Heller syndrome (childhood dementia)

Heller’s syndrome is a mental disorder of pervasive development of children aged 2-10 years, in which the patient completely loses all formed skills and functions. At the same time, children stop talking, lose the ability to solve intellectual problems and perform self-service activities. Their character changes, becoming more irritable, hyperactive, naughty, anxious.

Geller’s syndrome is diagnosed during a conversation with a patient, in the course of observing his behavior, psychological testing of the child’s cognitive sphere. The problem is that there is currently no specific effective treatment. Therapy is reduced to symptomatic treatment with medications and various corrective exercises.

Forms and their manifestations

The symptoms of Heller’s syndrome are very specific. The prodromal period of the disease is characterized by the occurrence of emotional abnormalities – waywardness, anxiety, irritability, anger. The patient is no longer available for some complex activity, for which it is necessary to concentrate and distribute attention, show perseverance, and do something according to the proposed model. The child loses all previously acquired skills, he can no longer assemble a constructor, paint pictures, draw, play role-playing games, like playing in a store or hospital. Perseverance completely disappears, the child begins to get angry and refuse to take further actions at the slightest mistake. This period is dangerous because the intellectual decline at this stage is not noticeable, and it is not possible to diagnose Heller’s syndrome.

Within a few months of the onset of a process characterized by emotional instability and hyperactivity, the specific symptoms of Heller’s syndrome begin to develop. The child’s speech is depleted, vocabulary becomes scarce, sentences are built in a monosyllabic way. As a result, this leads to the disintegration of speech, the loss of spoken language, misunderstanding of the appeal. The child ceases to be interested in communicating with others, does not want to play games, an autistic disorder and isolation occur. His cognitive ability is lost, the emotional sphere is depleted, and indifference to everything arises.

The child’s motor skills also regress, they are replaced by stereotypical actions. The ability to wash, dress, eat, fold toys, go to the toilet disappears. Very often, the symptoms of Geller’s syndrome are complicated by neurological pathology, and after a year from the onset of the disease, the child completely loses all speech, everyday and social skills.

According to the dynamics of the course of the disease process, dementia can be residual (residual) and progressive. The residual type of dementia is characterized by single pathological effects on the brain, as a result of which psychological functions have already been impaired. But further destruction of brain activity does not occur. Dementia is the residual result of brain damage. At the same time, the child’s psyche is able to develop in the future, but only under existing conditions – a damaged brain and partially disintegrated functions. The child’s ability for cognitive activity is preserved, he will learn to communicate with people and perform certain functions, but at the level of his new capabilities.

Experts talk about progressive dementia when the process of decay of brain functions is not completed. Harmful factors provoking decay can be endogenous, genetically determined, or they can be exogenous in nature. Progressive dementia occurs as a consequence of chronic inflammation of the brain (for example, encephalitis or meningitis), progressive tumor processes in the brain, severe epilepsy, childhood schizophrenia, sclerosis of brain tissue, and various degenerative nervous diseases. At the same time, brain cells are constantly destroyed, which ultimately leads to the disintegration of psychological functions. This process continues until the complete collapse of the child’s psyche. In this case, mental development becomes impossible, only health workers should work with children with such a pathology, since psychological and pedagogical work in cases with progressive dementia does not make sense.

Etiology of Heller’s syndrome (childhood dementia)

The etiology of Heller’s syndrome has not yet been studied. Recent studies indicate the relationship of this disease with neurobiological mechanisms in the central nervous system. During encephalography, changes in the electrical activity of the brain were found in 50% of all sick children. Also, experts do not exclude the relationship of Geller’s syndrome with convulsions, Schilder’s disease, leukodystrophy. There is a hypothesis that this disease is of an infectious nature with persistent viruses that are so small that it is impossible to see them with a microscope.

The spasmodic development of children in the physical and mental plane does not make it possible to detect pathology in a timely manner. Sometimes a normally developing child suddenly takes a step back in his own development. This may indicate the onset of childhood disintegrative psychosis, a dangerous disease in which children after 3 years of age develop speech disorders, their ability to perceive what has been said decreases, activity in games disappears, control of the function of urination and stool disappears, uniformity in behavior occurs.

Most often, childhood dementia appears between the ages of 2 and 10 years. By the time differentiated symptoms appear, the child has already lost some (sometimes all) of the functions acquired earlier.

Features of the course and pathogenesis

The patterns of development of Geller’s syndrome have long been studied. Before the onset of the disease, from 2 to 10 years of normal development of the psyche and skills of the child pass, he fully or partially masters speech skills, is able to perceive and understand appeals to him, and uses social skills in life. The first symptoms of dementia break into the established course of the process of becoming a child’s personality – hyperactivity, a violation of the emotional sphere. From that moment on, over the course of 6-12 months, the level of intelligence sharply decreases, all previously acquired skills fall apart, control of natural needs is lost. After that, the regression stops with the stabilization of the achieved state. To start the process of developing lost skills with a child, it is necessary to carry out thorough and constant psychological and pedagogical work.

Damaged functions of the psyche are characterized not by the totality, but by the partiality of disorders.

Previously accumulated experience and knowledge can be partially preserved, but significant problems arise with the acquisition of new ones. If the defeat occurred at an early age, then all the skills of the child are greatly impoverished. Purposefulness can be violated in two ways – the occurrence of apathy, adynamia, loss of interest or lack of purposefulness of actions, disinhibition of motor activity. In the case of an early onset of dementia, there is an inhibition in the development of mental activity, children have a low level of reasoning, they are not able to interconnect objects and phenomena.

If the disease occurs at preschool age, then the child’s play activity is destroyed, it becomes monotonous and stereotypical. All acquired skills at this age suffer to a lesser extent, but their regression can be easily observed. If the disease occurred at the age of elementary school, then the child usually retains speech skills, the possibility of self-care, and even some degree of learning ability. The knowledge that exists at the time of the development of the disease can also be preserved, but intelligence and working capacity are sharply reduced, which leads to a drop in school interests and a disruption in the process of purposefulness in any kind of activity.

Diagnosis of Heller’s syndrome

At the very beginning of the diagnosis of Heller’s syndrome, the patient is consulted with a neurologist or pediatrician. The child’s parents are embarrassed by the gradual loss of some previously acquired functions, which leads them to the doctor. First, instrumental examinations and examinations are carried out to identify more common pathologies – epilepsy, brain injury, tumor. If these diagnoses are excluded, a psychiatric examination and specific diagnostics are necessary.

The specific diagnosis of Heller’s syndrome includes:

• conversation;
• patient monitoring;
• psychological testing.

The conversation is conducted, to a greater extent, with the parents of the child, since it is important for the doctor to find out the features during the course of the disease – how long the normal period of development lasted, how strongly the progress of the decay of functions manifests itself, is there regression in more than two areas, are there speech disorders, are there any failures in social, play, household or motor functions.

During consultations, the psychiatrist records the patient’s behavioral reactions and his emotional state. The hyperactivity characteristic of Heller’s syndrome, in this case, should be combined with manifestations of autism, such as stereotypy, loss of interest in communication, lack of facial expressions, pantomime, gestures, which demonstrates to the doctor the lack of tools for live communications.

By testing the intellectual abilities of the child, the specialist determines the depth of the defect that has arisen. Methods for these purposes vary, depending on the age of the patient and his ability to maintain productive contact. For testing purposes, Raven’s progressive matrix tests, Wechsler’s test, pyramid, box of forms are used. Geller’s syndrome must be distinguished from early childhood autism, Rett’s disease, and schizophrenia. The main criteria for diagnosing the disease are the period of normal development, the presence of symptoms that arose before 10 years, the speed of decay of skills (6-12 months). Also, the clinic of the disease suggests deficiency and impoverishment of all the functions of the child’s psyche.

Therapies

Therapeutic methods for dementia in childhood have similar directions with the treatment of early age autism. At the same time, the emphasis in treatment is on early intensive measures, a behavioral approach to the problem, and a clear structure. The effectiveness of drug therapy in this matter has not been scientifically proven, therefore, drugs are used only at the first stage of the disease to relieve behavioral symptoms. Each time the development program for the patient is drawn up on an individual basis with the involvement of psychologists, doctors of other specializations, teachers, parents in this process. A comprehensive therapeutic approach for Heller’s syndrome includes:

• corrective and developmental measures;
• psychotherapy and family counseling;
• social rehabilitation.

The development and adjustment of skills and functions implies special activities to restore speech activity and intelligence. Such classes are conducted by specialists in various fields – psychotherapists, speech therapists, speech pathologists, psychologists. The resumption of social skills occurs in group activities, where children with identical problems learn to interact and communicate with each other.

At the family counseling sessions, parents learn how to care for children with this disease, attend information sessions on the course of the disease, and get acquainted with the prognosis. Psychotherapeutic assistance is also provided to families in group classes, where meetings take place between families in which there are children with Geller syndrome, which reduces the social isolation of these families, allows you to get emotional support, practical experience in everyday matters and in the field of child development at home.

Social rehabilitation helps, through the efforts of specialists, to form the necessary practical skills in a child. The child will be taught to dress, wash, use a fork and spoon, hold a pencil, sculpt from plasticine. In parallel with this, emotional and behavioral deviations are corrected, concentration of attention and perseverance develop.

What is the danger of dementia for a child and methods of its prevention

Usually, after the intensive development of the disease, there is a period of stability. The risk of somatic complications and mental progressive deviations cease to be significant, but social adaptation for the child remains impossible. Children with Geller syndrome must receive specialized training. In ordinary schools and technical schools, such people will not be able to study, they cannot master a profession, and they cannot start a family. Children with this disease develop very slowly and require constant careful care from the outside. With a favorable course of the disease, care can gradually be replaced by control of the patient’s actions. Such a disease in the family leads to the impossibility of full-fledged social functioning of its other members (parents), so most of them refuse their profession and hobbies.

The prognosis for children with Heller’s syndrome is generally unfavorable, since it is almost impossible to completely restore lost skills. Sometimes it is only possible to slowly resume some of them. With intensive care in the early periods of the disease, it is possible to restore only up to 20% of speech skills, self-service functions, work, household and social skills. It is impossible to prevent the onset of childhood dementia, since the real causes of this disease are unknown today, and therefore there are no ways to solve this problem and prevent it.

Parenting and teaching children with dementia

Science is called upon to educate and educate children with Geller’s syndrome – oligophrenopedagogy, which corrects all manifestations of mental retardation in a child. The education of such children should be carried out in special educational institutions. At the same time, the goal of educating children at preschool age with this pathology is the harmonization of personality development (intellectual, physical and spiritual), the reduction of dependence on outsiders, the formation of independence and vitality. The main direction of teaching children with intellectual disabilities is their maximum social adaptation to life in the environment.

The parents of children with Geller’s syndrome, including in adulthood, are subject to huge “burdens” associated with the constant care and control of the patient. The upbringing and education of patients with this disease (in modern medical language with “autism spectrum disorders”) is assigned to special correctional teachers, and also, to a greater extent, to parents. According to the law, children with such a disease must be trained according to a special program, which is indicated in Federal Law No. 273-FZ of December 29.12.2012, XNUMX.

Education for children with such problems is organized in the form of special classes in groups and individually with speech therapy, psychomotor, sensory orientation, as well as with a bias in the field of physical therapy. All training is carried out in auxiliary schools, where the first department trains patients with a mild form of the disease under the age of 9 years, the second – more severe patients. Severe forms of the disease do not allow children to study in such schools; Centers for correctional and developmental education and rehabilitation are provided for them.

Education affects all spheres of the usual educational process, but in a volume determined for each degree of severity of the pathology. The correctional part of the curriculum is designed to correct the speech and intellectual activity of the child. In elementary grades, children are taught only their native language. In preparation for school, classes are built in a playful way. In the upper grades, at least a quarter of the entire educational process is devoted to vocational training.

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