Description of the presentation on 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 mass of children who are united by the presence of damage to the cerebral cortex, which has a diffuse character.
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The vast majority of mentally retarded children are those in whom 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.
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Oligophrenia belongs to a large group of diseases associated with impaired ontogenesis (dysontogeny). It is considered as an anomaly with underdevelopment of the psyche, personality and the whole organism of the patient. Oligophrenia is a clinically homogeneous group of diseases of various etiologies, united by two mandatory features: 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 an 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 various kinds 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 fetopathy: a) oligophrenia caused by rubella measles suffered by the mother during pregnancy (rubeolar embryopathy); b) oligophrenia caused by other viruses (influenza, parotitis, infectious hepatitis, cytomegaly); c) oligophrenia caused by toxoplasmosis and listeriosis; d) mental retardation caused by congenital syphilis; e) clinical forms of oligophrenia due to hormonal disorders of the mother and toxic factors (exo- and endotoxic agents); e) oligophrenia due to hemolytic disease of the newborn.
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III. Oligophrenia arising in connection with various harmful effects during childbirth and in 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 transferred 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 a toy hanging in front of them, trying to get it, and also later they begin to sit, stand, crawl in space, and walk. The slow development of the motor sphere significantly reduces the child's ability to get acquainted with the objective world around him, to navigate 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 mostly involuntary. It is characterized by a 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 outward manifestations of their slowness and pathological inertia. Mentally retarded elementary school students are also very inattentive, which greatly interferes with their learning, 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 activities performed, work without being distracted. The unformedness of the interests of mentally retarded children is also of great importance.
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Interests Mentally retarded preschoolers are characterized by a reduced interest in the immediate objective environment. Excitable children grab everything that comes into their field of vision, without thinking about whether it is possible to do it. However, they are not driven by interest, but by their inherent impulsiveness. They immediately drop what they have taken, because the subject itself is not interesting for them. Inhibited oligophrenics, as it were, do not notice what is around them. Nothing draws their attention. Children with safe behavior behave somewhat more adequately. Attracted by bright colors or novelty, they take the object in their hands, look at it for a while, but show no real interest in the object. They rarely ask adults questions, do not try to learn something new about the subject on their own. Their actions with the object consist in the fact that they try to put it in their mouth or knock it on the table or floor.
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Perception In mentally retarded children, more often than in normally developing children, there are violations of sensation of various modalities and, accordingly, the perception of objects and situations. Visual perception has been studied most comprehensively. The peculiarity of children's observation of the space surrounding them has been established, which significantly affects their behavior. Insufficient differentiation of visual perception is found in children's inaccurate recognition of colors close in spectrum and color shades inherent in certain objects, in the global vision of these objects, i.e. in the absence of separation of their characteristic parts, particles, proportions and other peculiarities of the structure. There is also a more frequent decrease in visual acuity than during normal development, which deprives the image of the object of its inherent specificity. Mentally retarded children are characterized by a peculiar recognition of objects and phenomena. They tend to identify to some extent similar objects. So, pupils of kindergartens, first-graders often do not see the difference between a cat and a squirrel, a compass and a clock, they combine such geometric shapes as a square and a rectangle into one group.
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Memory The memory of mentally retarded preschool children is very poorly developed. This is especially evident in the examples of memorizing verbal material, which is not surprising. After all, children are just beginning to master their native language. They remember visual material somewhat more successfully - bright pictures depicting well-known objects, or real, frequently used objects. However, they remain in their memory for a short time. Experiments have shown that memorization is facilitated in those cases where the education of mentally retarded children relies to a large extent on memory processes, which are characterized by a great originality of children prompting them to name the perceived object. Great difficulties are caused in preschoolers by the need to remember the perceived material. Not owning deliberate processes, they remember only what made a great impression on them - they liked it very much, attracted, frightened, etc. Often, recollection is replaced by a conversation on another, far from the perceived topic.
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The volume of material memorized by the students of the auxiliary school is significantly less than that of their normally developing peers. It has been established that if normally developing children memorize 7 ± 2 objects simultaneously presented, then their mentally retarded peers - 3. The accuracy and strength of memorization by students of both verbal and visual material is low. When reproducing it, they miss a lot, rearrange the elements that make up a single whole, allow 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, show a particularly pronounced tendency to such introductions. Students with a predominance of inhibition processes memorize a smaller amount of material, but the number of introductions is insignificant.
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Thinking In mentally retarded children of preschool age there is an insufficiency of all levels of mental activity. Visual-effective forms of thinking are most accessible to the mentally retarded junior schoolchildren. However, when completing tasks, children experience difficulties. So, they, like preschoolers, find it difficult to put together a simple cut picture. At primary school age, the actions of children with objects are often impulsive, not associated with a mental task, and have no cognitive significance. Tasks involving the use of visual-figurative thinking cause great difficulties for students. Tasks that require verbal-logical thinking from children are especially difficult. 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 way, omit many, sometimes the most significant parts of it, change the sequence of semantic links of the text, and do not establish the necessary relationships between them.
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Speech The formation of the speech of a mentally retarded child is carried out in a peculiar way and with a great delay. He comes into emotional contact with his mother later and less actively. At the age of about one year, the sound complexes pronounced by children are poor and are 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 catch only the intonation, but not the content of the speech addressed to them. For a long time, sounding speech is of little interest to mentally retarded preschoolers. They don't listen enough to her and tend to start trying to speak with a big delay. However, gradually they at different times, but still master the elementary verbal communication even in cases where the family does not provide them with any real assistance, and they do not attend a special pre-school 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 - the 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 schooling is far from being fully formed. Exceptions are very rare.
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Volitional sphere Its formation is directly connected with the appearance of speech, which allows the child to understand the need for a particular mode of action. However, the formation of arbitrary regulation of the behavior of a mentally retarded child is difficult. Volitional activity, which is formed under the influence of remedial-oriented education, greatly contributes to the mental, speech and, in general, personal development of this category of children. Increases the amount of attention, its stability; increases the amount of memory, increases its productivity. In turn, the development of thinking, speech, memory stimulates the formation of volitional qualities of the personality of oligophrenic schoolchildren. From junior to senior school age, mentally retarded students develop voluntary mental processes.
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Drawing All normally developing children, starting from the younger preschool age, draw with pleasure. At first, they simply draw various scribbles on paper, then they begin to see in them a resemblance to some familiar object, and later they try to intentionally depict individual objects. The visual activity of mentally retarded children is formed slowly and in a peculiar way. In their drawings there are characteristic features that are diagnostic in nature. The skills of children deprived of special education in kindergarten or family, for a long time remain at the level of simple doodles, and only by the end of preschool childhood can one see subject and to some extent plot drawings, made 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 activities, in addition to teaching, is labor. Tasks presented at an accessible level create the most favorable conditions for correcting deficiencies in mental development. It has been established that oligophrenic schoolchildren, especially those in the lower grades, cannot always subordinate their actions to the task assigned to them. Often they begin to perform a labor task without proper preliminary orientation in it, without its analysis. Pupils do the task without realizing it 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 results that are necessary, as required by the task.
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Mentally retarded schoolchildren tend to transfer elements of past experience unchanged to the solution of a new labor task. They perform the task without realizing its specifics. When completing a task, there is often a "slip" from a difficult activity for children to an easier one: not realizing the task and not showing due interest in the work, the student tries to complete it in the usual way, i.e. refers to it formally. Most mentally retarded students are characterized by the inability to adequately assess their capabilities.
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The motivational-need sphere In mentally retarded schoolchildren, it is characterized by immaturity, weak expression and short duration of motives for activity, limited motives, insufficient formation of social needs. These children are characterized by a "short" ("close") motivation of activity, as well as a weak and elementary motivation of relationships. The motives that prompt them to perform a particular activity (whether it be play, work, or study) often turn out to be weakly expressed, unstable, and are 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 in them stable and active motives for learning and moral order.
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 capacity) *
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 ("Little Mind") 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 a child in the period before the development of his speech, that is, up to about a year 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 trauma and asphyxia
- diseases that affect the central nervous system of a child at an early age.
- early damage to the central nervous system
- non-progredient - 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 predominance of braking processes
oligophrenia with psychopathic behaviors
oligophrenia, complicated by disorders in the system of individual analyzers
oligophrenia with impaired function 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 for the most part are already formed, the psyche has also reached certain level and its decay begins after the time of normal development.
Rheumatic damage to the nervous system
Syphilis of the brain
Epilepsy
Schizophrenia
hydrocephalus phenomena
IRREGULARITY OF LESIONS
TOTAL CHARACTER OF UNDEVELOPMENT OF COGNITIVE FUNCTIONS
REGULATORY FRAMEWORK
- Order No. 1015 dated August 30, 2013 “On approval of the procedure for organizing and implementing educational activities in the main general educational programs- educational programs of primary general, basic general and secondary general education" (Section III "Features of the organization of 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 that carry out educational activities on adapted basic general education programs for students with disabilities" from 09/01/2016
ORGANIZATION OF EDUCATION FOR STUDENTS WITH LIMITED HEALTH OPPORTUNITIES
INCLUSION
CORRECTION CLASSES
INDIVIDUAL EDUCATIONAL ORGANIZATIONS
for the deaf
for the hearing impaired
for the blind
for the visually impaired
with severe speech impairment
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"
- GEF IEO for students with disabilities
- GEF On students with mental retardation (intellectual disabilities)
- Vygotsky L. S. Sobr. op. T. 5. M., 1983
- Kalmykova E.A. Psychology of persons with mental retardation: Uch. -- method. allowance / Compiled by E.A. Kalmykov. - Kursk: Kursk. state university, 2007
- Lebedinsky VV Violations of mental development in children. M., 1985
- Pevzner M. S. et al. Clinical and genetic studies of oligophrenia. M, 1972
- Rubinshtein S. Ya. Psychology of a mentally retarded schoolchild. M., 1986
- www.presentationmagazine.com
Definition of family forms of oligophreniaFamily forms of oligophrenia are understood as
cases of birth in the same family of siblings (relatives
brothers and sisters) suffering from oligophrenia.
Their frequency is established: up to 10% of the total number
oligophrenics.
To address the question of the relative role
heredity in the etiology of oligophrenia
researchers often turn to the study
twins and families of oligophrenics.
A significant contribution to research on the heredity of dementia was made by the study of twins.
Out of 6700 registeredin Denmark the feebleminded
researchers selected 122
groups of twins
suitable for
research turned out 66
steam.
In 80% of cases in identical
couples both twins were
imbeciles
In 8% of cases in fraternal
pair of twins both twins
turned out to be stupid. Defects matching shapes
hereditary dementia
V. P. Efroimson described
a large number of forms
hereditary dementia,
combined with others
defects: violation
motility, spastic
syndrome, myatrophy,
cataract, microphthalmos and
anophthalmos, underdevelopment
hair and teeth, etc.
Vladimir Pavlovich
Efroimson (19081989) - Soviet
geneticist Of practical importance are data on
empirical risk of mentally retarded offspring,
which is according to E.W. Reed, S.C. reed:
Married between two
mentally retarded42.1%
In a marriage between mentally
retarded and normal19.9%
Research by V.F. Shalimova
In order to establish the numbermentally retarded children in
families where one or both
parents are mentally retarded
V. F. Shalimov researched 62
families. Based on the study
families were made up
pedigrees that
detailed
characteristics of each member
families, regardless of whether they have
him any pathology.
Genealogy of the studied family
covered at least three
generations. Depending on the
suspected etiology
diseases in the parent of the child
were divided into 3 groups.
Valery Fedorovich Shalimov
- psychiatrist,
psychotherapist
Study groups of children
The 1st group consisted of children from families, in etiologyoligophrenia which played an important role
exogenous factors;
The 2nd group consisted of children from families, in etiology
oligophrenia which took place either only
hereditary factors, or the last
combined with exogenous hazards;
the 3rd group consisted of children, etiology
oligophrenic parents whose parents cannot be established
managed.
Survey progress
During the examination of children and their families,assessed the impact of various options
marriages to the occurrence of mental underdevelopment
in descendants:
a patient with oligophrenia is a healthy spouse;
a patient with oligophrenia - a patient with another
mental illness;
oligophrenic patient oligophrenic patient
The influence of the microenvironment was taken into account
the strength of each family, the presence of relatives with
any deviations in mental development.
The results of the examination of children of the 1st group
With oligophrenia of exogenous origin in cases where marriagea patient with oligophrenia and a healthy one enter, are born
predominantly healthy children (out of 13 children, 12 are healthy).
There were also healthy children in other marriages. However, if
the second parent suffers from oligophrenia, then in these families more often
there were children with various mental disorders
development, including oligophrenia.
In a family whose etiology of oligophrenia is dominated by
hereditary factors, with the option of marriage, oligophrenia is healthy
relatively often, children with oligophrenia are born. 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.
The results of the examination of children of the 2nd group
In children of the 2nd subgroup, along with symptoms characteristic offor 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 slow,
slowness, lethargy and passivity.
Others - underdevelopment of cognitive activity
associated with psychopathic behavior.
In the 2nd group there were 4 families in which oligophrenia
traced over several generations: in children, parents,
grandfathers and grandmothers. In these cases, oligophrenia was noted in
children born from several (2 or 3) marriages mentally
retarded parent. The defect in most of the children named
4 families in its structure belongs to the uncomplicated form
oligophrenia (according to the classification of M. S. Pevzner).
The results of the examination of children of the 3rd group
The results of a survey of children from familieswhich the etiology of oligophrenia remains
unclear (3rd group), show that in these
families have children with different
developmental disabilities, including
including those suffering from oligophrenia. Number
sick children in these families significantly
increases if the other parent suffers
or oligophrenia, or some other
mental illness.
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Contents: 1. The concept of developmental delay 2. Historical background 3. Forms and classification of mental retardation 4. Reasons for the occurrence of mental retardation 5. Psychological and pedagogical characteristics of children with mental retardation. 6. Treatment and prevention of mental retardation 7. References slide 3
1. The term "developmental delay" means 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, gaming interests. With mental retardation, children cannot get involved in school activities, perceive school lessons and fulfill them. ("A Brief Defectological Dictionary", 1964) 3. This is a complex polymorphic disorder in which various components of their mental and psychophysiological activity are underdeveloped in different children. slide 4
In the 30-50s, developmental delays denoted a wide clinical group of mental developmental disorders, including oligophrenia, and for the group of children under discussion, the term “mental infantilism” was used. "temporary delays in mental development" These terms were proposed by G.E. Sukhareva. It is conditionally possible to distinguish four main periods in the development of psychological assistance to children with developmental problems: 1. Descriptive (XIX century, Eduard Segen; P.Ya. Troshin) 2. Implementation experimentally -psychological methods (late 19th - early 20th century, E. Clapered, M. Montessori, A.N. Grabov, V.P. Kashchenko, P.P. Kashchenko.) 3. The 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. Ya Semago, M.M.Semago 2000; Dubrovina I.V. 1990; A.I. Zakharov 1982, A.S. Spiva kovskaya 1988; V.V. Gorbuzov 1990) slide 5
- Due to mental and psychophysical infantilisms (uncomplicated and complicated underdevelopment of cognitive activity and speech, where the main place is occupied by the underdevelopment of the emotional-volitional sphere.) - Occurring in the early stages of life, due to prolonged asthenic and cenbrasthenic conditions. slide 6
There are several classifications of ZPR: 1. Classification by V.V. Kovalev in 1979. He distinguishes three variants of ZPR due to the influence of biological factors: - Dysontogenetic (in states of mental infantilism) - mild organic lesions of the central nervous system) - ZPR of a secondary nature with sensory defects (with early hearing and vision disorders) And the fourth option V.V. Kovalev associates with early social deprivation Slide 7
Classification K.S. Lebedinskaya (1980) 1. ZPR of constitutional origin - true infantilism - harmonic or psychophysical infantilism - mental infantilism 2. Delay of mental development of somatogenic genesis 3. Delay of mental development of psychogenic genesis 4. Delay of cerebral-organic genesis. Slide 8
M.S. Pevzner and T.A. Vlasov distinguish: 1. Unfavorable course of pregnancy: - mother's illness during pregnancy (rubella, mumps, influenza); - chronic diseases mother (heart disease, diabetes, thyroid disease); - toxicosis, especially the second half of pregnancy; - toxoplasmosis; - intoxication of the mother's body due to the use of alcohol, nicotine, drugs, chemical and medicines, hormones; - incompatibility of the blood of the mother and the baby according to the Rh factor. Slide 9
2. Pathology of childbirth: - trauma due to mechanical damage to the fetus when using various means of obstetrics (for example, 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 old) 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 Slide 10
- Decrease in working capacity due to the phenomena of cerebrosthenia, psychomotor disinhibition, and excitability that occur in children. - Low level of cognitive activity and a slow rate of information processing - The thresholds of absolute sensitivity 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 over logical predominates - visual-effective thinking is developed to a greater extent than visual-figurative and especially verbal-logical - There are slight violations of speech functions - Immaturity of the emotional sphere and motivation, unformed voluntary behavior slide 11
1. Immaturity of the emotional-volitional sphere 2. Violation of attention 3. Violation of perception 4. Features of memory 5. Problem of speech 6. Lag in the development of all forms of thinking slide 12
Diagnosis of ZPR in the early stages of development seems to be quite problematic. His methods are based primarily on comparative analysis development of the child with the norms corresponding to his age. slide 13
development of the gnostic processes of a child with mental retardation, which correspond to his physical and mental capabilities; strengthening the social position of the personality of a 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. The solution of particular problems is also of great importance: the elimination of secondary personal reactions to an existing defect, the diagnosis and correction of the style of family education, the prevention of the development of hospitalism or its consequences, etc. Slide 14
slide 15
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1. Making the most favorable conditions for the course of pregnancy and childbirth, avoidance of risk factors, 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. Body-emotional contact. Slide 17
1. Ulyenkova 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. Kalashnikov "Fundamentals correctional pedagogy and Special Psychology” Textbook. 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: "Rech" 2004 5.I.I.Mamaychuk, M.N.Ilyina. "Help of a psychologist for a child with mental retardation" St. Petersburg: "Rech" 2004 6. Plan
1. The structure of the defect in oligophrenia
2. Forms of oligophrenia
3. Genetic forms of mental retardation. Chromosomal forms
4.Monogenic forms
5. Syndromes of mental retardation with an unspecified type of inheritance
6. Exogenous forms of mental retardation
Literature.
1. STRUCTURE OF THE DEFECT IN OLIGOPHRENIA
Oligophrenia is a special clinical form of mental retardation, which characterizes the general irreversible underdevelopment of brain structures, and above all, the cortical brain structures that form the latest in ontogeny, especially the frontal and parietal-temporal-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 is manifested in the underdevelopment of all neuropsychic functions. Hierarchy - in the predominant underdevelopment of cognitive functions, and above all abstract thinking. Thinking in oligophrenia has a specific, situational character: children experience the greatest difficulties in the processes of generalization, in understanding cause-and-effect relationships.
In senior preschool age they do not cope with tasks for differentiating objects according to essential features, and therefore they experience special difficulties in tasks for classifying, highlighting the fourth “extra” picture, while understanding the hidden meaning of the story. The lack of abstract thinking makes it difficult for children to learn how to count, and especially in solving problems. Not understanding the meaning of the tasks, they often solve them mechanically, they hardly accept the help of an adult.
Especially difficult in oligophrenia is the transfer of the method of action learned in a particular task to new analogous conditions. This is due both to the specifics of thinking itself and to the low mobility and inertia of mental processes. Children with oligophrenia are characterized by a tendency to stereotyping in thinking and actions.
All mental processes in oligophrenia are characterized by pronounced stiffness. All types of activities and behavior of children are monotonous, stereotyped, with a predominance of elements of imitation and copying. The underdevelopment of mental activity and the inertness of mental processes in oligophrenia adversely affect the formation of all cognitive processes and, above all, perception.
The underdevelopment of analytical and synthetic activity in oligophrenia causes a specific feature of perception: children are able to single out only their individual specific properties and qualities (color, shape, size) in objects, but they cannot establish connections between them and form an independent holistic view of the object. Naturally, these disorders manifest themselves differently depending on age, the severity of the intellectual defect and training. Unable to identify the main thing in objects and phenomena, children experience the main difficulties in comparison operations according to essential features. They cannot establish differences in similar objects and the general in different ones.
L. S. Vygotsky wrote that concreteness in the realm of thinking and rigidity in the realm of dynamic systems are internally connected and appear as a unity, and not as a dual trait that happens to be combined in a weak-minded child. The concreteness of the thought and action of a weak-minded 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, the isolation of the main disorder of cognitive activity allows you to correctly understand the entire structure of the defect, that is, the relationship and interdependence of symptoms in a holistic clinical picture. Such a leading symptom in oligophrenia is the 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. So, a feature of speech in oligophrenia is not only the later development of active speech, but also a significant lag in the understanding of addressed speech.
With oligophrenia, insufficient formation of both the phonetic-phonemic and lexical-grammatical side of speech is noted. In addition, with oligophrenia, much more often than in children with normal intelligence, persistent disturbances in sound pronunciation are noted. This is due both to the underdevelopment of analytical and synthetic processes, the function of self-control, the underdevelopment of phonemic perception and analysis, articulatory motility disorders of various origins, and 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 respond poorly to serious life events. Only direct experiences are relevant for them, they cannot assess the possible consequences of certain actions, events, as well as in thinking, stiffness, weak switchability, inertness and stereotyping of emotions are characteristic. Cognitive emotions in these children are usually not developed, often there is an inadequacy of emotional reactions, an inability to suppress their immediate desires. With oligophrenia, abstract concepts of good and evil, a sense of duty, the ability to self-control and predict the consequences of one's actions are hardly formed.
Features of violations of the emotional-volitional sphere in the main, "nuclear", uncomplicated form of oligophrenia are in accordance with the degree of decrease in intelligence.
The inability to abstract thinking manifests itself in all areas of neuropsychic activity. In the processes of perception, there is a lack of analytical and synthetic activity, therefore the perceptions of children are poor, they do not fully reflect the surrounding reality, 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 a weakness in logical memory. In the memorized material, the child cannot single out the essential and establish logical connections in the plot. A manifestation of the predominant underdevelopment of the youngest and specifically human functions, characteristic of oligophrenia, is the close relationship between the underdevelopment of cognitive activity, and above all abstract thinking, and arbitrary regulation of all mental activity, self-control functions, higher emotions, speech, fine differentiated motor skills. This determines the main specifics of the defect in oligophrenia - 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 and pathogenetic principles. In defectology, the most common classification is M. S. Pevzner, according to which five main forms of oligophrenia are distinguished: uncomplicated; complicated by disorders of neurodynamics by the type of increased excitability or inhibition; complicated by disorders of various analyzer systems (vision, hearing, musculoskeletal system, speech); oligophrenia with psychopathic behaviors; oligophrenia with severe frontal insufficiency.
Among the clinical classifications of oligophrenia, etiological ones occupy a large place, that is, the allocation of its various forms depending on the cause. For clinical diagnosis, it is important to distinguish among all forms of oligophrenia specific syndromes of exogenous or hereditary origin, in which peculiar combinations of an intellectual defect with sensory, speech, motor disorders, and developmental defects are characteristic. internal organs, various anomalies in the structure of the face, skull, limbs, endocrine and neurological pathology. For a doctor, first of all, it is important to highlight the forms of oligophrenia, in which an intellectual defect is the main component or consequence of various diseases of the nervous system and anomalies in 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, accompanied by an intellectual defect. In addition, more than ten new forms of genetic diseases associated with mental retardation are described annually.
In recent years, the predominant frequency of hereditary forms of mental retardation has been proven and the following classification is proposed: chromosomal, due to a change in the number or structure of chromosomes; monogenic, arising in connection with 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 adverse influences, can play a certain role in their occurrence. environment.
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 malformations include not only manifestations of hereditary diseases, but also any other anomalies that manifest themselves at birth, the cause of which may be harmful environmental factors that affect the fetus during critical periods in the development of its brain and certain organs and systems. organism. Such factors include hypoxia (oxygen starvation) of the fetus, mother's alcoholism in the first months of pregnancy, viral diseases, especially rubella of pregnant women 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 genetic counseling of the family in order to prevent the rebirth of sick children in family.
The frequency of chromosomal forms of oligophrenia is about 15.7% of all its cases. Most often, chromosomal forms of oligophrenia due to abnormalities of autosomes (ie, any of the 22 pairs of non-sex chromosomes) occur in children with a profound intellectual defect. The most common autosomal abnormality syndrome is Down's disease. In the chromosome set in Down's disease, there is an additional 21st chromosome. There are three cytogenetic variants of Down's disease.
The main causes of chromosomal disorders are: 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 pollution environment. For some chromosomal diseases, including Down's disease, the age of the parents, especially the mother, also matters. In chromosomal forms of oligophrenia, underdevelopment of the intellect is combined with anomalies in the structure of the face and skull characteristic of each syndrome, general dysplastic 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 sloping occiput, narrow palpebral fissures with an overhanging third eyelid, a flat face with protruding zygomatic arches, asymmetric, low-lying small auricles, 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, posture disorders, congenital heart defects and other anomalies.
It is important to keep in mind that mental developmental disorders in these children can be further aggravated due to visual and hearing defects. In addition, in many cases, children with Down's disease have various endocrine disorders with obesity, insufficiency of the thyroid and sex glands. Early detection of this deficiency is essential for proper treatment to reduce the severity of the intellectual defect. Underdevelopment of intelligence in Down's disease varies from deep to more moderate degrees of oligophrenia with a clear predominance of severe forms.
A feature of oligophrenia in Down's disease is a combination of pronounced underdevelopment of abstract thinking, logical memory and the function of active attention with a 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. With age, their intellectual defect becomes more and more noticeable and the degree of their lagging behind their peers may increase.
Intellectual and emotional disturbances in Down's disease are usually combined with speech disorders. First of all, attention is drawn to its late development. Speech disorders are especially pronounced in the presence of hearing impairments. A high frequency of conductive hearing loss is characteristic. Currently, the issues of speech disorders and especially the processes of speech perception in children with Down's disease are considered not only in connection with their intellectual defect, but also in the aspect of ear and hemispheric dominance. Dedicated ear dominance studies using dichotic listening found left ear dominance in many of these children. At the same time, a clear connection was found between the dominance of the left ear and the syndrome itself, and not with the fact of mental retardation. Given the relationship between ear and hemispheric dominance, it is suggested that children with Down's disease 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 an intellectual defect in Down's disease and Alzheimer's disease. It is characteristic that this similarity in Down's disease begins to appear in adulthood. Molecular biological studies of recent years confirm this similarity: the predominance of degenerative vascular disorders, the presence of diabetes mellitus, developing cataracts, a tendency to malignant neoplasms, specific disorders hearing, 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 an 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. In all these forms, a complex defect is observed, i.e., profound intellectual underdevelopment is combined with defects in vision, hearing, the musculoskeletal system, and other disorders. So, such violations occur with trisomy 13 (Patau syndrome) and trisomy 18 (Edwards syndrome). The combination of deep oligophrenia with anomalies of the musculoskeletal system is observed with trisomy 8 (Varkany's syndrome). The combination of oligophrenia with defects in vision and the musculoskeletal system is characteristic of the ring chromosome 13.
In recent years, special interest has been attracted by anomalies of chromosome 15, in which oligophrenia is combined with microcephaly, a characteristic facial structure (oval face, high cheekbones and full cheeks). An intellectual defect is combined with a syndrome of motor disinhibition and sometimes convulsive seizures.
With anomalies of the sex chromosomes, oligophrenia is observed much less frequently. Only 1% of all forms of oligophrenia are caused by abnormalities of the sex chromosomes.
4.MONOGENIC FORMS
The term "mental retardation" is used here due to the fact that many of the described diseases have a complex structure of an intellectual defect, including features of both mental retardation and dementia. In addition, some of these diseases are characterized by progressive 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 congenital metabolic disorders. Most hereditary metabolic defects are fermentopathies and are caused by violations of protein, carbohydrate, fat and other types of metabolism.
Mental retardation in 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 a lag in psychomotor development in combination with skin disorders and often convulsive syndrome. These disorders are progressive. The rate of the course 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 violation of the metabolism of this amino acid, there is an excessive accumulation of toxic substances that affect the central nervous system.
The frequency of this disease is 1:10,000 newborns. The number of children among the mentally retarded is approaching 12%. The harder in socially the contingent of the examined, the more often the disease is detected. In a socially prosperous contingent, 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, as a result of which the development and growth of the brain is impaired. In the first two or three years of life, progressive dementia is especially clearly observed.
The growth of an intellectual defect is combined with signs of moderate microcephaly and a lag 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, perseverations. Many of these children do not seek communication, their emotions are poor and often inadequate.
Many children are characterized by behavioral disorders in the form of motor disinhibition, increased excitability, impulsive and aggressive forms of behavior can be observed. Quite often at patients spasms which tend to seriality are noted. The frequency of convulsive syndrome is from 20 to 50%.
The appearance of patients is peculiar: blond hair, blue eyes, weak skin pigmentation in combination with individual dysplastic signs (moderate microcephaly, high palate, deformities of the auricles, etc.).
A variety of neurological disorders are also characteristic in the form of violent movements, imbalance and coordination of movements, strabismus, muscle tone disorders, 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 Tatri test and the Felling test (qualitative test for phenylpyruvic acid in urine using 10% iron trichloride solution) are the most widely used.
Histidinemia is a hereditary disease caused by a violation of the metabolism of the amino acid histidine. A characteristic feature is the presence of a complex defect: a combination of mental retardation with severe underdevelopment of speech - 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 frequent. These children are usually fair-haired, blue-eyed. Practice shows that early psychomotor retardation in these children, combined with movement disorders, often leads to an erroneous diagnosis of cerebral palsy.
Mental retardation in hereditary disorders metabolism of carbohydrates
Galactosemia is caused by a violation of the metabolism of galactose. According to the frequency of hereditary metabolic disorders, galactosemia ranks second after PKU.
In severe cases, the disease manifests itself from the first days of a 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 the early diagnosis of galactosemia has been developed.
Mental retardation in 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 amavrotic idiocy, or Tay-Sachs disease. The disease manifests itself for the first time 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. So, in violation of the metabolism of intracellular enzymes, toxic products accumulate in the cells, which are the cause of a special group of diseases - accumulation diseases. The clinical manifestations of these diseases include a combination of progressive mental retardation with defects in vision, the musculoskeletal system, and various disorders of the internal organs. These diseases include various forms of mucopolysaccharidoses, as well as neural ceroid lipofuscinosis.
Mental retardation in 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 revealed 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 the timely implementation of therapeutic and corrective measures and for medical genetic counseling of the family, which makes it possible to prevent the further birth of children with this disease. However, the absence of pronounced congenital malformations in this syndrome complicates its early diagnosis. Therefore, for the diagnosis of this disease, early detection of specific abnormalities in the physical, psychomotor and speech development.
Since the predominance of this form of mental retardation in males is known, boys with a lag in psychomotor and speech development and having a number of characteristic anomalies should be especially carefully examined.
Body weight and length, as well as head circumference, are usually slightly increased in affected boys. Also noteworthy are large hands and feet, protruding ears, an elongated face, a high forehead, a flattening of the middle part of the face, and a somewhat enlarged lower jaw. Usually they are blondes with light irises. An increased frequency of submucosal clefts of the palate or uvula is characteristic. There is often increased joint mobility and skin extensibility.
Neurological symptoms are of little specificity and include muscle hypotension, impaired coordination of movements, uniform revival of tendon reflexes, individual violent stereotyped movements in the form of grimaces, frowns, squinting of the eyes, etc. In some cases, convulsive seizures are noted.
The severity of an intellectual defect varies widely, with mild forms predominating quite often. Since an intellectual defect is always combined with a variety of speech disorders, these children are often in speech therapy institutions. Speech disorders are manifested in the form of its systemic underdevelopment, various forms of dysarthria, combined with peculiar speech perseverations, tempo disturbances (usually the speech tempo is accelerated), hesitation, difficulty in choosing the right word: There may be a kind of oral apraxia, as well as alalia.
One of the characteristic signs of fragile X syndrome is macroorchism (an increase in the size of the testicles): in some boys, it manifests itself from birth.
To characteristics This syndrome includes typical changes in the electroencephalogram (EEG), which show flashes of slow activity against the background of an unformed alpha rhythm. Mental retardation in fragile X syndrome manifests itself not only 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 the predominant insufficiency of a number of higher mental functions. The defectiveness of these functions causes and intensifies specific difficulties in the children's learning of counting, reading, writing, as well as the skills of designing, drawing, etc.
The complicated nature of the intellectual defect is due to severe disorders 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 experience behaviors reminiscent of autism. Such children avoid communication with others, get used to new people for a long time, prefer to avoid eye contact, they are characterized by stereotypical hand movements in the form of shaking, patting. However, unlike children with autism, they are emotionally adequate, attached to loved ones, and their behavior is usually situational.
5. SYNDROMES OF MENTAL RETARDATION 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 malformations, the mode 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 is Rett syndrome.
The disease was first described as an independent nosological form about twenty years ago by the Austrian psychiatrist A. Rett. The disease is observed only in girls, and its signs become apparent from five months to one and a half years, when the girl, until then developing normally, begins to lose her acquired psychomotor skills. The loss of skills and the lag in acquiring new ones is combined with stereotypical rhythmic hand movements (patting, rubbing, wringing 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 its 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 its first trimester), and alcoholism. So, with rubella in the mother during pregnancy, children often have malformations of the brain that cause mental retardation in combination with visual and hearing defects, as well as heart defects - “rubella, or rubeolary, fetal syndrome”. With maternal alcoholism 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 malformations: microcephaly, craniofacial anomalies, pre- and postnatal growth retardation, malformations of internal organs, etc. With ASD, mental retardation of varying severity is always combined with emotional and personal immaturity and behavioral disorders. Mental disorders in children with ASD may be aggravated due to hearing and vision defects.
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