Idiocy

The extreme degree of mental retardation is idiocy. To alleviate symptoms, pathogenetic, i.e. hormonal, anti-infective, and symptomatic treatment (neuroleptics, sedatives, nootropics) is used.

Let us consider the pathogenesis and etiology of the disease, its types and degrees of disorders, methods of diagnosis, treatment and prevention.

Epidemiology

The prevalence of dementia has not been sufficiently studied. According to the World Health Organization, about 3-5% of cases of severe mental retardation have been registered. A large percentage of pathology is observed in areas with the impact of ecopathogenic factors.

The epidemiology of the pathological disorder indicates that the disease is more common in men than in women. This ratio is more often observed in mild oligophrenia. In severe forms, i.e. idiocy, there is no significant difference between women and men.

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

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Causes idiocies

Mental development 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, Angelman syndrome) associated with abnormal chromosomes and gene structure, signs of physical retardation.
2. Harmful factors that affect the female body during pregnancy and negatively affect the development of the fetus:
   • Ionizing radiation.
   • Various chemical agents (household toxins, drugs, poisons, alcohol).
   • Infectious diseases (rubella, cytomegalovirus infection, toxoplasmosis, syphilis).
   • Iodine deficiency, which is necessary for the normal development of the nervous system.
   • Improper nutrition or malnutrition (deficiency of vitamins and minerals has a pathological effect on the development of the fetus).
3. Child brain injury, birth trauma (intracranial hemorrhage, compression of the child's head with obstetric forceps), asphyxia.
4. Iodine deficiency in the diet of a child at an early age (from birth to 3-4 years).

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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 social and 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 retardation and other pathologies increases significantly if a woman does not give up bad habits during pregnancy. Smoking, alcoholism, drug addiction have a negative impact on the development of the fetus at the moment when its body is being laid down. Children born to such mothers are susceptible to various diseases. Most often, these are diseases of the respiratory, endocrine and cardiovascular systems, congenital pathologies. Premature pregnancy is another factor that increases the possibility of the birth of a baby with defects. Such children may lag behind their peers in both intellectual and physical development.

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Pathogenesis

The mechanism of idiocy origin is associated with the disruption of lipid metabolism and enchymnoses, which lead to the deposition of the lipid ganglioside in the central nervous system. In healthy people, ganglioside is located in the gray matter of the brain. It has a complex structure, contains many acids, glucose, sucrose and galactose. Pathogenesis is based on a chronogenic factor, i.e. ontogenesis and damage to the developing brain under the influence of various pathogenic factors.

About 75% of oligophrenia (mild idiocy) develops due to intrauterine damage. If there is a family history, there is a malfunction in the neural system, which leads to morphological changes in the brain. Such changes can cause small size and weight of the brain, delays in differentiation of convolutions and furrows, underdevelopment of the frontal lobes.

Regardless of the degree of expression 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.

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Symptoms idiocies

Idiocy is the most severe form of oligophrenia. It is characterized by congenital or early acquired pathologies of mental development, which intensify and increase with age. The disease makes itself known from the first days of a child's life and manifests itself as a sharp lag in psychomotor development. Patients cannot speak and do not express mental activity; they are unable to master primitive skills.

The emotional background is not developed, patients do not recognize even the closest people. The pathological condition is diagnosed based on early anamnesis and assessment of the mental functioning of the body.

As a rule, people with severe mental disorders stand out among healthy people. Signs of idiocy can be recognized from the first months of a child's life. The first thing that indicates the disease is a violation of the structure of internal organs. Patients lag behind in development from an early age, begin to hold their head, sit and walk late. Even after learning to move, there is a lack of coordinated movements of the limbs.

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

Idiocy is characterized by the predominance of instinctive life, the patient is noted for gluttony and open persistent masturbation. Very often there are signs of focal neuralgia. In some patients, lethargy and apathy dominate, while in others, angry and spiteful outbursts. Children with pathology are unable to learn and, with parental consent, are placed in specialized institutions. Even at a relatively adult age, patients with profound mental retardation are unable to take care of themselves, so they need constant supervision and care.

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First signs

Symptoms of mental retardation appear at an early age. The first signs depend on factors such as: systemic impairment of intelligence, organic diffuse lesions of the cerebral cortex, severity and irreversibility of deviations. If at least one of these factors is missing, then 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 that manifest as feelings of discontent or pleasure.
• There is a lack of meaningful activity and self-care.
• Incontinence of urine and feces.
• Underdevelopment of locomotor and static functions.
• Low mobility, lethargy or monotonous motor agitation, primitive movements (arm swings, body swaying).
• Episodic attacks of unmotivated aggression.
• Perverted and heightened desires are often observed – eating filth, masturbation.

Children with idiocy are unable to master meaningful activity, since the thinking process is impaired. Patients do not perceive the speech of others, cannot distinguish loved ones from strangers, and do not master self-care skills. Therefore, they require constant care and supervision.

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Characteristics of idiocy

The pathology makes itself known during the first six months of the baby's life. 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 facial activity. There is an inexpressive look, a late smile. With growing up, the degree of motor disorders becomes more pronounced.

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

The main clinical symptom in old age is underdevelopment of mental functions and lack of speech. It is difficult to establish contact with patients, as they do not show reactions to external stimuli. They have access to elementary mental operations, while mental development is at an unconditional reflex level.

Attention to idiocy

Severe mental retardation is characterized by a complete or partial absence of speech and thinking. Attention in idiocy is unfocused, the patient cannot concentrate on the simplest things. There is no reaction to external stimuli, instead of speech, patients only make sounds and do not perceive the speech addressed to them.

It is impossible to attract the attention of such people, they do not follow objects, do not defend themselves when someone tries to harm them, and do not listen to sounds. Sensitivity is reduced, defects in smell and hearing are possible. Intellectual retardation affects perception, various cognitive processes, and memory. Underdevelopment of emotions, impulses, and motives makes life in society impossible.

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Stages

Mental deficiencies have a certain classification with the help of which it is possible to accurately determine the severity of disorders. The degrees of idiocy depend on the depth of damage to the brain. The pathological condition has three stages: imbecility, debility and idiocy. According to the international classification of mental illnesses, there are 4 degrees of disorders. They are correlated with the characteristics of intellectual development.

The following degrees of idiocy are distinguished:

ICD 10		IQ	Alternative classification	Social characteristics
Class	Degree of violations
F 70	Easy	70-50	Moron	Trainable, employable
F 71	Moderate	50-35	Imbecility	Unable to learn, unable to work
F 72	Heavy	34-20
F 73	Deep	<20	Idiocy

In mild cases, it is possible to learn basic self-care skills. Moderate and severe cases are characterized by significant developmental problems. Patients do not respond to external stimuli, cognitive activity is completely absent, and there are no self-care skills. There is a decrease in all types of sensitivity, primitive emotions, and anger and malice are most often manifested. Motor reactions are primitive and chaotic, and speech is absent.

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Profound idiocy

Severe dementia is characterized by a virtually complete absence of cognitive activity. Deep idiocy has an IQ below 20. Patients do not react to their surroundings, they do not pay attention even to clearly expressed irritants. Speech and thinking are not developed, emotions are inadequate, meaningful activity and self-care are not available.

Deep idiocy has primitive coordination, motor reactions are awkward, uncoordinated, repetitive. Uniform monotonous swaying of the body, nodding movements and arm swings are noted. Emotions are weakly expressed, most often it is anger or satisfaction. Patients are characterized by instinctive behavior, gluttony. They need constant care, most often they are placed in specialized institutions, since their life in society is impossible.

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Forms

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

1. Excitable - patients are in a state of stereotypical psychomotor agitation, that is, they sway, make primitive movements and clap their hands.
2. Torpid - patients are motionless and left to their own devices.

Idiocy is classified according to several other characteristics; let's look at its main types:

• Amaurotic - this category includes hereditary diseases caused by disorders of ganglioside metabolism. The main symptoms are associated with a progressive decrease in intelligence and vision in an autosomal recessive manner.
• Congenital – (Norman-Wood syndrome) – the first signs appear after birth, as a rule, these are convulsions, hydrocephalus, disorders of neuropsychic development, hypotonia of the muscular system.
• Late childhood (Bielschowsky-Jansky syndrome) – most often manifests itself at the age of 4-5 years. Characterized by slow progression, increasing dementia, atrophy of the visual system, convulsions.
• Early childhood (Tay-Sachs syndrome) - symptoms appear in the first year of a child's life. The pathology has a progressive decrease in vision, up to blindness, hyperacusis and delays in mental development.
• Late – manifests itself in adulthood. Changes of the organic psychosyndrome type, deafness, pigment retinitis, cerebellar disorders are observed.
• Juvenile (Spielmeyer-Vogt-Batten syndrome) – the first symptoms appear at 6-10 years of age. Patients have memory impairment, periodic movement disorders, decreased intelligence, vegetative-endocrine disorders.
• Hydrocephalic - occurs due to atrophy of the brain tissue caused by congenital hydrocephalus.
• Dysostotic (gargoilism) is a hereditary defect of connective tissue. It is expressed as damage to joints, bones, central nervous system, internal organs.
• Xeroderma (De Sanctis-Cacchione syndrome) is a hereditary disease with various neurological disorders, pigmented xeroderma and dementia of varying severity.
• Myxedema - occurs due to congenital dysfunction of the thyroid gland.
• Thymic – develops due to hereditary pathology of the thymus gland function.
• Moral - this type of illness is a general name for mental illnesses. It does not have pronounced mental disorders, but there are emotional disorders, problems in communicating with others.

The idiocy of Tay-Sachs

The most serious pathology, which occurs in 1-3% of children, is Tay-Sachs idiocy. The disease occurs as a result of damage to the meninges and manifests itself as progressive mental retardation with pronounced motor defects. As a rule, the malaise does not make itself known until six months. But then irreversible disorders of brain function appear. It is this pathogenesis that causes high child mortality up to 4-5 years.

The disease develops due to the accumulation of gangliosides in the cells of the nervous system. This substance controls higher nervous activity. In a healthy organism, gangliosides are constantly synthesized and broken down. While in sick children, the process of breaking down is impaired, which is associated with a deficiency of the enzyme hexominidase type A. The pathology has an autosomal recessive type of inheritance. That is, it occurs when two altered genes are inherited from each parent. If only one parent has such a gene, the child does not get sick, but in 50% of cases is a carrier of the pathology.

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

• Chronic form – the first symptoms appear at the age of 3-5 years and increase until 12-14 years. The disease has a mild course, that is, the impairment of motor skills, intelligence and speech apparatus are insignificant.
• Juvenile form – manifests itself in the first weeks of a child’s life. It progresses rapidly and is characterized by a high risk of death even with timely therapy.

Thea Sachs has a paroxysmal course, that is, sudden bursts of abnormal brain activity. Such manifestations negatively affect mental, motor and speech activity. The severity of seizures depends on the frequency of their occurrence.

As a rule, the disease is diagnosed before the child is born. That is, during pregnancy, a woman undergoes tests for various abnormalities. For this, blood is taken from the placenta (chorion biopsy) or a puncture of the amniotic sac is performed with subsequent examination of the amniotic fluid. If suspicions of the disease appeared at an early age, then the child needs an ophthalmological examination. There will be a reddish spot on the fundus - a cluster of gangliosides on the tissue.

The pathology is incurable, but symptomatic therapy is used to alleviate its course. This makes life easier for the child and parents. Prevention is based on proper pregnancy planning. Before having a baby, it is necessary to conduct genetic tests that will provide information about the presence of hereditary pathologies, including mental disabilities.

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Amaurotic idiocy

A hereditary disease with a progressive course and pathologies of intellectual activity is amaurotic idiocy. 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.

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Complications and consequences

Children with mental retardation 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 capabilities are significantly limited.

Idiocy is an extreme stage of dementia. Patients with this disease cannot adapt to the social environment, and their aggressive behavior is often combined with socially dangerous actions. The pathological condition is accompanied by somatic disorders, frequent seizures. The more severe the idiocy, the deeper its complications. Based on the etiology of dementia, the doctor makes a prognosis for the most likely consequences.

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Diagnostics idiocies

Many different methods are used to identify diseases associated with mental or psychological disorders. Diagnostics involves a comprehensive examination, which consists of:

1. Analysis of complaints and collection of family history.
   • At what age did symptoms of the pathology first appear (lack of motor activity, speech impairment or absence).
   • How the pregnancy proceeded (presence of infectious diseases, unbalanced diet, high blood pressure, 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, and ability to communicate in society.
3. Computer and magnetic resonance imaging of the head. This examination allows for a layer-by-layer study of the brain structure and the detection of its damage (structural abnormalities, hemorrhages).

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Differential diagnosis

Verification of idiocy with other forms of feeblemindedness is usually not difficult. This is due to the fact that the signs of the disease are characterized by deeply reduced intellectual and mental abilities that appear in early childhood. Differential diagnostics begins with collecting anamnesis and studying the course of pregnancy in the mother. If there were infectious diseases, pathological influence of exogenous factors, poor nutrition or problems during the birth process during gestation, this increases the risk of confirming idiocy.

Mental retardation must be differentiated from similar conditions:

• Pedagogical neglect – slowing down of mental development is possible even in absolutely healthy people. The absence of conditions for proper development and the deficit of necessary information cause degradation.
• Long-term asthenia - this is possible with severe somatic diseases or dysfunctions of the body due to improper nutrition. The child has a developmental delay, absent-mindedness, slow thinking and poor memory.
• Progressive mental illnesses, such as epilepsy or schizophrenia, arise in early childhood and, without proper treatment, lead to dementia.

In-depth clinical examinations are conducted to determine mental, physical, intellectual, speech and neurological disorders. Laboratory and instrumental diagnostics, genetic research and psychophysiological testing are necessary.

Treatment idiocies

Since idiocy is congenital, i.e. hereditary, it is impossible to eliminate it. Treatment is pathogenetic therapy: enzymopathy (inflammation of enzyme deficiency) and endocrinopathy (hormonal correction of enzyme composition). Specific therapy is carried out for toxoplasmosis or congenital syphilis. Symptomatic treatment consists of dehydration, sedative and general strengthening 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.
• To normalize intracranial pressure, magnesium injections are administered, and diacarb and glycerin are prescribed.
• In cases of severe inhibition, various stimulants are used, usually plant-based: Chinese magnolia vine, sidnocarb, ginseng.
• In case of increased agitation and epileptic seizures – neuroleptics and anticonvulsants.

Despite 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 round-the-clock care.

Prevention

In most cases, prevention consists of protecting people of childbearing age from factors that cause mutations at the genetic level. Primary prevention begins during pregnancy. The woman undergoes medical genetic counseling and regular diagnostics of fetal development.

Prevention of pathology consists of the following procedures:

• Prevention of intrauterine infections
• Refusal to use pharmaceuticals with teratogenic effects
• Limit any contact with surfactants
• Correction of endocrine disorders during pregnancy

Prevention of somatic lesions, brain trauma and neurotoxicosis in children at an early age is mandatory.

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Forecast

The outcome of dementia depends on its stage and form. The prognosis for idiocy is negative, as the disease is characterized by irreversible disorders of mental and psychic activity. Both children and adults with this pathology are incapable of independent living and require constant care and supervision. Such patients are placed in specialized institutions where they receive round-the-clock care.

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

Life expectancy

Idiocy is a congenital pathology that cannot be treated. The life expectancy of patients with mild and moderate retardation is about 50 years, with a deep form of the lesion, people do not live to 20 years.

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