Presentation within the framework of psychological education "Psychological characteristics of mentally retarded children. Oligophrenia

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Contents: 1. The concept of developmental delay 2. Historical background 3. Forms and classification of mental retardation 4. Causes of developmental retardation 5. Psychological and pedagogical characteristics of children with mental retardation. 6. Treatment and prevention of mental retardation 7. References

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1. The term “developmental delay” refers to syndromes of temporary lag in the development of the psyche as a whole or its individual functions (motor, sensory, speech, emotional-volitional). continues to remain in the circle of preschool and gaming interests. With mental retardation, children cannot engage in school activities, perceive school activities and carry them out. ("Concise Defectological Dictionary", 1964) 3. This is a complex polymorphic disorder in which different children have underdeveloped various components of their mental and psychophysiological activity.

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In the 30-50s, developmental delays denoted a wide clinical group of mental development disorders, including mental retardation, and for the group of children under discussion the term “mental infantilism” was used. Since the early 60s, the diagnosis “delayed rate of mental development” came into clinical use, and a little later - “temporary delays in mental development” These terms were proposed by G.E. Sukhareva. Conventionally, four main periods of development of psychological assistance for children with developmental problems can be distinguished: 1. Descriptive (XIX century, Eduard Seguin; P.Ya. Troshin) 2. Experimental implementation -psychological methods (end of the 19th beginning of the 20th century, E. Claperd, M. Montessori, A.N. Grabov, V.P. Kashchenko, P.P. Kashchenko.) 3. Development of psychological correction (L.S. Vygotsky, A. .Adler, Z. Freud, J. Piaget) 4. Intensive formation of practical psychology (Yu.V. Mikadze, N.K. Korsakova 1994; N.N. Pylaeva, T.V. Akhutina, 1997; N. I Semago, M.M. Semago 2000; Dubrovina I.V.; A.I. Zakharov 1982; V.V.Gorbuzov 1990)

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- Caused by mental and psychophysical infantilism (uncomplicated and complicated underdevelopment of cognitive activity and speech, where the main place is occupied by underdevelopment of the emotional-volitional sphere.) - Occurring in the early stages of life, caused by long-term asthenic and cenbrasthenic states.

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There are several classifications of mental retardation: 1. Classification by V.V. Kovalev in 1979. He identifies three variants of mental retardation, caused by the influence of biological factors: - Dysontogenetic (in states of mental infantilism) - mild organic lesions of the central nervous system) - Mental retardation of a secondary nature with sensory defects (with early hearing and vision impairment) And the fourth option by V.V. Kovalev associates with early social deprivation

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Classification by K.S. Lebedinskaya (1980) 1. ZPR of constitutional origin - true infantilism - harmonic or psychophysical infantilism - mental infantilism 2. Delayed mental development of somatogenic genesis 3. Delayed mental development of psychogenic genesis 4. Delay of cerebral-organic genesis.

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M.S. Pevzner and T.A. Vlasov are distinguished by: 1. Unfavorable course of pregnancy: - maternal illnesses during pregnancy (rubella, mumps, influenza); - chronic diseases of the mother (heart disease, diabetes, thyroid disease); - toxicosis, especially in the second half of pregnancy; - toxoplasmosis; - intoxication of the mother’s body due to the use of alcohol, nicotine, drugs, chemicals and medications, hormones; - incompatibility of the blood of mother and baby according to the Rh factor.

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2. Pathology of childbirth: - injuries due to mechanical damage to the fetus when using various means of obstetrics (for example, applying forceps); - asphyxia of newborns and its threat. 3. Social factors - pedagogical neglect as a result of limited emotional contact with the child both in the early stages of development (up to 3 years) and in later age stages - the presence of a criminal environment - Low level of education of parents 4. Hereditary factors - Congenital (including including hereditary) inferiority of the central nervous system

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- Decreased performance due to the phenomena of cerebrospinal gravis, psychomotor disinhibition, and excitability that occur in children. - Low level of cognitive activity and slow rate of information processing - Absolute sensitivity thresholds are not violated - Instability of attention, disturbances in the speed of switching attention, its volume is reduced - memory is limited in volume, short-term mechanical predominates over logical - visual-effective thinking is developed to a greater extent than visual-figurative and especially verbal-logical - There are slight disturbances of speech functions - Immaturity of the emotional sphere and motivation, lack of formation of voluntary behavior

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1. Immaturity of the emotional-volitional sphere 2. Impaired attention 3. Impaired perception 4. Features of memory 5. Speech problem 6. Lag in the development of all forms of thinking

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Diagnosing mental retardation in the early stages of development seems quite problematic. His methods are based primarily on a comparative analysis of the child’s development with age-appropriate norms.

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development of gnostic processes of a child with mental retardation, which correspond to his physical and mental capabilities; strengthening the social position of the individual child with mental retardation in the group of peers and in the family; formation of adequate self-esteem, independence and activity in children with mental retardation. solving particular problems is also of great importance: eliminating secondary personal reactions to an existing defect, diagnosing and correcting the style of family education, preventing the development of hospitalism or its consequences, etc.

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1. Creating the most favorable conditions for pregnancy and childbirth, avoiding risk factors, paying close attention to the development of the baby from the very first days of his life. 2. It is necessary to show the newborn to a neurologist. 3. Bodily-emotional contact.

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1. Ulienkova U.V., O.V. Lebedeva “Organization and content of special psychological assistance to children with developmental problems.” M. Publishing center “Academy”, 2002. 2.M.B.Kalashnikova “Fundamentals of correctional pedagogy and special psychology” Educational methodological manual. 3. M. B. Kalashnikova “Age sensitivity in children with mental retardation” V. Novgorod, 2002 4. I. I. Mamaichuk “Psychocorrectional psychology for children with developmental problems.” St. Petersburg: “Speech” 2004 5. I. I. Mamaichuk, M. N. Ilyina. “Psychologist’s help to a child with mental retardation” St. Petersburg: “Rech” 2004 6.

Plan

1. Structure of the defect in oligophrenia

2.Forms of oligophrenia

3.Genetic forms of mental retardation. Chromosomal forms

4. Monogenic forms

5. Mental retardation syndromes with an unspecified type of inheritance

6. Exogenous forms of mental retardation

Literature.

1. STRUCTURE OF DEFECT IN OLIGOPHRENIA

Oligophrenia is a special clinical form of mental retardation, characterizing a general irreversible underdevelopment of brain structures, and above all, the cortical brain structures that are most late in ontogenesis, especially the frontal and parieto-temporo-occipital regions of the cerebral cortex.

The structure of the defect in oligophrenia is characterized by the totality and hierarchy of underdevelopment of cognitive activity, especially thinking and personality. Totality manifests itself in the underdevelopment of all neuropsychic functions. Hierarchy lies in the predominant underdevelopment of cognitive functions, and above all abstract thinking. Thinking in mental retardation has a specific, situational nature: children experience the greatest difficulties in generalization processes and in understanding cause-and-effect relationships.

In older preschool age, they cannot cope with tasks on differentiating objects according to essential features, and therefore experience particular difficulties in tasks on classification, identifying the fourth “extra” picture, and understanding the hidden meaning of a story. The lack of abstract thinking makes it difficult for children to master counting and especially in solving problems. Not understanding the meaning of problems, they often solve them mechanically and have difficulty accepting adult help.

It is especially difficult in mental retardation to transfer a method of action learned in a specific task to new, similar conditions. This is due both to the specifics of thinking itself and to the low mobility and inertia of mental processes. Children with mental retardation are characterized by a tendency toward stereotypical thinking and actions.

All thought processes in mental retardation are characterized by severe stiffness. All types of activities and behavior of children are monotonous, stereotypical, with a predominance of elements of imitation and copying. Underdevelopment of mental activity and inertia of thought processes in mental retardation adversely affect the formation of all cognitive processes, and above all perception.

The underdevelopment of analytical-synthetic activity in mental retardation causes a specific feature of perception: children are able to identify in objects only certain specific properties and qualities (color, shape, size), but cannot establish connections between them and independently form a holistic idea of ​​the object. Naturally, these disorders manifest themselves differently depending on age, the severity of the intellectual defect and training. Not being able to identify the essentials in objects and phenomena, children experience major difficulties in comparison operations based on essential features. They cannot establish differences in similar objects and commonality in different ones.

L. S. Vygotsky wrote that concreteness in the field of thinking and stiffness in the field of dynamic systems are internally connected and appear to be a unity, and not a double sign that is randomly combined in a weak-minded child. The concreteness of the thinking and action of a mentally retarded child thus means that every thing and every event acquires its meaning depending on the situation. Therefore, any abstraction is difficult. Everything connected with the concept, with the imagination, with the unreal turns out to be extremely difficult for such a child.

When diagnosing oligophrenia, identifying the main disorder of cognitive activity allows us to correctly understand the entire structure of the defect, i.e., the relationship and interdependence of symptoms in the holistic clinical picture. The leading symptom of oligophrenia is underdevelopment of cognitive activity. A defect in cognitive activity is characteristic of any oligophrenic dementia, regardless of the degree of its severity. It is the underdevelopment of cognitive activity in an uncomplicated form of oligophrenia that leads to specific disorders of speech, affective-volitional sphere, motor skills and personality. Thus, a feature of speech in mental retardation is not only the later development of active speech, but also a significant lag in the understanding of addressed speech.

In oligophrenia, there is insufficient development of both the phonetic-phonemic and lexical-grammatical aspects of speech. In addition, with oligophrenia, persistent disturbances in sound pronunciation are observed much more often than in children with normal intelligence. This is due both to the underdevelopment of analytical-synthetic processes, the function of self-control, underdevelopment of phonemic perception and analysis, disorders of articulatory motor skills of various origins, and to the inertia of mental processes.

Such characteristics of abnormal development as violations of verbal mediation and the formation of concepts are especially pronounced in mentally retarded children (V. I. Lubovsky, V. G. Petrova).

A feature of the emotional sphere of children with oligophrenia is the underdevelopment of more complex and differentiated emotions, which develop most late in postnatal ontogenesis. In their emotional experiences, as well as in mental activity, children cannot separate the main from the secondary, and therefore they can give violent emotional reactions on insignificant occasions and react weakly to serious life events. Only immediate experiences are relevant for them; they cannot assess the possible consequences of certain actions or events, just as in thinking they are characterized by stiffness, weak switchability, inertia and stereotyped emotions. Cognitive emotions in these children are usually not developed; inadequacy of emotional reactions and inability to suppress their immediate instincts are often observed. With mental retardation, it is difficult to form abstract concepts of good and evil, a sense of duty, the ability to self-control and predict the consequences of one’s actions.

Features of disturbances in the emotional-volitional sphere in the main, “nuclear”, uncomplicated form of oligophrenia are in accordance with the degree of decline in intelligence.

The inability to think abstractly manifests itself in all areas of neuropsychic activity. In the processes of perception, there is a lack of analytical-synthetic activity, so children’s perceptions are poor, they do not fully reflect the surrounding reality, and there is no active creative nature of perception. As G. E. Sukhareva noted, “a mentally retarded child sees little when he looks, and hears little when he listens.”

With oligophrenia, there is also a lack of voluntary focused attention, a violation of its distribution in the process of mental activity, and weakness of logical memory. In the material being memorized, the child cannot identify the essentials and establish logical connections in the plot. A manifestation of the predominant underdevelopment of the youngest and specifically human functions, characteristic of mental retardation, is the close relationship between the underdevelopment of cognitive activity, and above all abstract thinking, and the voluntary regulation of all mental activity, self-control functions, higher emotions, speech, and fine differentiated motor skills. This determines the main specificity of the defect in mental retardation - totality and hierarchy.

2. FORMS OF OLIGOPHRENIA

There are several clinical forms of oligophrenia, the differentiation of which is based on both etiological and clinical-pathogenetic principles. In defectology, the classification of M. S. Pevzner is most widespread, according to which there are five main forms of oligophrenia: uncomplicated; complicated by neurodynamic disorders such as increased excitability or inhibition; complicated by disorders of various analytical systems (vision, hearing, musculoskeletal system, speech); oligophrenia with psychopathic forms of behavior; oligophrenia with severe frontal insufficiency.

Among the clinical classifications of oligophrenia, etiological ones occupy a large place, i.e., the identification of its various forms depending on the cause. For clinical diagnosis, it is important to identify among all forms of oligophrenia specific syndromes of exogenous or hereditary origin, which are characterized by peculiar combinations of an intellectual defect with sensory, speech, motor disorders, malformations of internal organs, various anomalies in the structure of the face, skull, limbs, endocrine and neurological pathology. For a doctor, it is first of all important to identify forms of oligophrenia in which an intellectual defect is the main component or consequence of various diseases of the nervous system and anomalies of its development of various etiopathogenesis. The teacher and educator need basic modern ideas about these forms of oligophrenia for a correct understanding of the clinical diagnosis, clinical and social prognosis and career guidance. Among these forms of oligophrenia, genetically determined diseases of the central nervous system are of particular importance. Currently, several hundred hereditary diseases are known that are accompanied by an intellectual defect. In addition, more than ten new forms of genetic diseases combined with mental retardation are described every year.

In recent years, the predominant frequency of hereditary forms of mental retardation has been proven and the following classification is proposed: chromosomal, caused by changes in the number or structure of chromosomes; monogenic, arising due to changes in one gene; mental retardation syndromes with an unspecified type of inheritance. It is assumed that among the latter, multifactorial influences, including both genetic factors and unfavorable environmental influences, may play a certain role in their occurrence.

When analyzing the causes of mental retardation, combined with multiple congenital malformations, it is important to distinguish between the concepts of congenital and hereditary pathology. It should be borne in mind that congenital defects include not only manifestations of hereditary diseases, but also any other anomalies that appear at birth, the cause of which may be harmful environmental factors affecting the fetus during critical periods of development of its brain and certain organs and systems body. Such factors include hypoxia (oxygen starvation) of the fetus, maternal alcoholism in the first months of pregnancy, viral diseases, especially rubella in pregnancy in the first trimester of pregnancy, the use of drugs with teratogenic (i.e., causing deformities) properties and other factors. Under the influence of all these and many other external factors, exogenous forms of oligophrenia arise. However, as noted above, the most common forms of mental retardation are genetic.

3. GENETIC FORMS OF MENTAL RETARDATION. CHROMOSOMAL FORMS

The orientation of specialists in various clinical manifestations of chromosomal syndromes combined with oligophrenia is important for further targeted genetic examination of the child, determining the prognosis of his further development and the profile of a special preschool and school institution, as well as medical and genetic counseling of the family in order to prevent re-births of sick children in family.

The frequency of chromosomal forms of oligophrenia is about 15.7% of all cases. The most common chromosomal forms of oligophrenia, caused by abnormalities of autosomes (i.e., any of the 22 pairs of non-sex chromosomes), occur in children with profound intellectual defects. The most common syndrome caused by autosomal abnormalities is Down syndrome. In the chromosome set of Down disease there is an additional 21st chromosome. There are three cytogenetic variants of Down syndrome.

The main causes of chromosomal disorders are considered to be: ionizing radiation, severe infections and intoxications, endocrine disorders, exposure to chemicals, including some medications used one to two years before conception, as well as general environmental pollution. For some chromosomal diseases, including Down's disease, the age of the parents, especially the mother, also matters. In chromosomal forms of oligophrenia, intellectual underdevelopment is combined with characteristic abnormalities in the structure of the face and skull, general dysplasticity of the physique, often with bone anomalies, developmental defects of the fingers and toes, malformations of internal organs and other disorders.

The external signs of Down's disease are especially well known; they are so specific that the diagnosis of the disease is usually made in the maternity hospital by the appearance of the child. First of all, attention is drawn to the reduced size of the skull with a flattened and oblique occiput, narrow palpebral slits with an overhanging third eyelid, a flat face with protruding zygomatic arches, asymmetrical, low-lying small ears, a high palate, a half-open mouth with a large tongue and thick lips. These external signs are combined with abnormalities in the structure of the chest, limbs, fingers and toes, poor posture, congenital heart defects and other anomalies.

It is important to keep in mind that mental development disorders in these children may be further aggravated by visual and hearing defects. In addition, in many cases, children with Down syndrome have various endocrine disorders with obesity, insufficiency of the thyroid and gonads. Early detection of this deficiency is important for proper treatment to reduce the severity of the intellectual defect. Underdevelopment of intelligence in Down's disease varies from profound to more moderate degrees of mental retardation with a clear predominance of severe forms.

A feature of oligophrenia in Down's disease is the combination of pronounced underdevelopment of abstract thinking, logical memory and the function of active attention with relatively intact mechanical memory, pronounced imitation and great suggestibility. These children are usually affectionate, attached to loved ones, they are able to differentiate the attitude of others towards them, but many of them are prone to mood swings and irritability. As they age, their intellectual defect becomes more and more noticeable and the degree to which they lag behind their peers may increase.

Intellectual and emotional disorders in Down syndrome are usually combined with speech disorders. First of all, its late development attracts attention. Speech impairments are especially pronounced in the presence of hearing defects. Characterized by a high incidence of conductive hearing impairment. Currently, issues of speech disorders and especially the processes of speech perception in children with Down syndrome are considered not only in connection with their intellectual defect, but also in the aspect of ear and hemispheric dominance. Special studies of ear dominance using dichotic listening have found left ear dominance in many of these children. At the same time, a clear connection was revealed between the dominance of the left ear and the syndrome itself, and not with the fact of mental retardation. Given the connection between aural and hemispheric dominance, it has been suggested that children with Down syndrome use the less efficient right hemisphere for linguistic processing of speech information.

The main thing that has drawn the attention of specialists in recent years is the similarity in the structure of the intellectual defect in Down's disease and Alzheimer's disease. It is characteristic that this similarity in Down syndrome begins to appear in adulthood. Molecular biological studies in recent years confirm this similarity: the predominance of degenerative vascular disorders, the presence of diabetes mellitus, developing cataracts, a tendency to malignant neoplasms, specific hearing impairments, etc.

In recent years, much attention has been paid to the issues of early rehabilitation of children with Down syndrome with the help of special correctional programs aimed at developing adequate educational interaction between mother and child, as well as programs that stimulate their mental development.

Other chromosomal forms of oligophrenia associated with autosomal abnormalities are quite rare. With all these forms, a complex defect is observed, that is, deep intellectual underdevelopment is combined with defects in vision, hearing, musculoskeletal system and other disorders. Thus, similar disorders occur with trisomy 13 (Patau syndrome) and trisomy 18 (Edwards syndrome). The combination of deep mental retardation with abnormalities of the musculoskeletal system is observed in trisomy 8 (Varkani syndrome). The combination of oligophrenia with visual and musculoskeletal defects is characteristic of ring chromosome 13.

In recent years, anomalies of chromosome 15 have attracted great interest from specialists, in which mental retardation is combined with microcephaly and a characteristic facial structure (oval face, high cheekbones and full cheeks). The intellectual defect is combined with motor disinhibition syndrome and sometimes convulsive seizures.

With abnormalities of the sex chromosomes, mental retardation is observed much less frequently. Only 1% of all forms of oligophrenia are caused by sex chromosome abnormalities.

4. MONOGENIC FORMS

The term “mental retardation” is used here due to the fact that many of the diseases described have a complex structure of intellectual defect, including features of both mental retardation and dementia. In addition, some of these diseases are characterized by increasing dementia. These forms of mental retardation are caused by changes in a single gene. The group of monogenic forms of mental retardation is the most numerous, despite the rarity of its individual forms. This group includes some hereditary diseases of the central nervous system, many of which are associated with inborn errors of metabolism. Most hereditary metabolic defects are classified as enzymopathies and are caused by disorders of protein, carbohydrate, fat and other types of metabolism.

Mental retardation due to hereditary diseases of amino acid metabolism

Many hereditary diseases of amino acid metabolism manifest themselves in the first or second year of life in the form of delayed psychomotor development in combination with skin disorders and often convulsive syndrome. These disorders are progressive. The rate of progression of the disease depends on the type of biochemical defect and the time of initiation of treatment. Effective treatments for many of these diseases have now been developed. These diseases include primarily phenylketonuria - PKU - a disease caused by hereditary disorders of phenylalanine metabolism. Due to a disturbance in the metabolism of this amino acid, there is an excessive accumulation of toxic substances that affect the central nervous system.

The incidence of this disease is 1:10,000 newborns. The number of children among the mentally retarded is approaching 12%. The more severely socially affected the population being examined is, the more often the disease is detected. In a socially prosperous population, the frequency is only about 1%.

It is important to keep in mind that children with PKU are born with a full-fledged brain, since the biochemical processes of the fetus occur at the expense of the mother’s body. Biochemical disturbances that occur after birth have a toxic effect on the developing brain of the child, resulting in impaired brain development and growth. In the first two to three years of life, progressive dementia is especially clearly observed.

The increase in intellectual defect is combined with signs of moderate microcephaly and retardation in physical development. Speech disorders are considered characteristic: in some cases it may be completely absent, in others its general underdevelopment is observed in combination with echolalia and perseverations. Many of these children do not strive to communicate; their emotions are poor and often inadequate.

Many children are characterized by behavioral disorders in the form of motor disinhibition, increased excitability, and impulsive and aggressive forms of behavior may be observed. Patients often experience convulsions, which tend to be serial. The frequency of seizures ranges from 20 to 50%.

The appearance of the patients is peculiar: blond hair, blue eyes, weak skin pigmentation combined with individual dysplastic signs (moderate microcephaly, high palate, ear deformities, etc.).

Various neurological disorders are also characteristic in the form of violent movements, disorders of balance and coordination of movements, strabismus, disorders of muscle tone, the presence of paralysis and paresis. In addition, skin disorders are common in PKU: exudative diathesis, eczema, dermatitis.

Biochemical diagnosis of the disease is important. Currently, mass screening programs for PKU are being implemented, the most widely used are the Tatri test and the Felling test (a qualitative reaction to phenylpyruvic acid in urine using a 10% solution of ferric chloride).

Histidinemia is a hereditary disease caused by a disorder in the metabolism of the amino acid histidine. A characteristic feature is the presence of a complex defect: a combination of mental retardation with severe speech underdevelopment - motor alalia, behavioral disorders, movement disorders in the form of lack of balance and coordination of movements. In severe forms of the disease, seizures are also observed, and hearing defects are also common. These children are usually fair-haired and blue-eyed. Practice shows that early delay in psychomotor development in these children in combination with motor disorders often leads to an erroneous diagnosis of cerebral palsy.

Mental retardation due to hereditary disorders carbohydrate metabolism

Galactosemia is caused by a disorder of galactose metabolism. In terms of the frequency of hereditary metabolic disorders, galactosemia ranks second after PKU.

In severe cases, the disease manifests itself from the first days of the child’s life in the form of digestive disorders and signs of intoxication (hypotrophy, vomiting, diarrhea, refusal to feed), jaundice also develops with an increase in the size of the liver. The degree of decline in intelligence varies. In the most severe cases, a complex defect is observed: a combination of mental retardation with visual impairment (often blindness) due to congenital or early developed cataracts (usually at 4-7 weeks of life).

Currently, a system for early diagnosis of galactosemia has been developed.

Mental retardation due to hereditary disorders of lipid (fat) metabolism.

With hereditary disorders of fat metabolism, mental retardation is often combined with motor and sensory defects, seizures, and behavioral disorders.

One of the serious diseases from this group is amaurotic idiocy, or Tay-Sachs disease. The disease first appears at the age of 4-6 months and is characterized by increasing dementia, progressive loss of vision, convulsions, paresis and paralysis. There are many other forms of mental retardation associated with hereditary metabolic disorders. Thus, when there are disturbances in the exchange of intracellular enzymes, toxic products accumulate in cells, which are the cause of a special group of diseases - storage diseases. Clinical manifestations of these diseases include a combination of progressive mental retardation with defects in vision, musculoskeletal system and various disorders of the internal organs. These diseases include various forms of mucopolysaccharidosis, as well as neural ceroid lipofuscinosis.

Mental retardation due to fragile chromosome syndrome .

Monogenic forms of mental retardation include a special disease that is currently attracting the attention of specialists - fragile X syndrome, or Martin-Bell syndrome. This disease is the most common among all monogenic forms of mental retardation. Its name is associated with a gap at the end of the long arm of the X chromosome, which is detected during a special cytogenetic examination. The frequency of this syndrome is 1:1350 among boys and 1:2033 among girls, or 1:1634 in children of both sexes.

Early diagnosis of this form of mental retardation is important for timely implementation of therapeutic and corrective measures and for medical and genetic counseling of the family, which helps prevent further births of children with this disease. However, the absence of pronounced congenital malformations in this syndrome makes its early diagnosis difficult. Therefore, for the diagnosis of this disease, early detection of specific abnormalities in physical, psychomotor and speech development is of great importance.

Since the predominance of this form of mental retardation in males is known, boys with delays in psychomotor and speech development and having a number of characteristic anomalies should be especially carefully examined.

Body weight, length, and head circumference are usually slightly increased in affected boys. Also noteworthy are large hands and feet, protruding ears, an elongated face, a high forehead, flattening of the middle part of the face and a slightly enlarged lower jaw. They are usually blondes with light irises. An increased incidence of submucosal clefts of the palate or uvula is characteristic. There is often increased joint mobility and skin stretchability.

Neurological symptoms are nonspecific and include muscle hypotonia, impaired coordination of movements, uniform revival of tendon reflexes, individual violent stereotypical movements in the form of grimaces, frowning eyebrows, squinting eyes, etc. In some cases, convulsive seizures are observed.

The severity of the intellectual defect varies widely; mild forms often predominate. Since an intellectual defect is always combined with various speech disorders, these children are often placed in speech therapy institutions. Speech disorders manifest themselves in the form of systemic underdevelopment, various forms of dysarthria, combined with peculiar speech perseverations, tempo disturbances (usually the rate of speech is accelerated), hesitation, difficulties in selecting the right word: A kind of oral apraxia, as well as alalia, may be observed.

One of the characteristic signs of fragile X syndrome is macroorchidism (an increase in the size of the testicles): in some boys it appears already from birth.

Characteristic signs of this syndrome include typical changes in the electroencephalogram (EEG), which shows bursts of slow activity against the background of an unformed alpha rhythm. Mental retardation in fragile X syndrome not only manifests itself in varying degrees of severity and is combined with speech disorders, but often has an atypical character. Atypicality is manifested in the unevenness of the intellectual defect with a predominant deficiency of a number of higher mental functions. The defectiveness of these functions causes and intensifies specific difficulties in children’s learning to count, read, write, as well as design, drawing, etc. skills.

The complicated nature of the intellectual defect is due to severe impairments of attention, mental performance, memory, emotional sphere and behavior.

Children, as a rule, are distinguished by motor disinhibition, impulsiveness, and a tendency to develop neurotic and neurosis-like disorders, especially in the form of fears. In addition, some of them may exhibit behavioral characteristics reminiscent of autism. Such children avoid communicating with others, take a long time to get used to new people, prefer to avoid eye contact, and are characterized by stereotypical hand movements in the form of shaking and patting. However, unlike children with autism, they are emotionally adequate, attached to loved ones, and the characteristics of their behavior are usually situationally determined.

5. MENTAL RETARDATION SYNDROMES WITH UNSPECIFIED TYPE OF INHERITANCE

Currently, there is a whole group of genetic syndromes, including mental retardation in combination with sensory, motor and other multiple developmental defects, the type of inheritance of which is still not clear. These syndromes are described both in foreign and domestic literature.

One example of such complicated forms of mental retardation may be Rett syndrome.

The disease was first described as an independent nosological form about twenty years ago by the Austrian psychiatrist A. Rhett. The disease is observed only in girls, and its signs become obvious from five months to one and a half years, when the girl, who had previously developed normally, begins to lose acquired psychomotor skills. Loss of skills and delay in acquiring new ones are combined with stereotypical rhythmic movements of the hands (patting, rubbing, wringing of hands), alternating with bouts of screaming. After a few weeks or months, the condition stabilizes and the child’s profound mental retardation becomes apparent.

6.EXOGENOUS FORMS OF MENTAL RETARDATION

The frequency of exogenous forms of mental retardation is about 25% of all cases. Among the exogenous forms of mental retardation, the attention of many researchers is attracted by various intrauterine lesions of the fetus, leading to underdevelopment of the brain. Among the causes of these forms of mental retardation, great importance is attached to viral infections suffered by the mother during pregnancy (especially in the first trimester) and alcoholism. Thus, with rubella in the mother during pregnancy, children often experience brain developmental defects, causing mental retardation in combination with visual and hearing defects, as well as heart defects - “rubella, or rubeolar, fetal syndrome.” If the mother is alcoholic during pregnancy, fetal alcohol syndrome (FAS) often occurs. In this syndrome, mental retardation of varying degrees is combined with a complex of specific somatic and neurological disorders and developmental defects: microcephaly, craniofacial anomalies, pre- and postnatal growth retardation, malformations of internal organs, etc. With ASP, mental retardation of varying degrees of severity is always combined with emotional and personal immaturity and behavioral disorders. Mental development disorders in children with ASP may be aggravated by hearing and vision defects.

Literature

1. Auerbach S. Heredity. - M., 1999.

2. Badalyan L.O. Hereditary diseases in children. - M., 1991.

3. Bochkov N.P. Human genetics (Heredity and pathology). - M., 1987

4. Kozlova S.I. Hereditary syndromes and medical genetic counseling. - M., 1996.

5. Muntzing A. Genetics. - M., 1989.

6. Ravich-Scherbo I. V. Psychogenetics. - M., 2003.

Description of the presentation by individual slides:

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Psychological characteristics of mentally retarded children. Oligophrenia. Systematics of oligophrenia according to G.E. Sukhareva. Features of the development of oligophrenic children. Click to edit Master title style

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Mentally retarded children are one of the most numerous categories of children who deviate from the norm in their development. They make up about 2.5% of the total child population. The concept of “mentally retarded child” includes a very diverse group of children, who are united by the presence of damage to the cerebral cortex, which is diffuse in nature

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The overwhelming majority of mentally retarded children are those whose mental retardation arose as a result of various organic lesions, mainly the most complex and late-forming brain systems, in the period before the development of speech (up to 2 - 3 years). These are the so-called oligophrenic children (from the Greek: low-minded).

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Oligophrenia belongs to a large group of diseases associated with disorders of ontogenesis (dysontogeny). It is considered as an anomaly with underdevelopment of the psyche, personality and entire body of the patient. Oligophrenia is a clinically homogeneous group of diseases of various etiologies, united by two obligatory characteristics: 1) mental underdevelopment with a predominance of intellectual disability; 2) lack of progression.

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G. E. Sukhareva divides all clinical forms of oligophrenia into three groups depending on the time of exposure to the etiological factor. I. Oligophrenia of endogenous nature (due to damage to the generative cells of the parents): a) Down's disease; b) true microcephaly; c) enzymopathic forms of oligophrenia with hereditary disorders of various types of metabolism, including phenylpyruvic oligophrenia, oligophrenia associated with galactosemia, sucrosuria, and other enzymopathic forms of oligophrenia; d) clinical forms of oligophrenia, characterized by a combination of dementia with impaired development of the skeletal system and skin (dysostotic oligophrenia, xerodermic oligophrenia).

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II. Embryo- and fetopathies: a) oligophrenia caused by rubella measles contracted by the mother during pregnancy (rubeolar embryopathy); b) mental retardation caused by other viruses (influenza, mumps, infectious hepatitis, cytomegaly); c) oligophrenia caused by toxoplasmosis and listeriosis; d) oligophrenia caused by congenital syphilis; e) clinical forms of oligophrenia caused by hormonal disorders of the mother and toxic factors (exo- and endotoxic agents); f) oligophrenia caused by hemolytic disease of the newborn.

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III. Oligophrenia arising in connection with various harmful effects during childbirth and early childhood: a) oligophrenia associated with birth trauma and asphyxia; b) oligophrenia caused by traumatic brain injury in the postnatal period (in early childhood); c) oligophrenia caused by encephalitis, meningoencephalitis and meningitis suffered in early childhood.

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Motor skills Mentally retarded children of both infancy and later preschool age are characterized by significant deviations in the development of motor skills. Much later than their peers, they begin to reach for the toy hanging in front of them, trying to get it, and also later they begin to sit, stand, move in space by crawling, and walking. Slow development of the motor sphere significantly reduces the child’s ability to get acquainted with the objective world around him and orient himself in space. The movements of mentally retarded preschoolers are characterized by awkwardness, poor coordination, excessive slowness or, on the contrary, impulsiveness.

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Attention The attention of mentally retarded children is predominantly involuntary. It is characterized by small volume, instability, and in some cases, difficult switchability. Oligophrenic preschoolers with characteristic lethargy may seem attentive at first glance, but usually these are just external manifestations of their slowness and pathological inertia. Mentally retarded elementary school students are also very inattentive, which significantly interferes with their learning and contributes to the appearance of many errors when performing even tasks that are quite accessible to them in terms of the level of development of their cognitive activity. The inattention of oligophrenic children of all ages is to a certain extent due to the weakness of their volitional sphere. They cannot properly concentrate on the activity at hand or work without distractions. The unformed interests of mentally retarded children are also of great importance.

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Interests Mentally retarded preschoolers are characterized by decreased interest in the immediate object environment. Excited children grab everything that comes into their field of vision, without thinking about whether it is possible to do so. However, they are not driven by interest, but by their inherent impulsiveness. They immediately throw away what they took, because the item itself is not interesting to them. Inhibited oligophrenics do not seem to notice what is around them. Nothing attracts their attention. Children with intact behavior behave somewhat more adequately. Attracted by the bright color or novelty, they pick up the object and look at it for a while, but do not show real interest in the object. They rarely ask adults questions and do not try to learn something new about a subject on their own. Their actions with the object consist of trying to put it in their mouth or banging it on the table or floor.

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Perception In mentally retarded children, more often than in normally developing children, there are disturbances in the sensation of various modalities and, accordingly, in the perception of objects and situations. Visual perception has been studied most comprehensively. The uniqueness of children's viewing of the space around them has been established, which significantly affects their behavior. Insufficient differentiation of visual perception is revealed in children’s inaccurate recognition of colors and color shades that are close in spectrum, inherent in certain objects, in the global vision of these objects, i.e. in the absence of identifying their characteristic parts, particles, proportions and other structural features. There is also a more frequent decrease in visual acuity than during normal development, which deprives the image of an object of its inherent specificity. Mentally retarded children are characterized by a peculiar recognition of objects and phenomena. They tend to identify somewhat similar objects. Thus, kindergarten students and first-graders often do not see the difference between a cat and a squirrel, a compass and a clock, and combine geometric shapes such as a square and a rectangle into one group.

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Memory The memory of mentally retarded children of preschool age is very poorly developed. This is especially clearly revealed in the examples of memorizing verbal material, which is not surprising. After all, children are just beginning to master their native speech. They are somewhat more successful in remembering visual material - bright pictures depicting well-known objects, or real, frequently used objects. However, they are not retained in their memory for long. Experiments have shown that memorization is facilitated in cases where the teaching of mentally retarded children relies heavily on memory processes, which are characterized by greater originality and encourage children to name the perceived object. The need to remember perceived material causes great difficulties for preschoolers. Not mastering intentional processes, they remember only what made a great impression on them - they really liked it, attracted them, scared them, etc. Often, remembering is replaced by a conversation on another topic that is far from what was perceived.

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The amount of material memorized by auxiliary school students is significantly less than that of their normally developing peers. It has been established that if normally developing children remember 7±2 objects presented simultaneously, then their mentally retarded peers remember 3. The accuracy and strength of students’ memorization of both verbal and visual material is low. In reproducing it, they skip a lot, rearrange elements that make up a single whole, allow for many repetitions and at the same time introduce unnecessary elements as a result of various, often random, associations. At the same time, mentally retarded children, characterized by a predominance of excitation processes, display a particularly pronounced tendency to such additions. Students with a predominance of inhibition processes remember a smaller amount of material, but the number of additions they make is insignificant.

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Thinking In mentally retarded children of preschool age, there is a deficiency of all levels of mental activity. Visually effective forms of thinking are most accessible to mentally retarded primary schoolchildren. However, children experience difficulties when completing tasks. Thus, they, like preschoolers, find it difficult to put together a simple cut-out picture. At primary school age, children's actions with objects are often impulsive, not related to a mental task, and have no cognitive significance. Students experience great difficulties in tasks involving the use of visual-figurative thinking. Particularly difficult are tasks that require children to use verbal and logical thinking. They often do not understand even simple texts intended for normally developing preschoolers that contain temporal, causal and other relationships. Mentally retarded students reproduce the material in a simplified manner, omit many, sometimes the most significant parts of it, change the sequence of semantic links in the text, and do not establish the necessary relationships between them.

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Speech The development of speech in a mentally retarded child occurs in a unique way and with great delay. He comes into emotional contact with his mother later and less actively. At the age of about a year, the sound complexes pronounced by children are poor and characterized by reduced emotional coloring. They have a weak desire to imitate the speech of an adult. They do not respond to the simplest situational commands; they only catch the intonation, but not the content of the speech addressed to them. Speech heard for a long time is of little interest to mentally retarded preschoolers. They do not listen to her enough and, as a rule, begin to try to speak with a great delay. However, gradually, at different times, they still master basic verbal communication, even in cases where the family does not provide them with any real help and they do not attend a special preschool institution. This is due to the need, living among people, to interact with others. Many mentally retarded preschoolers pronounce their first words at 2-3 years old or even at 5 years old. These are mainly nouns - names of objects in the immediate environment and verbs denoting frequently performed actions. The phonetic structure of speech in almost all children by the beginning of school is far from fully formed. Exceptions are very rare.

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Volitional sphere Its formation is directly related to the appearance of speech, which allows the child to understand the need for one or another method of action. However, the formation of voluntary regulation of behavior in a mentally retarded child is difficult. Volitional activity, formed under the influence of correctional-oriented training, significantly contributes to the mental, speech and overall personal development of this category of children. The volume of attention and its stability increases; The memory capacity increases and its productivity increases. In turn, the development of thinking, speech, and memory stimulates the formation of strong-willed personality traits of oligophrenic schoolchildren. From junior to senior school age, mentally retarded students experience the development of voluntary mental processes.

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Drawing All normally developing children, starting from early preschool age, enjoy drawing. At first, they simply draw various scribbles on paper, then they begin to see in them the resemblance to some familiar object, and later they try to deliberately depict individual objects. The visual activity of mentally retarded children is formed slowly and in a unique way. Their drawings contain characteristic features that are diagnostic in nature. The skills of children deprived of special education in kindergarten or family remain for a long time at the level of simple scribbles, and only towards the end of preschool childhood can one see subject and, to some extent, plot drawings, executed very imperfectly, with gross errors and inaccuracies.

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Labor activity In a special (correctional) general education school for mentally retarded children, one of the main types of activity, in addition to educational activities, is labor. Tasks presented at an accessible level create the most favorable conditions for correcting mental development deficiencies. It has been established that oligophrenic schoolchildren, especially primary school students, cannot always subordinate their actions to the task assigned to them. Often they begin to perform a work task without proper preliminary orientation in it, without analyzing it. Students complete a task without realizing its ultimate goal and without a plan of action. This leads to the fact that in the process of activity the child’s methods of action do not change even when they turn out to be clearly erroneous and do not lead to the desired results, as required by the task.

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Mentally retarded schoolchildren tend to transfer, unchanged, elements of past experience to the solution of a new work task. They complete the task without realizing its specifics. When completing a task, there is often a “slipping” from an activity that is difficult for children to an easier one: not realizing the task at hand and not showing proper interest in the work, the student tries to complete it in the usual way, i.e. refers to it formally. The majority of mentally retarded students are characterized by the inability to adequately assess their capabilities.

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The motivational-need sphere of mentally retarded schoolchildren is characterized by immaturity, weak expression and short-term motivation for activity, limited motives, and insufficient formation of social needs. These children are characterized by a “short” (“close”) motivation for activity, as well as a weak and elementary motivation for relationships. The motives that motivate them to perform one or another activity (be it play, work, study) often turn out to be weakly expressed, unstable and quickly exhausted. They are assimilated by children formally, without becoming their own impulses. Correctional and educational work with mentally retarded students is aimed at developing stable and active motives for learning and moral order.

Definition of familial forms of oligophreniaFamily forms of oligophrenia mean
cases of birth of siblings in one family
brothers and sisters) suffering from oligophrenia.
Their frequency has been established: up to 10% of the total number
oligophrenic.
To resolve the issue of the relative role
heredity in the etiology of oligophrenia
researchers often turn to studying
twins and families of oligophrenics.

A significant contribution to research on the heritability of dementia has been made by the study of twins.

Of the 6,700 registered
in Denmark the weak-minded
the researchers selected 122
sets of twins from
which are suitable for
research turned out to be 66
steam.
In 80% of cases in monozygotic
both twins were couples
feeble-minded
In 8% of cases in dizygotic
pairs of twins both twins
turned out to be weak-minded.

Defects combined with forms
hereditary dementia
V. P. Efroimson described
a large number of forms
hereditary dementia,
compatible with others
defects: violation
motor skills, spastic
syndrome, myatrophy,
cataracts, microphthalmos and
anophthalmos, underdevelopment
hair and teeth, etc.
Vladimir Pavlovich
Efroimson (1908-1989) - Soviet
geneticist

Of practical importance are data on
empirical risk of mental retardation in offspring,
which according to E.W. Reed, S.C. Reed:
In a marriage between two
mentally retarded42.1%
In a marriage between mentally
retarded and normal19.9%

Research by V.F. Shalimova

In order to establish the number
mentally retarded children in
families where one or both
parents have oligophrenia,
V. F. Shalimov researched 62
families. Based on study
families were made up
pedigrees in which
detailed
characteristics of each member
family, regardless of whether they have
any pathology.
Genealogy of the family being studied
covered at least three
generations. Depending on the
suspected etiology
illnesses in parents and children
divided into 3 groups.
Valery Fedorovich Shalimov
- psychiatrist,
psychotherapist

Groups of children studied

Group 1 consisted of children from families whose etiology was
oligophrenia which played a major role
exogenous factors;
The 2nd group consisted of children from families whose etiology
oligophrenia which occurred either only
hereditary factors, or the latter in
combination with exogenous hazards;
The 3rd group consisted of children, etiology
mental retardation of parents whose parents cannot be determined
managed.

Progress of the examination

During the examination of children and their families there was
the impact of various options was assessed
marriages on the occurrence of mental underdevelopment
in descendants:
a person with oligophrenia is a healthy spouse;
a patient with oligophrenia - a patient with another
mental illness;
a patient with oligophrenia - a patient with oligophrenia
The influence of the microenvironment was taken into account
the strength of each family, the presence of relatives with
any deviations in mental development.

Results of examination of children of group 1

For oligophrenia of exogenous origin in cases where marriage
a person with mental retardation and a healthy person enter, they are born
mostly healthy children (out of 13 children, 12 were healthy).
There were healthy children in other marriage options. However, if
the second parent suffers from mental retardation, then in these families more often
children appeared with various mental disorders
development, including oligophrenia.
In a family in which the etiology of oligophrenia is dominated by
hereditary factors, in case of marriage, oligophrenic is healthy
Children with oligophrenia are born relatively often. But
the largest number of such children was in families where both parents
suffered from oligophrenia.
According to the structure of the defect, all identified children with oligophrenia were
divided into 2 subgroups.

Results of examination of children of group 2

In children of the 2nd subgroup, along with symptoms characteristic
for oligophrenic dementia, atypical for
oligophrenia symptoms that dramatically change the structure
oligophrenic defect.
In some children, against the background of general underdevelopment of cognitive
activity is extremely inhibited,
slowness, lethargy and passivity.
Others have underdevelopment of cognitive activity
combined with psychopathic behavior.
In the 2nd group there were 4 families with mental retardation
could be traced in several generations: among children, parents,
grandparents. In these cases, oligophrenia was noted in
children born from several (2 or 3) marriages mentally
retarded parent. The defect is present in most of the children named
4 families in their structure belong to the uncomplicated form
oligophrenia (according to the classification of M. S. Pevzner).

Results of examination of children of group 3

Results of examination of children from families with
which etiology of oligophrenia remains
unclear (3rd group), show that in these
families have children with different
deviations in mental development, including
including those suffering from oligophrenia. Number
sick children in these families are significantly
increases if the other parent is suffering
either oligophrenia or some other
mental illness.

MAOU "School No. 54 for students with disabilities" Perm

CONCEPT

ABOUT MENTAL RETARDATION

Teacher of the Highest Qualification Category

Kataeva Elena Viktorovna

• Mental retardation(F70-F79) - a state of delayed or incomplete mental development, which is characterized primarily by a decrease in developmental skills and skills that determine the general level of intelligence (i.e., cognitive abilities, language, motor skills, social functioning) *

THE CONCEPT OF MENTAL RETARDATION

Disease code *

Degree of mental retardation

mild mental retardation

moderate mental retardation

severe mental retardation

profound mental retardation

* International Classification of Diseases - 10

• Oligophrenia (“dementia”) is not the name of any specific disease. Oligophrenia is the name of a condition that occurs after various types of damage to the central nervous system of a child in the period before the development of his speech, i.e., until about one or two years of life.

Causes of oligophrenia:

• inferiority of genetic cells of parents (including hereditary diseases and pathology of embryogenesis)
• intrauterine damage to the fetus
• natural injuries and asphyxia
• diseases affecting the central nervous system of a child at an early age.

• early stage of damage to the central nervous system
• non-progressiveness - subsequent cessation of the disease

• Classification of states of oligophrenia (according to M.S. Pevzner)

the main uncomplicated form of oligophrenia

oligophrenia complicated by neurodynamic disorders:

• with a predominance of excitation processes with a predominance of inhibition processes
• with a predominance of excitation processes
• with a predominance of inhibition processes

oligophrenia with psychopathic forms of behavior

mental retardation, complicated by disturbances in the system of individual analyzers

oligophrenia with dysfunction of the frontal lobes of the brain

• Dementia (“decay”) is an acquired dementia of a progressive nature. Lack of intelligence develops when the structures of the brain are mostly already formed, the psyche has also reached a certain level and its decay begins after a time of normal development.

Rheumatic lesions of the nervous system

Syphilis of the brain

Epilepsy

Schizophrenia

phenomena of hydrocephalus

UNEVENITY OF LESIONS

TOTAL CHARACTER OF UNDERDEVELOPMENT OF COGNITIVE FUNCTIONS

REGULATORY FRAMEWORK

• Order No. 1015 of August 30, 2013 “On approval of the procedure for organizing and implementing educational activities in basic general educational programs - educational programs of primary general, basic general and secondary general education” (Section III “Features of organizing educational activities for persons with disabilities” )
• SanPiN 2.4.2.3285-15 “Sanitary and epidemiological requirements for the conditions and organization of training and education in organizations carrying out educational activities according to adapted basic general education programs for students with disabilities” from 01.09.2016

ORGANIZATION OF EDUCATION FOR STUDENTS WITH LIMITED HEALTH OPPORTUNITIES

INCLUSION

CORRECTION CLASSES

SELECTED EDUCATIONAL ORGANIZATIONS

for the deaf

for the hearing impaired

for the blind

for the visually impaired

with severe speech impairments

with mental retardation

with autism spectrum disorders

List of information sources:

• Federal Law of December 29, 2012 No. 273-FZ “On Education in the Russian Federation”
• Federal State Educational Standard for Special Education for Students with Disabilities
• Federal State Educational Standard On students with mental retardation (intellectual impairment)
• Vygotsky L. S. Collection. op. T. 5. M., 1983
• Kalmykova E.A. Psychology of persons with mental retardation: Proc. -- method. manual / Compiled by E.A. Kalmykova. – Kursk: Kursk. state University, 2007
• Lebedinsky V.V. Mental development disorders in children. M., 1985
• Pevzner M. S. et al. Clinical and genetic studies of oligophrenia. M, 1972
• Rubinstein S. Ya. Psychology of mentally retarded schoolchildren. M., 1986
• www.presentationmagazine.com