Idiocy

The extreme degree of mental retardation is idiocy. To alleviate the symptoms, pathogenetic, that is, hormonal, anti-infective, as well as symptomatic treatment (neuroleptics, sedatives, nootropics) are used.

Consider the pathogenesis and etiology of the disease, its types and extent of disorders, methods of diagnosis, treatment and prevention.

Epidemiology

The prevalence of dementia is not well understood. According to the World Health Organization, about 3-5% of cases of severe mental retardation are registered. A large percentage of pathology can be traced in areas affected by eco-pathogenic factors.

The epidemiology of the pathological disorder indicates that most often the disease occurs in men, in comparison with the female sex. This ratio is more observed with mild degree of oligophrenia. In severe form, that is, idiocy, there is no significant difference between women and men.

To date, more than 300 pathological conditions have been identified, which can lead to idiocy. It can be both endogenous, that is hereditary factors, and exogenous, external factors. Particular attention is paid to family history. The risk of a child with pathology in the family with two oligophrenics is 42%, and in cases of violations of only one parent, 20%. Endogenous forms are associated with gene mutations and chromosomal aberrations.

[1], [2], [3], [4], [5], [6]

Causes of the idiocy

Mental disorders occur in patients of different ages. But if the disease manifests itself from an early age, then this indicates one of the forms of idiocy.

The causes of the disorder are associated with factors such as:

1. Various genetic diseases (Down syndrome, Prader-Willi syndrome, Angelmann syndrome) associated with chromosome disruption and gene structure, signs of physical retardation.
2. Harmful factors affecting the female body during pregnancy and adversely affecting the development of the fetus:
   • Ionizing radiation.
   • Various chemical agents (household toxins, drugs, poisons, alcohol).
   • Infectious diseases (rubella, cytomegalovirus infection, toxoplasmosis, syphilis).
   • Deficiency of iodine, which is necessary for the normal development of the nervous system.
   • Malnutrition or malnutrition (deficiency of vitamins and minerals pathologically affects fetal development).
3. Traumas of the child's brain, birth trauma (intracranial hemorrhage, squeezing the baby's head with obstetric forceps), asphyxia.
4. Iodine deficiency in the baby's diet at an early age (from birth to 3-4 years).

[7], [8], [9],

Risk factors

The development of any disease is associated with certain circumstances or agents (hereditary, infectious, viral, bacterial). Risk factors for dementia are a number of socio-hygienic stressors. That is, the health of a pregnant woman at the time of the birth process, the presence of chronic and acute diseases, genetic pathologies, occupational hazards, artificial feeding and even material well-being can affect the health of the child.

The risk of developing mental abnormalities and other pathologies significantly increases if during pregnancy a woman has not abandoned bad habits. Smoking, alcoholism, drug addiction negatively affect the development of the fetus at the time when its body is laid. Children born to such mothers are susceptible to various diseases. Most often these are respiratory, endocrine and cardiovascular diseases, congenital pathologies. Premature pregnancy is another factor that increases the possibility of a baby with defects. Such children may lag behind in their intellectual and physical development from their peers.

[10], [11],

Pathogenesis

The mechanism of the origin of idiocy is associated with a violation of the exchange of lipids and enchymnoses, which lead to the deposition in the central nervous system of lipid ganlioside. In healthy people, ganglioside is found in the gray matter of the brain. It has a complex structure, contains a lot of acids, glucose, sucrose and galactose. Pathogenesis is based on a chronogenic factor, that is, ontogenesis and lesion of the developing brain under the influence of various pathogenic factors.

About 75% of oligophrenia (a mild stage of idiocy) develops due to intrauterine lesions. In the presence of a family history, a malfunction in the work of the neural system, which leads to morphological changes in the brain, is observed. Such changes can cause small size and weight of the brain, delay in differentiation of convolutions and furrows, underdevelopment of frontal lobes.

Regardless of the severity of mental disorders, they have a similar clinical picture. First of all, this is a total underdevelopment of the psyche, problems with abstract thinking, impulsiveness, lack of independence, suggestibility, underdevelopment of emotions and problems with intellectual development.

[12], [13], [14], [15], [16], [17], [18], [19]

Symptoms of the idiocy

Idiocy is the most severe degree of oligophrenia. It is characterized by congenital or acquired at an early age pathologies of mental development, which increase with age and increase. The disease gives birth to itself from the first days of a child's life and manifests itself as a sharp lag in psychomotor development. Patients do not know how to speak and do not express mental activity, they are not able to master primitive skills.

Emotional background is not developed, the patients do not recognize even the closest people. The pathological condition is diagnosed on the basis of an early history and evaluation of the mental functioning of the body.

As a rule, people with a serious degree of mental disorders are allocated among the healthy. The signs of idiocy can be recognized from the first months of the child's life. The first thing that indicates a disease is a violation of the structure of internal organs. Patients from an early age lag behind in development, begin to hold their head late, sit and walk. Even having learned to move around, there is a lack of friendly limb movements.

The expression of the face, as a rule, is meaningless, sometimes a shade of joy or anger appears. Face puffy, thick tongue, awkward speech. Patients publish inarticulate sounds and syllables, repeating them non-stop. If the disease has a deep degree, then all types of sensitivity, including pain, are reduced. People do not distinguish between hot and cold, edible and inedible.

Idiocy is characterized by a predominance of instinctual life, for the patient there is a gluttony and open obstinate masturbation. Very often, there are signs of focal neuralgia. Some patients are dominated by lethargy and apathy, while others have angry and angry outbreaks. Children with pathology are not able to learn and with parental consent stay in specialized institutions. Even in the relatively adult age, patients with deep mental retardation are not able to serve themselves, so they need constant supervision and care.

[20], [21], [22], [23],

First signs

Symptoms of mental retardation appear even at an early age. The first signs depend on such factors as: systemic impairment of the intellect, organic diffuse lesions of the cerebral cortex, severity and irreversibility of the deviations. If at least one of these factors is not present, this indicates dysontogenesis, that is, problems with mental health, and not mental retardation.

The first signs of the disease:

• Inadequate or reduced response to others.
• Limited emotions, which manifest themselves as feelings of discontent or pleasure.
• There is no meaningful activity and self-service.
• Incontinence of urine and feces.
• Underdevelopment of locomotor and static functions.
• Lack of mobility, lethargy or monotonous motor excitement, primitive movements (swings of hands, swinging of the trunk).
• Episodic attacks of unmotivated aggression.
• Often observed perverted and increased cravings - eating impurities, masturbation.

Children with idiocy are not able to master meaningful activities, since the thinking process is broken. Patients do not perceive the speech of others, can not distinguish relatives from outsiders, do not master the skills of self-service. Therefore, they require constant care and supervision.

[24], [25], [26], [27]

Characteristic features of idiocy

Pathology makes itself felt during the first half of the life of the baby. The characteristic features of idiocy are the absence or weakness of reactions to others. The patient is unable to recognize people close to him, there is no expressive mimic activity. There is a little expression, a late smile. With age, the degree of motor disturbances is becoming more pronounced.

The disease is very often combined with pathologies and malformations of physical development. Most often these are limb defects (six fingers, fusion of fingers), spinal and cerebral hernias, defects of internal organs (cardiovascular system, genitourinary system, gastrointestinal tract).

The main clinical symptom in the older age is the underdevelopment of mental functions and the lack of speech. With patients, it is difficult to come into contact, since they do not manifest a reaction to external stimuli. Elementary mental operations are accessible to them, while mental development is at an unconditionally reflex level.

Attention when idiocy

Deep mental retardation is characterized by a complete or partial lack of speech and thinking. Attention to idiocy is relaxed, the patient can not concentrate on the simplest things. There is no reaction to external stimuli, instead of speaking, patients pronounce only sounds and do not perceive the speech addressed to them.

The attention of such people can not be attracted, they do not follow the objects, they are not protected when they try to harm them and do not listen to the sounds. Sensitivity is reduced, smell and hearing defects are possible. Intellectual backwardness affects perception, various cognitive processes and memory. Underdevelopment of emotions, motivations and motives makes life impossible in society.

[28],

Stages

Mental deficiency has a certain classification by which you can accurately determine the severity of the violations. Degrees of idiocy depend on the depth of brain damage. The pathological state has three stages: imbecility, debility, and idiocy. According to the international classification of mental diseases, there are 4 degrees of violations. They correlate with the characteristics of intellectual development.

The following degrees of idiocy stand out:

ICD 10		IQ	Alternative classification	Social Characteristics
Class	Degree of violations
F 70	Lightweight	70-50	Degeneracy	Teachable, able-bodied
F 71	Moderate	50-35	Imbecility	Unsound, incapacitated
F 72	Heavy	34-20
F 73	Deep	<20	Idiocy

With an easy degree, it is possible to learn basic skills of self-service. Moderate and severe degrees are characterized by significant developmental problems. Patients do not react to external stimuli, cognitive activity is completely absent, there are no self-service skills. There is a decrease in all types of sensitivity, primitive emotions, most often angry and malicious. The motor reactions are primitive and chaotic, speech is absent.

[29]

Deep Idiocy

For a severe degree of dementia, there is almost no cognitive activity. Deep idiocy has IQ below 20. Patients do not react to the environment, they do not pay attention even to pronounced stimuli. Speech and thinking are not developed, emotions are inadequate, meaningful activity and self-service are unavailable.

Deep idiocy has primitive coordination, motor reactions are awkward, inconsistent, repeated. There is a uniform monotonous swaying of the trunk, nodding movements and swinging hands. Emotions are weakly expressed, most often anger or satisfaction. Characteristic of the patients is instinctive behavior, gluttony. They need constant care, most often they are placed in specialized institutions, since their life in society is impossible.

[30]

Forms

There are two clinical forms of idiocy - excitable and torpid.

1. Excitable - the patients are in a state of stereotyped psychomotor agitation, that is, swing, make primitive movements and clap their hands.
2. Torpid - patients are immobile and presented to themselves.

Idiocy is classified according to several features, consider its main types:

• Amavrotic - this category includes hereditary diseases due to impaired ganglioside metabolism. The main symptomatology is associated with a progressive decline in intelligence and vision in the autosomal recessive type.
• Congenital - (Norman-Wood syndrome) - the first signs appear after birth, as a rule, it is seizures, hydrocephalus, disorders of neuropsychological development, hypotonia of the muscular system.
• Children's late (Bilshovsky-Yansky syndrome) - most often manifested at the age of 4-5 years. It is characterized by slow progression, increasing dementia, atrophy of the visual system, convulsions.
• Early childhood (Teya-Saks syndrome) - the symptoms appear on the first child of the child's life. Pathology has a progressive decline in vision, up to blindness, hyperacusis and delays in mental development.
• Late - manifests itself in adulthood. There are changes in the type of organic psychosyndrome, deafness, pigment retinitis, cerebellar disorders.
• Juvenile (Spielmeier-Vogt-Butten syndrome) - the first symptoms appear in 6-10 years. Patients with memory impairment, periodic motor disorders, decreased intelligence, vegetative endocrine disorders.
• Hydrocephalus - arises from the atrophy of the brain substance caused by the congenital edema of the brain.
• Dysostotic (gargoyilism) is a hereditary defect in connective tissue. It is expressed as a lesion of the joints, bones, central nervous system, internal organs.
• Xeroderma (De Sanctis-Kakkione syndrome) is a hereditary disease with various neurological disorders, pigment xeroderma and dementia of varying severity.
• Myxedema - occurs due to congenital thyroid dysfunction.
• Timic - develops due to hereditary pathology of the thymus gland function.
• Moral - this type of disease is the common name of mental illness. Has no pronounced violations of mental activity, but there are disorders of emotions, problems in communicating with others.

Idiot Thea-Saksa

The most serious pathology that occurs in 1-3% of children is the idiocy of the Saxon. The disease occurs as a result of the defeat of the cerebral membrane and manifests itself as a progressive mental retardation with pronounced motor defects. As a rule, up to six months of malaise does not make itself felt. But then irreversible dysfunction of the brain appears. It is this pathogenesis that causes high infant mortality up to 4-5 years.

The disease develops because of the accumulation of gangliosides in the cells of the nervous system. This substance controls the higher nervous activity. In a healthy organism, gangliosides are constantly synthesized, they are broken down. While in diseased children the process of cleavage is disturbed, which is due to the deficiency of the enzyme hexominidase type A. Pathology has an autosomal-recessive type of inheritance. That is, it occurs when inheriting two mutated genes from each parent. If such a gene exists only in one parent, then the child does not get sick, but in 50% of cases it is a carrier of pathology.

The pathological condition has two clinical forms associated with a deficiency of hexosaminidase type A:

• Chronic form - the first symptoms appear at the age of 3-5 years and grow to 12-14 years. The disease has an easy course, that is, motor disorders, intellect and verbal apparatus are insignificant.
• Juvenile form - manifested from the first weeks of the child's life. Rapidly progressing, characterized by a high risk of death even with timely therapy.

Thea Saksa has a paroxysmal course, that is, sudden outbreaks of abnormal cerebral activity. Such manifestations negatively affect the mental, motor and speech activity. Severity of seizures depends on the frequency of their appearance.

As a rule, the disease is diagnosed before the birth of the child. That is, during pregnancy the woman undergoes studies for the presence of various abnormalities. To do this, blood is taken from the placenta (chorionic biopsy) or a puncture of the fetal bladder is carried out followed by study of the amniotic fluid. If the suspicion of the disease appeared at an early age, then the child needs an ophthalmological examination. On the fundus there will be a reddish stain - a cluster of gangliosides on the cellulose.

Pathology refers to the incurable, but to facilitate its course, symptomatic therapy is used. This makes it easier for the child and parents. Prevention is based on the proper planning of pregnancy. Before you have a baby it is necessary to conduct genetic tests that will give information about the presence of hereditary pathologies, including mental deviations.

[31], [32], [33]

Amavrotic idiocy

Hereditary disease with a progressive course and pathologies of intellectual activity is an amaurotic idiot. The disorder has several forms with symptoms of varying severity. The peculiarity of the ailment is that it manifests itself at an early age and is not subject to treatment.

[34], [35], [36], [37], [38]

Complications and consequences

Children with mental disorders suffer not only from idiocy, but also from the pathologies that accompany it. The consequences and complications of the disease depend on the stage and degree of its severity. Most often, such patients have visual and hearing impairments, behavioral and emotional disorders, problems with the musculoskeletal system. Without proper therapy and rehabilitation of such defects, the patient's possibilities are substantially limited.

Idiocy is the last stage of dementia. Patients with such a disease can not adapt to the social environment, and their aggressive behavior is often combined with socially dangerous behavior. The pathological condition is accompanied by somatic disorders, frequent convulsive seizures. The harder the idiocy, the deeper its complications. Focusing on the etiology of dementia, the doctor makes a prediction on the most likely consequences.

[39], [40], [41], [42], [43],

Diagnostics of the idiocy

To identify diseases associated with mental or psychological disorders, many different methods are used. Diagnosis involves a comprehensive examination, which consists of:

1. Analysis of complaints and collection of family history.
   • At what age did the symptoms of pathology first appear (lack of motor activity, disturbance or absence of speech).
   • How was pregnancy carried out (the presence of infectious diseases, unbalanced nutrition, increased blood pressure, the use of alcohol and drugs).
   • Frequent colds in early childhood or malnutrition.
2. Psychiatric analysis of the patient's condition and consultation with a neurologist. The doctor evaluates his mental abilities, speech, the possibility of communication in society.
3. Computer and magnetic resonance imaging of the head. With the help of this study, it is possible to study the structure of the brain layer by layer and to reveal its damage (structural disorders, hemorrhages).

[44], [45], [46], [47], [48], [49], [50],

Differential diagnosis

Verification of idiocy with other forms of dementia, as a rule, does not present difficulties. This is due to the fact that the signs of the disease are characterized by deeply reduced intellectual and psychic abilities that appear in early childhood. Differential diagnosis begins with the collection of anamnesis and the study of the course of pregnancy in the mother. If during the period of gestation there were infectious diseases, the pathological influence of exogenous factors, inadequate nutrition or problems during the birth process, this increases the risk of idiocy confirmation.

Mental retardation must be differentiated with similar states:

• Pedagogical neglect - slowing of mental development is possible even for absolutely healthy people. Lack of conditions for proper development and lack of necessary information, cause degradation.
• Long-term asthenia - this is possible with severe somatic diseases or disorders of the functioning of the body due to malnutrition. The child has a delay in development, lack of attention, slow thinking and poor memory.
• Specific mental illnesses - for example, epilepsy or schizophrenia occur even in early childhood and without proper treatment provoke dementia.

There are in-depth clinical examinations with the definition of mental, physical, intellectual, speech and neurological disorders. Laboratory-instrumental diagnostics, genetic studies and psychophysiological testing are needed.

Treatment of the idiocy

Since idiocy has an innate, that is hereditary character, it is impossible to eliminate it. Treatment is a pathogenetic therapy: enzymopathy (inflammation of the enzyme deficiency) and endocrinopathy (hormonal correction of enzyme composition). With toxoplasmosis or congenital syphilis, specific therapy is performed. Symptomatic treatment consists of dehydration, sedation and restorative procedures.

Therapy is based on the cause of the pathological condition and symptoms:

• To improve metabolic processes, the patient is prescribed vitamin therapy, nootropics, glutamic acid and other drugs. 
• For the normalization of intracranial pressure, magnesium injections are administered, diacarb and glycerol are prescribed.
• With a strong inhibition, various stimulants are used, usually on a plant basis: Chinese magnolia vine, sydnocarb, ginseng.
• With increased excitement and epileptic seizures - neuroleptics and anticonvulsants.

In spite of the fact that idiocy is an incurable disease, drug treatment can alleviate its symptoms. In addition to taking medications and various physiotherapy procedures, the patient needs 24-hour care.

Prevention

In most cases, prevention is to protect people of childbearing age from factors that cause mutations at the genetic level. Primary prevention begins during pregnancy. A woman undergoes medical genetic counseling and regular diagnosis of fetal development.

Prevention of pathology consists of such procedures:

• Prevention of intrauterine infections
• Refusal to use pharmaceuticals with teratogenic effect
• Limitation of any contacts with surface active substances
• Correction of endocrine disorders in the period of gestation

The prevention of somatic lesions, traumatization of the brain and neurotoxicosis in children at an early age is mandatory.

[51], [52], [53],

Forecast

The outcome of dementia depends on its stage and form. The prognosis of idiocy is negative, since the disease is characterized by irreversible mental and mental disorders. Both children and adults with this pathology are incapable of independent life, they need constant care and supervision. Such patients are placed in specialized institutions, where they are provided with round-the-clock help.

The prognosis of mild to moderate stage of dementia depends on the treatment and preventive and rehabilitation measures taken. So, with oligophrenia in the stage of debility, adaptation in the society is possible, but imbecility, like idiocy, has an unfavorable outcome.

Lifespan

Idiocy is an innate pathology that does not respond to treatment. Life expectancy of patients with mild and moderate backwardness of about 50 years, with a deep form of lesion, people do not live up to 20 years.

If idiots are diagnosed in the first months of a child's life, then very often the life of the baby ends at the age of 4-5 years. Such a negative prognosis of survival is associated with significant biochemical and metabolic changes, immune system defects and abnormalities in the functioning of internal organs.