychological). Along with preferential endogenous and hereditary it is also exogenous caused by O. there are forms, in an etiology to-rykh hereditary and exogenous factors interact. According to a number of researchers, patients with hereditary forms make no more than 10% of total number of sick O. Protsent of patients with hereditary forms among patients with deep mental retardation considerably increases. Dominant, recessive and polygenic modes of inheritance are established. Cases of superficial mental retardation are more often connected with dominant and polygenic modes of inheritance, a deficiency of intellect and an idiocy — with recessive type.
A specific place among internal causes is held by mutations (see. Mutation ), in an origin to-rykh ionizing radiation, intoxication, and also age of mother matter.
Factors perinatal, acting on an embryo and a fruit in the pre-natal period, and post-natal can be distinguished from exogenous and biological factors. Carry to the first hron, the diseases of mother and heavy toxicoses of pregnancy causing a pre-natal hypoxia; pre-natal infections, napr, clumsy rubella, flu, infectious hepatitis, listeriosis, cytomegaly, Toxoplasmosis, syphilis; the various intoxications caused by alcohol, industrial chemical substances, pharmaceuticals (nek-ry antibiotics, sulfanamide drugs, etc.); asphyxia and mechanical cranial mozgo-vuyu an injury of a fruit at the birth, and also immunol, the conflict on a Rhesus factor and factors of AB0 between an organism of mother and a fruit. Postna-talnymi factors are neuroinfections (meningitis, encephalitis and an encephalomeningitis), a serious dystrophic illness, cherepnomozgovy injuries and in some cases the heavy intoxications transferred to the first 2 years of life of the child.
The question of a role of a mikrosotsialno-psychological factor («a sotsio-cultural deprivation») in O.'s etiology is disputable. Most of the Soviet psychiatrists consider this factor as the important additional external condition promoting formation of the Lake. At the same time this factor can be the reason in emergence of boundary intellectual insufficiency (so-called boundary mental retardation).
O.'s pathogeny is up to the end not clear. Pathogenetic mechanisms of separate forms significantly differ, the general for them is the time factor, or a hronogenny factor, i.e. that period of ontogenesis, in Krom the developing brain is surprised. At defeats in the embryonal period, i.e. at embryopathies (see), rough anomalies of development of a brain, internals and the general dismorfiya are observed. At defeats in the fetalis period, i.e. at fetopathies (see), development of the most late forming structures of a brain, napr is broken, frontal and parietal bark, changes of a brain — necroses, the centers of an inflammation, cicatricial processes appear local patol, to-rye lead to mental defect, development by the atypical and complicated O. Naiboley general pathogenetic factor of perinatal damages of a brain which is the cornerstone of O. the hypoxia is, leads edges to disturbances of myelination and development of capillaries of a brain. Asphyxia and a mechanical birth trauma cause intracerebral bleedings, especially in bark and subcrustal gangliya of big hemispheres.
Metabolic O.' pathogeny is connected with hereditary defect of the enzymes regulating certain exchange processes. The nature of biochemical disturbances at metabolic O.' number, napr, at a fenilketonuriya, a galactosemia, is established, however specific pathogenetic mechanisms of development of intellectual insufficiency remain obscure.
Disturbances of century of N of at O. it is characterized by an underdevelopment of the second alarm system, inertness of the basic nervous processes, weakness of internal inhibition, disturbances of interaction of bark and subcrustal educations. On EEG immaturity of bioelectric potential usually comes to light (absence or insufficiency of an alpha rhythm, disritmiya, low level of reactivity, insufficient differentiation of cortical rhythms on areas).
Pathomorphologic changes in a brain are most expressed at O. caused by embryopathies, for to-rykh the small sizes of a brain, lack of crinkles of brain bark are characteristic (agiriya), depression of crinkles (pakhigiriya), dominance of small, strongly curved crinkles (microgyria), an underdevelopment or sharp expansion of cerebral cavities, internal and outside hydrocephaly which are followed by an atrophy of bark of big hemispheres and flattening of crinkles. At O. caused by chromosomal embryopathies also the underdevelopment of a cerebellum and a brainstem and malformations of other bodies and systems are noted. At O. caused by brain infections and injuries in a brain cicatricial changes, cysts, sites of an atrophy of marrow and other changes are found.
At gistol, a research reveal disturbances of myelination, anomaly of a structure of gray and white matter and an underdevelopment of layers of bark of big hemispheres.
the Diagnosis is established on the basis of the anamnesis, by a wedge, pictures (structures of mental defect, the central place in a cut is taken by an underdevelopment of abstract thinking, lag in mental development at children's and teenage age, lack of a progrediyentnost, in particular signs of deepening of mental defect), patopsikhologichesky (a research of thinking, memory, combinatory activity etc.) and somatoneurologic researches. In diagnosis of the differentiated forms O. methods of a research are important laboratory biol (cytogenetic, biochemical, immunological, bacteriological).
Differential diagnosis carry out with residual organic dementia as a result of the brain infections, injuries and intoxications postponed at early children's age (in the main ambassador of the 2nd year of life); with the progressing organic dementia at such hereditary and degenerative diseases as fakomatoza — neurofibromatosis (see), tuberous sclerosis (see), a disease of Sterdzha — Weber (see. Entsefalotrigeminalny angiomatosis ) and neurolipidoses — amaurotic idiocy (see), Nimann's disease — Peak (see. Nimanna — Peak a disease ), a disease to Gosha (see. to Gosha disease ), a disease of maple syrup (see. Dekarboksilaznaya insufficiency ), and also with leukoencephalitis (see) and nek-ry rare forms of dementia of children's age. The lake needs to differentiate from conditions of weak-mindedness at the progreduated mental diseases which began at early children's age — schizophrenia (see) and epilepsies (see), and also from borderline cases of intellectual insufficiency, including so-called delays of mental development.
Treatment generally symptomatic. Apply the pharmaceuticals stimulating brain metabolism (nootropil, Aminalonum, Cerebrolysinum, lipocerebrinum, glutaminic to - that, etc.); psychostimulants (Phenaminum, Sydnocarbum, Indopanum, etc.); vitamins of group B; dehydrating agents (magnesium sulfate, Diacarbum, lasixum); drugs of resorptional action (biyokhinol, potassium iodide), biogenic stimulators (e.g., extract of an aloe). At a convulsive syndrome appoint systematic reception of antiepileptic means. At O. complicated by psychopatholike and neurosis-like frustration apply neuroleptics (aminazine, melleril, Tisercinum, neuleptil) and tranquilizers (Elenium, Seduxenum, Radedormum, Trioxazinum). Specific (pathogenetic) therapy is possible at nek-ry metabolic forms O. (at a fenilketonuriya, a gomotsisteinuriya, a galactosemia, a fruktozuriya, a sakharo-zuriya). It includes observance of a diet in the form of restriction of the natural proteinaceous products or products containing certain carbohydrates (milk, fruit, sugar, sweets); at a fenilketonuriya, besides, use a hydra of lysates of protein or mixes of amino acids, free of phenylalanine (aminogran, Berlophenum, etc.). At O. connected with an inborn hypothyroidism carry out treatment by Thyreoidinum.
Rehabilitation sick O. provides actions for special types of education and training of mentally retarded children and teenagers (oligophrenopedagogy): consultations concerning education in a family, education in children's homes and a day nursery for children with effects of early organic lesions of c. N of page, training of children with moronity at auxiliary schools (according to special programs), education of children and teenagers with sharp and deep mental retardation in specialized nursing homes of social security. Labor adaptation and the prof. orientation of mentally retarded children in degree of moronity begins at auxiliary schools, at a deficiency of intellect — in activity therapy workshops at psychoneurological clinics. Adults with easy degree of mental retardation, as a rule, work at a factory where high qualification is not required. Sick O. with a superficial deficiency of intellect adapt in activity therapy workshops at psychoneurological clinics and by nek-ry productions (on simple operations). Teenagers with deeper deficiency of intellect receive elementary labor skills in nursing homes of social security or in a family. Adult patients in degree of a superficial deficiency of intellect usually become disabled II group, at a deep deficiency of intellect and an idiocy appoint the I group of disability. In cases of the expressed moronity the III group of disability can be appointed. The solution of a question of group of disability at superficial mental retardation depends on the accompanying psychological frustration.
Forecast also the wedge, forms O depends on depth of mental retardation. At uncomplicated O. in degree of moronity full social adaptation is possible, edges are excluded by need for observation of the psychiatrist. The social forecast of a superficial deficiency of intellect and idiocy and the forecast in the relation a wedge, recovery are adverse.
Primary prevention includes medicogenetic consultation (see. Medicogenetic consultation ), prenatal diagnosis of anomalies of development of c. N of page of an embryo and a fruit with the help amniocentesis (see), allowing to warn the birth of the mentally retarded child; exchange hemotransfusion in cases of incompatibility of an organism of mother and a fruit on a Rhesus factor and factors of AB0; specific treatment of pre-natal and post-natal neuroinfections. Secondary prevention consists in early identification of mental retardation and timely use of actions for treatment and rehabilitation of patients.
In the Soviet Union the differentiated wide network of institutions for assistance mentally retarded in system of healthcare institutions, education and social security is created, edges are carried out by early identification, treatment and different types of rehabilitation of sick Lakes.
Forensic-psychiatric examination at O. first of all includes the solution of questions of sanity and capacity. Mentally retarded usually admit deranged degree of an idiocy and the expressed deficiency of intellect (see. Diminished responsibility ) and incapacitated (see. Incapacity ). At moronity the question of sanity and capacity is solved individually, taking into account not only degrees of intellectual insufficiency, but also features of the emotional and strong-willed sphere. Degree of intellectual insufficiency and expressiveness of personal immaturity are also criteria for evaluation of testimony of sick O. at their examination as witnesses and the victims.
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V. V. Kovalyov.