Intellectual Disability: Clinical Guidelines

List of Abbreviations

AEDs — anti-epileptic drugs (N03A antiepileptics)

VKK — Medical Consultative Commission

VNVLP – Vital and Essential Drugs for medical use

CT – computed tomography

ICD-10 – International Classification of Diseases 10th Revision

MRI — magnetic resonance imaging

MRO – medical and rehabilitation department of the psychoneurological dispensary

PND – psychoneurological dispensary

RCT – randomized controlled trials

ultrasound – ultrasound examination

FGOS – Federal State Educational Standard

EchoEG – echoencephalography

EEG – electroencephalography

IQ – intelligence quotient

ID – intellectual disability

GPP – good practice point (GPP)

Terms and Definitions

Intellectual disability is a heterogeneous group of conditions caused by congenital or early-acquired mental underdevelopment with pronounced insufficiency of intelligence (cognitive, speech, and social abilities), accompanied by impaired adaptive behaviour that makes adequate social functioning of the individual difficult or completely impossible. 

Mental underdevelopment is a set of etiologically diverse hereditary, congenital, or early-acquired persistent non-progressive syndromes of general mental retardation, manifesting in difficulty with social adaptation primarily due to a predominant intellectual defect.

Oligophrenia – the concept of "oligophrenia" has a narrower meaning, referring to the developmental formation in a child of a defect limited to the intellectual sphere

1. Brief information on the disease or condition

1.1 Definition of the disease or condition

According to ICD-10, intellectual disability is a condition of arrested or incomplete development of mind, which is especially characterised by impairment of skills manifested during the developmental period, contributing to the overall level of intelligence, i.e., cognitive, language, motor, and social abilities [11, 22]. Intellectual disability can develop with any other mental or somatic disorder, or occur without one. However, individuals with intellectual disability may exhibit the full range of mental disorders, the frequency of which among them is at least 3–4 times higher than in the general population. Adaptive behaviour is always impaired, but in protected social environments that provide support, this impairment in patients with mild intellectual disability may not be apparent at all.

1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)

The diversity of pathogenic factors and the lack of a single etiology of intellectual disability make it difficult to assess causal relationships in the origin of intellectual disability. In most cases, environmental and hereditary factors act in a complex interaction and unity. However, there are also pathogenetic links common to all forms of intellectual underdevelopment that lead to similar clinical manifestations of the intellectual disability syndrome, among which a particularly important role belongs to the chronogenic factor, i.e., the period of ontogenesis in which damage to the developing brain occurs. Various pathogenic influences, both genetic and exogenous, acting during the same period, can cause identical clinical manifestations, while the same etiological factor acting at different stages of ontogenesis can lead to different consequences [5, 12].

In intellectual disability, which represents a polymorphic group of pathological conditions, a wide variety of clinical and psychopathological disorders is noted. Clinically differentiated and clinically undifferentiated forms of intellectual disability are distinguished.

The group of clinically differentiated intellectual disability includes independent diseases with a specific clinical, psychopathological, and somatoneurological picture, for which mental underdevelopment is one of the symptoms, usually the most severe one. More often, these are genetically determined disorders or clinically delineated syndromes with characteristic somatoneurological manifestations whose etiology is not yet clear [9, 10].

Various forms of intellectual disability differ significantly from each other in their pathogenetic mechanisms. However, there are also common links of pathogenesis,

About 75% of intellectual disability is caused by brain damage during the prenatal period. The impaired formation of organs and systems most often occurs during critical stages of their development. All prenatal developmental anomalies are subdivided into blastopathies, caused by damage to the embryo during the period of blastogenesis (up to 4 weeks of pregnancy); embryopathies, i.e., damage during the period of embryogenesis (from 4 weeks to 4 months of pregnancy); and fetopathies, i.e., damage to the fetus from 4 months until the end of pregnancy.

1.3 Epidemiology of the Disease or Condition

Global prevalence rates of intellectual disability, according to official medical reporting, vary widely: from 3.04 to 24.6 and higher per thousand population. According to the World Health Organization, as well as a number of domestic and foreign authors, the prevalence of intellectual disability in the population ranges from 1% to 3% [5]. In regions exposed to ecopathogenic factors and in isolated social groups, the prevalence of this pathology can reach 7% [5]. Intellectual disability occurs approximately 1.5 times more frequently in males than in females. This ratio is most noticeable in mild mental underdevelopment, whereas in severe degrees of intellectual disability, as a rule, there is no quantitative difference between boys and girls. According to Rosstat, as of 2022, there are about 1.5 million people with intellectual disabilities living in Russia. Of these, about 700,000 are children under the age of 18. Thus, the proportion of people with intellectual disability is about 1% of the country's total population [5], [48].

1.4 Features of Coding the Disease or Condition

In accordance with ICD-10, intellectual disabilities belong to Chapter VII [11].

Mild intellectual disability (F70):

F70.0 – Mild intellectual disability, stating no or minimal impairment of behavior;

F70.1 – Mild intellectual disability, significant impairment of behavior requiring attention and treatment;

F70.8 – Mild intellectual disability, other behavioral impairments

F70.9 Mild intellectual disability, without mention of impairment of behaviour

Moderate intellectual disability (F71):

F71.0 Moderate intellectual disability, with statement of no or minimal impairment of behaviour

F71.1 Moderate intellectual disability, significant impairment of behaviour requiring attention or treatment

F71.8 Moderate intellectual disability, other impairments of behaviour

F71.9 Moderate intellectual disability, without mention of impairment of behaviour

Severe intellectual disability (F72):

F72.0 Severe intellectual disability, with statement of no or minimal impairment of behaviour

F72.1 Severe intellectual disability, significant impairment of behaviour requiring attention or treatment

F72.8 Severe intellectual disability, other impairments of behaviour

F72.9 Severe intellectual disability, without mention of impairment of behaviour

Profound intellectual disability (F73):

F73.0 – Profound intellectual disability, stating no or minimal impairment of behavior;

F73.1 Profound intellectual disability, significant impairment of behaviour requiring attention or treatment

F73.8 Profound intellectual disability, other impairments of behaviour

F73.9 Profound intellectual disability, without mention of impairment of behaviour

Other intellectual disabilities (F78)

F78.0 Other intellectual disabilities, with statement of no or minimal impairment of behaviour

F78.1 Other intellectual disabilities, significant impairment of behaviour requiring attention or treatment

F78.8 Other intellectual disabilities, other impairments of behaviour

F78.9 Other intellectual disabilities, without mention of impairment of behaviour

Unspecified intellectual disability (F79)

F79.0 Unspecified intellectual disability, with statement of no or minimal impairment of behaviour

F79.1 Unspecified intellectual disability, significant impairment of behaviour requiring attention or treatment

F79.8 Unspecified intellectual disability, other impairments of behaviour

F79.9 – Unspecified intellectual disability, without mention of behavioral impairment

1.5 Classification of the disease or condition

The systematics of forms of intellectual disability includes classifications based on the severity of the intellectual defect, morphological classifications, classifications based on psychological and clinical signs, etiological classifications, and clinical-physiological classifications.

An important variant of typology from a clinical standpoint is the clinical-physiological classification of mental underdevelopment proposed by S.S. Mnukhin and subsequently supplemented by D.N. Isaev [5]. According to this classification, intellectual disability is subdivided into four forms: asthenic, atonic, sthenic, and dysphoric.

1.6 Clinical presentation of the disease or condition

The characteristic clinical features of intellectual disability are, first of all, the predominance of intellectual insufficiency over the defect of other mental functions and the absence of progression in the main symptoms of the disease. It should be noted that a progressive course is possible in certain forms of intellectual disability. For example, in phenylketonuria, progressive dynamics of the disease are observed in the first years of life [5].

As a rule, intellectual disability represents a manifestation of dysontogenesis of the brain and the entire organism as a whole. In patients with intellectual disability, the highest forms of thinking—the processes of generalization and abstraction—are impaired. The thinking of these patients is at a developmental stage where the establishment of particular, strictly concrete connections is paramount in cognitive activity, while the highest forms of abstract thinking are absent. The inability to engage in conceptual thinking is most clearly manifested in the "object classification" experiment: the isolation of species and generic concepts, and the operation with the most general features, are practically inaccessible to these patients. The insufficiency of the process of abstraction is also manifested in the inability to understand the figurative meaning of proverbs, sayings, and metaphors.

The inability to engage in abstract thinking, characteristic of intellectual disability, is also reflected in the features of attention, perception, and memory. Attention in patients with intellectual disability is difficult to attract, poorly fixed, and characterized by easy distractibility. Perception is relatively poor and inadequately reflects the surrounding environment. Memory is also deficient: everything new is assimilated extremely slowly and only after repeated repetition. While in some variants of intellectual disability, rote memory can be quite satisfactory, semantic memory is always impaired. Thus, when reproducing read text, the patient cannot convey the main meaning in their own words, but strives to recount everything verbatim.

In close connection with the underdevelopment of the highest forms of cognitive activity is the underdevelopment of the patients' speech, which manifests primarily in the delayed stages of its development. The degree of speech underdevelopment in most cases correlates with the severity of the intellectual defect. Both the development of active speech and its comprehension suffer. There is a limited vocabulary, speech impediments, the passive vocabulary significantly exceeds the active one, speech is often inexpressive, phrases are short, quite often agrammatic, and incorrect usage of words according to meaning is characteristic.

Emotions are characterized by inertia. For these patients, only immediate experiences are relevant; they are incapable of responding to joy and sorrow that may occur in the future. Low emotional differentiation, monotony, the absence of subtle shades of experience, and a deficient ability to suppress their emotional expressions are typical. At the same time, the emotional sphere is noticeably better preserved compared to the depth of the intellectual defect. Thus, even with pronounced intellectual disability, manifestations of sympathy and the desire to help others can often be noted; such patients may experience a sense of hurt, shame for their wrongdoing, and a feeling of deep attachment. Their temperament and character are also quite diverse. Among them, affectionate and obedient, malicious and aggressive, or sullen and gloomy subjects can be observed; alongside calm and balanced individuals, one can see sluggish, apathetic, or overly active and fussy ones.

Disturbances in the volitional sphere of patients with intellectual disability are highly dependent on the level of intellectual underdevelopment. The more pronounced the intellectual defect, the less motivated the patient's actions are, and the more frequently they are determined solely by momentary desires (emotions) or drives. There are always signs of impulsivity in their activity; actions are committed without the struggle of motives that takes place in healthy people.

Mild intellectual disability is generally characterized by relatively good rote memory. In some cases, these patients are able to acquire a significant stock of knowledge and elementary skills in reading, writing, and arithmetic. Sometimes a good rote memory and a relatively rich vocabulary can mask the weakness of mental activity and the inability to form abstract concepts. These patients do not show independence and initiative in work (study); the main difficulty is the transition to mental tasks, the solution of which requires replacing the habitual mode of activity with a new and yet unknown one. The emotional and volitional aspects of the personality in mild intellectual disability are quite diverse, but the common features are an insufficient ability for self-control and suppression of drives, impulsivity against the background of increased suggestibility and a tendency toward imitation. With proper upbringing and education, the implementation of psychological and pedagogical correction with the instilling of labor skills in the patients, they can master a number of unskilled professions, primarily manual labor, and show good everyday adaptation to life. Thus, the majority of these patients are trainable (special education school program) and can subsequently live independently. The optimal age range for the final diagnosis of mild intellectual disability is 6 to 8 years. In ICD-10, when characterizing this group of patients, it is noted that, overall, the behavioral, emotional, and social disorders in the majority of individuals with mild intellectual disability, and the resulting need for therapy and support, much more closely resemble the problems of people with a normal level of intelligence than the specific problems of patients with deeper degrees of mental underdevelopment.

Patients with moderate intellectual disability are able to accumulate some store of knowledge, and mechanical memory is sufficiently developed in many of them. Such patients also acquire speech, although their vocabulary is small, speech is agrammatic, and the comprehension and use of speech are limited. In mild intellectual disability, self-awareness is more or less developed. Many of these patients are egocentric, demand increased attention, and show joy at positive evaluations of their actions and resentment when scolded. When conversing with these patients, one can notice that to questions requiring effort, they answer "I can't", "I don't know", "I don't want to". Special educational programs can provide certain opportunities for acquiring a number of basic skills and abilities, but the volume of material assimilated is extremely low. Given sufficient motor development, patients with moderate intellectual disability can master simple types of labor, but their work is mechanical in nature; they are not able to modify their activity according to a changing environment. As a rule, these patients are not adapted to independent living and require constant guardianship and care. The optimal age range for the definitive diagnosis of moderate intellectual disability is from 3 to 5 years.

The clinical picture of severe intellectual disability is similar, first of all, to the symptomatology of moderate disability. There is a low level of social functioning, which manifests as the almost complete inability to master elementary school knowledge, household skills, and abilities. Usually, these patients need guardianship and constant care throughout their lives. Unlike patients with moderate intellectual disability, these patients exhibit pronounced motor disorders, which also hinders even their simple social adaptation.

Patients suffering from profound intellectual disability are characterized by a sharp decrease in reaction to the environment. They either do not react at all to everything happening around them or give inadequate reactions, responding to any stimulus without lingering on any of them. Articulate speech is absent; in speech addressed to them, patients perceive intonation but do not understand the meaning. Left to themselves, many of them remain immobile, while others are in purposeless constant motion, crawl, grab objects, can be aggressive, or exhibit auto-aggression. States of motor excitation occur periodically and often without any apparent cause. Manifestations of pleasure or displeasure are primitive. Somatic well-being and the sensation of satiety lead to complacency and calmness, while the feeling of hunger or other unpleasant sensations are expressed by cries with an expression of displeasure, agitation, or other similar manifestations. In response to the unknown, fear and crying may be observed. Perverted or altered drives are often noted: bulimia or perversions of eating drives, hypersexuality, for example, accompanied by persistent masturbation, etc. The profound underdevelopment of all mental activity correlates with various physical development defects, including gross ones: dysplastia, skull deformations, malformations, and developmental anomalies. Neurological symptoms, including paresis and paralysis, are frequently observed. The optimal age for diagnosing profound intellectual disability, especially from the perspective of prognosis and the social consequences of the disease, is the first months (the first year) of life. In any case, profound intellectual disability should be diagnosed before the age of 3.

2. Diagnostics, medical indications and contraindications for diagnostic methods

Establishing a diagnosis of mild intellectual disability is in most cases advisable by the ages of 6 and 8. In a number of obvious cases, in the absence of significant shifts in the clinical picture against the background of adequate medical and pedagogical assistance, the diagnosis of intellectual disability is established at 3–4 years. Diagnostics is carried out by a child psychiatrist based on the clinical picture characteristic of intellectual disability and the developmental history—a combination of symptoms of intellectual defect, impairments and the degree of preservation of perception, memory, attention, thinking, the emotional-volitional sphere, and the degree of adaptation to the environment [5]. When examining a child to make or confirm a diagnosis, it is required to assess the dynamics of mental underdevelopment associated with age and the treatment and rehabilitation measures carried out.

Criteria for establishing the diagnosis/condition:

1. anamnestic data,

2. laboratory data,

3. instrumental diagnostic data (differential diagnosis),

4. data from other diagnostic methods.

2.1 Complaints and Anamnesis

Comments: The following require an examination to establish, confirm, or rule out a diagnosis of intellectual disability: (1) children referred to territorial medical, psychological, and pedagogical commissions to determine their educational pathway; (2) children referred to medical and social evaluation for disability status determination; (3) adolescents referred to military medical boards to determine fitness for military service in the Armed Forces of the Russian Federation based on mental health status. Diagnosing intellectual disability should not be limited to merely establishing the fact of general mental underdevelopment; therefore, the goal is to formulate a comprehensive diagnosis that primarily reflects: an assessment of the severity and characteristics of the intellectual defect, along with the clinical and psychological profile of the defect structure. The presence of comorbid disorders, etiological factors, degree of environmental adaptation, and socio-psychological factors contributing to the clinical presentation of the disease and its social adaptation. Intellectual disability may develop alongside any other mental or somatic disorder, or occur without them. However, individuals with intellectual disabilities may exhibit the full range of mental disorders, the frequency of which among them is at least 3–4 times higher than in the general population [5, 7, 11, 18].

Comments: As a group, children with neurodevelopmental disorders have poorer health and face greater barriers in accessing primary healthcare than the general population. Due to the high prevalence of coexisting physical and mental disorders, children with intellectual disability require more attention from medical staff and greater healthcare resources than children and adolescents in the general population. Without adequate primary healthcare, health problems in patients with intellectual disability often go unnoticed.

2.2 Physical Examination

Comment: During a physical examination, it is important to assess neurological symptoms (a proper neurological examination) and the presence of potential somatic abnormalities, malformations, and dysmorphic stigmata. In some cases, the identified somatic symptoms help clarify the timing of a harmful exposure and the presence of somatic abnormalities specific to certain differentiated forms of intellectual disability.

A general physical examination includes: checking for potential somatic abnormalities and malformations (eye defects, heart defects, polydactyly, deafness, limb shortening, as well as hepatosplenomegaly), dysmorphic stigmata (abnormal shape of the auricles, high ["gothic"] palate, low hairline, hypertelorism, dental malocclusion), identifying signs of various injuries, examining organ and system status, and reviewing the history of somatic diseases. The neurological examination evaluates: cranial nerve dysfunction, reflexes and their changes, extrapyramidal disorders, cerebellar pathology and coordination disturbances, sensory function and its impairments, and autonomic nervous system dysfunction.

2.3 Laboratory Diagnostics

Comment: Genetic testing is performed by a geneticist upon referral from a child psychiatrist to determine the genetic nature of clinically indistinguishable states of intellectual underdevelopment, to establish the true cause of the disability when both hereditary and exogenous origins are suspected, to determine whether an autosomal dominant inherited anomaly is the result of a de novo mutation or reduced penetrance of a gene that was previously unmanifested in relatives, and to forecast the likelihood of transmitting the pathology to future children when one or both parents have a congenital anomaly or severe illness.

2.4 Instrumental Diagnostics

Most pediatric patients with intellectual disability exhibit various structural changes in the brain and sometimes in the skull bones. Instrumental examinations make it possible to clarify these changes and, in some cases, are also used in the differential diagnosis of intellectual disability.

Comment: Skull radiography in one or more projections allows for the detection of bone hypertrophy, as seen, for example, in Hurler syndrome. Premature suture closure may also be identified. Intrauterine calcifications are detected in toxoplasmosis, Sturge-Weber syndrome, tuberous sclerosis, and idiopathic hypoparathyroidism. Radiography is of particular importance for clarifying the diagnosis of hydrocephalus, which presents with increased skull size, bone thinning, and suture diastasis. Performing skull radiography in one or more projections avoids the need for routine brain MRI or CT in all patients.

2.5 Other Procedures

Comments: In clinical practice, Wechsler test scores are most frequently used to determine disability status. Full-Scale IQ scores from intelligence tests should be regularly used in diagnosing intellectual disability, except when the validity of the Full-Scale IQ score is in doubt; in such cases, an appropriate subscale score may be used instead. An intelligence test result indicating intellectual disability is two or more standard deviations (SD) below the mean (e.g., a score of 70 or below, where the mean = 100 and standard deviation = 15). Substantial and significant discrepancies between the scores of corresponding Wechsler scales may compromise the validity of the Full-Scale IQ score. In such situations, appropriate subscale scores may better reflect the individual's true overall cognitive functioning level. It is an error to establish a diagnosis of intellectual disability (including during various evaluations) solely based on numerical intelligence quotient figures (usually obtained via Wechsler testing). In all cases, the diagnosis must be established by a child psychiatrist based on clinical data, with any psychological testing serving as supplemental rather than definitive diagnostic tools.

3. Treatment, including drug and non-drug therapies, diet therapy, and pain management

Intellectual disability in children and adolescents may coexist with any psychiatric and neuropsychiatric disorders, the treatment of which is described in the relevant clinical guidelines. For the treatment of frequent comorbid disorders—such as anxiety, fears, and obsessive-compulsive states—please refer to the corresponding clinical guidelines.

To treat intellectual disability (or rather, its accompanying and comorbid disorders), other psychostimulants and nootropic drugs (ATX code: N06BX) are used—substances of various structures capable of normalizing metabolism, dilating cerebral blood vessels, increasing energy reserves, and enhancing oxygenation of brain cells. Thanks to these properties, they increase the brain's resistance to harmful influences, stimulate mental development, improve thinking, memory, and attention, accelerate the learning process, and reduce the severity of symptoms complicating intellectual disability. If it is possible to influence the etiological factors of intellectual disability, appropriate treatment of the underlying condition is carried out. Undoubtedly, etiological treatment of intellectual disability is effective only if irreversible brain damage has not yet occurred in the patient as a result of a particular etiological factor.

In most cases of intellectual disability, symptomatic treatment is administered.

The main goals of treatment for intellectual disability in children and adolescents are the reduction or elimination of comorbid behavioral disorders. Depending on the leading cause of the development or intensification of behavioral disorders, the structure of therapeutic measures shifts towards predominantly pharmacotherapy or strengthening the psychotherapeutic component of comprehensive treatment. The combined use of drug and non-drug treatment methods, in combination with occupational therapy and social work with the family and the patient, is one of the fundamental principles of managing behavioral disorders in individuals with intellectual disability.

The adequacy of therapeutic intervention depends on a correct assessment of the ratio of causal factors, determining the specific weight of biological and socio-psychological components that dictate the stage-by-stage orientation toward a particular intervention—pharmacological or correctional-pedagogical. The correction of behavioral disorders should be carried out, whenever possible, in an outpatient setting.

Psychopharmacotherapy is not the primary treatment method and should be carried out strictly according to clinical indications and for a limited period of time. Its use is justified only in cases of severe and uncorrectable forms of behavioral disorders, in the presence of a "biological soil" predisposing to behavioral disorders, as well as in the presence of concurrent pronounced emotional and somatovegetative disorders, to relieve affective tension in the initial stages of correctional and pedagogical work [3,5,12,23].

Comment: hopantenic acid possesses neurometabolic, neuroprotective, and neurotrophic properties [12], exerts a stimulating effect on the central nervous system against the background of cerebral insufficiency of exogenous-organic origin, improves performance, activates mental activity, reduces motor excitability, organizes behavior, and has an anticonvulsant effect. In Russian clinical practice, it is used for intellectual disability, delayed mental and speech development, cerebasthenic syndrome; for the correction of side effects of antipsychotics, in epilepsy (as part of combination therapy), and for residual manifestations of past neuroinfections, post-vaccination encephalitis, and traumatic brain injury.

Comments: cerebrolysin** is a drug with proven neuron-specific neurotrophic activity, similar to the action of natural neuronal growth factors, but manifested, unlike them, under conditions of peripheral administration.

Level of evidence – 2, Grade of recommendation - B

Comment: As a rule, #risperidone** and other atypical antipsychotics (other antipsychotic agents) in intellectual disability are needed only if the children's behavior is dangerous to themselves or others, or if there is a risk that they will not be admitted to a school classroom or will be hospitalized due to behavioral problems. In this case, #risperidone** is used to make the child calmer. The use of the drug for this purpose has been approved only for children with autism, but a large number of studies suggest that it is effective in reducing persistent behavioral problems across various

#Chlorpromazine** in children aged 7 years 7 months and older was used in a randomized study at doses of 25 mg/day daily for 60 days [56]. No later studies have been found.

GCR CLEL 3 Grade of recommendation – C, level of evidence – 3.

Comment: #chlorpromazine** is most effective in treating the most severe dysphoric form of intellectual disability during puberty, characterized by a persistent capricious and irritable mood with tantrums, fights, swearing, and destructive actions. This is usually accompanied by overt sexuality and self-injurious tendencies.

Comment: haloperidol** is an antipsychotic drug belonging to the butyrophenone derivatives. It has a pronounced antipsychotic and antiemetic effect. The administration of haloperidol** in children is associated with a significant reduction in behavioral symptoms and serves as a powerful therapeutic agent. The most frequent side effects include extrapyramidal disorders of varying severity and parkinsonism. Most patients experience transient akinetic-rigid syndrome, oculogyric crises, akathisia, and dystonic phenomena.

Comment: periciazine** is a neuroleptic (antipsychotic agent) with a pronounced sedative profile. It reduces aggressiveness, excitability, and disinhibition, making it effective for behavioral problems. It exerts a distinct sedative effect. Attention should be paid to the occurrence of extrapyramidal disorders and arterial hypotension.

Comment: The drug has antipsychotic and sedative effects. Adverse reactions: Central nervous system: psychomotor retardation, mild extrapyramidal symptoms, increased fatigue, and dizziness may occur.

Comment: The drug has weak antipsychotic, sedative, and hypnotic effects. Adverse reactions: Central nervous system: psychomotor retardation, mild extrapyramidal symptoms, increased fatigue, and dizziness may occur.

Comment: The drug has weak antipsychotic, sedative, and hypnotic effects. Adverse reactions: Central nervous system: psychomotor retardation, mild extrapyramidal symptoms, increased fatigue, and dizziness may occur.

Comment: Patients receiving the drug must be reminded that if any signs and symptoms of agranulocytosis or an infectious disease (e.g., fever, weakness, lethargy, or sore throat) appear, they should immediately contact their attending physician.

Comment: Simultaneous use with non-selective MAO inhibitors and selective MAO-A inhibitors is contraindicated.

3.1. Other treatment

Psychotherapy in this population is fundamentally no different from psychotherapy in individuals with average intelligence and is similar to the treatment of children, as in both cases methods and the therapist must adapt to the patient's developmental needs. Treatment should be determined by the patient's needs and responses rather than the therapist's theoretical orientation. Indications include: the presence of anxiety and conflicts, especially regarding oneself; impaired interpersonal communication skills; or other psychiatric disorders known to improve with psychotherapy. Prerequisites include communication skills that allow meaningful exchange of information with the therapist, the ability to develop even minimal relationships, and the presence of a trained, experienced, and unbiased therapist who is comfortable working in a team [55].

4. Medical Rehabilitation and Sanatorium-Resort Treatment

The goal of rehabilitation is to improve the quality of life and social functioning of patients with intellectual disabilities by overcoming their social alienation, increasing their level of social functioning and sustainable community adaptation, and choosing optimal pathways to develop their most preserved functions. The main tasks of rehabilitation include increasing patients' social competence through the development of communication skills, coping mechanisms for stress, and work activities; reducing discrimination and stigma; supporting families, creating and maintaining long-term social support, and meeting at least basic needs such as housing, employment, leisure organization, establishing a social network (circle of friends), and increasing patient autonomy (independence).

In general, we are talking about habilitation measures rather than rehabilitation ones. In particular, D.N. Isaev [5] recommended this specific term, stating that in intellectual disability we are not initially restoring function (rehabilitation) but rather performing habilitation measures, that is, working with an existing defect. The success of habilitation measures depends, of course, on the severity of the intellectual disability, as well as comorbid psychopathological or neurological disorders that may complicate habilitation.

Comment: Social and psychological rehabilitation and habilitation measures help create conditions for individuals with intellectual disabilities to develop an understanding of their own identity, foster cognitive, communicative, and creative abilities, improve motor coordination, and overcome behavioral and emotional disorders.

Comment: Children and adolescents with intellectual disabilities represent one of the most socially vulnerable categories. Most parents of disabled adolescents have managed to "work through" their grief and accept the child as they are, but they worry about how the child will live after their passing. Forms of work with parents may include: seminars structured around a specific program that include both theoretical aspects and practical parts; training sessions to maintain psychological health and a favorable family climate in order to constructively influence the individual with a disability; and parent support groups to establish friendly contacts and build additional inter-family connections [13].

5. Prevention and Dispensary Observation

For adolescents with intellectual disabilities, secondary and tertiary prevention measures are advisable. People with intellectual disabilities have complex health problems, some of which differ from those of the general population. Adequate primary health care is essential to identify these problems and prevent morbidity and premature mortality. Physical, behavioral, and mental health issues must be addressed, and primary health care providers must be especially attentive to the interplay of biological, psychological, and social factors affecting health, as these interactions can easily be overlooked in adolescents with intellectual disabilities.

Comment: Children and adolescents with intellectual disabilities have numerous undetected and often obvious health problems; therefore, a comprehensive assessment at the primary health care level facilitates the detection and subsequent treatment of somatic pathology.

Comment: In accordance with Articles 26 and 27 of the Law of the Russian Federation "On Psychiatric Care and Guarantees of Citizens' Rights in Its Provision," outpatient psychiatric care for individuals with intellectual disabilities is provided, depending on medical indications, in the form of consultative and treatment care or dispensary observation. Consultative and treatment care is provided by a psychiatrist upon the independent request of the individual suffering from a mental disorder, at their request or with their consent, and in respect of a minor under the age of 15, at the request or with the consent of their parents or other legal representative. Dispensary observation may be established regardless of the consent of the person suffering from a mental disorder or their legal representative in cases provided for by part one of Article 27 of this Law (dispensary observation may be established for a person suffering from a chronic and protracted mental disorder with severe, persistent, or frequently exacerbating morbid symptoms) and involves monitoring the person's mental health status through regular examinations by a psychiatrist and the provision of necessary medical and social assistance.

6. Organization of Medical Care

The standards of specialized medical care for children and adolescents with intellectual disabilities regulate the types of diagnostic and therapeutic measures, including the number and frequency of specialist examinations, types and nature of examinations, tests, and therapeutic procedures [16,17].

Indications for hospital admission.

Mentally retarded children and adolescents may exhibit the entire range of psychiatric disorders, the frequency of which among them is at least 3-4 times higher than in the general population. In addition, individuals with intellectual disabilities are more frequently victims of physical and sexual abuse. Adaptive behavior is invariably impaired, but in protected social environments with provided support, this impairment in children and adolescents with mild intellectual disability may not be overtly apparent. A direct indication for inpatient treatment is forms of intellectual disability complicated by psychotic symptoms (catatonic, hallucinatory-paranoid, manic, depressive, etc.). Non-psychotic behavioral disorders are not an indication for inpatient treatment.

Indications for patient discharge from a medical organization:

Discharge of patients from a psychiatric inpatient unit or day hospital is recommended after the successful completion of psychopharmacological treatment adjustment and stabilization of their condition. It is recommended to initiate rehabilitation techniques already at the inpatient/day-care stage once acute psychotic symptoms subside.

7. Additional Information

Adolescents with intellectual disabilities represent an extremely heterogeneous group with entirely different rehabilitation potentials.

Residents of social care institutions. Approximately 2/3 of such patients are transferred to the psychoneurological dispensary district from children's residential institutions upon reaching the age of 18. Such children are raised in specialized orphanages for mentally retarded children, designed

to provide inpatient social services to mentally retarded children

who have partially or completely lost the ability for self-care, require constant outside care, and need living conditions appropriate to their age and health status, as well as for organizing feasible work activities, recreation, and leisure. These are children who were abandoned by their parents, or removed from dysfunctional families at a very early age with a family history of alcoholism and drug addiction, representing the most vulnerable group. As a rule, these children graduate from 9 grades of a special school (Federal State Educational Standard Type 1), and are offered a choice of further vocational training (basket weaving, bookbinding, shoemaking, dressmaking). They rarely master these professions due to a lack of motivation. As orphans, they receive social housing from the state and disappear from the sight of psychiatrists, ceasing to visit the psychoneurological dispensary. For many of them, the connection with the dispensary is lost and only restored when investigative authorities send inquiries regarding some of them either as perpetrators of offenses or as victims of fraud and housing schemes. During forensic psychiatric examinations, offenders with mild intellectual disability are recognized as sane and end up in correctional facilities. There are differences in diagnostic approaches to this category of patients within the systems of the Ministry of Health and the Ministry of Labor and Social Protection. During examinations, some patients' Wechsler test scores turn out to be at borderline levels or at the lower limit of the age norm. At the same time, diagnostic practice fails to take into account the level of adaptive functioning.

Patients raised at home. The group of patients with F70 mild intellectual disability is more stable. In childhood, loving and socially responsible parents of these patients work with them additionally, take them to extracurricular classes, and hire tutors, so upon reaching 18 years of age, parents show an interest in their further socialization. This interest is manifested both in establishing disability status and in acquiring a trade. Unfortunately, only a few complete their training and acquire a specialty. Often they slip out of sight: they get married, change their place of residence, etc. Other patients who failed to socialize actively attend medical rehabilitation departments: they participate in workshops and art therapy. During periods of age-related crises, or as part of a reaction to psychotrauma, they may exhibit rudimentary psychotic symptoms, and in some cases develop a full-blown acute psychosis. Despite the statement in ICD-10 that mentally retarded individuals may experience the entire range of psychiatric disorders, the indications for the use of medications for psychoses in children with intellectual disabilities are not reflected in the instructions for medical use of the drugs. Any medication, whether an antipsychotic prescribed as a behavioral corrector or an antiepileptic drug used as mood-stabilizing therapy, requires a multi-page justification through the Medical Advisory Commission (VKK), which significantly complicates the treatment process.

Groups F72-73, severe and profound intellectual disability. All patients in this category have comorbid pathology. They are examined at home 1-2 times a year. All have disability group 1. A number of patients receive psychotropic medications, which must be justified through the Medical Advisory Commission (VKK) every time.

Criteria for Evaluating the Quality of Medical Care

References

Appendix A1. Working Group Composition

Appendix A2. Methodology for Developing Clinical Guidelines

Target audience of these clinical guidelines:

Specialists with higher medical education in the following specialties:

1. Psychiatry (psychiatrists, district psychiatrists) Specialists with higher professional (non-medical) education:

2. Medical psychologists.

Table 1. Scale for grading levels of evidence (LE) for diagnostic methods (diagnostic interventions)

Table 2. Scale for assessing levels of evidence certainty (LEC) for methods of prevention, treatment, and medical rehabilitation, including those based on natural therapeutic factors (preventive, therapeutic, and rehabilitative interventions)

Table 3. Scale for grading levels of recommendation (LoR) for prevention, diagnostic, treatment, and medical rehabilitation methods, including those based on natural therapeutic factors (preventive, diagnostic, therapeutic, and rehabilitative interventions)

Appendix A3. Reference Materials

• International Statistical Classification of Diseases and Related Health Problems, 10th Revision (ICD-10)

• Criteria for Evaluating the Quality of Medical Care

• Order of the Ministry of Health of the Russian Federation No. 203n dated May 10, 2017, "On Approval of Criteria for Assessing the Quality of Medical Care."

• Order of the Russian Ministry of Health No. 205n dated May 2, 2023 (as amended on December 4, 2023) "On approval of the Nomenclature of Positions of Medical and Pharmaceutical Workers" (Registered with the Ministry of Justice of Russia on June 1, 2023, No. 73664).

• Federal Law No. 323-FZ dated November 21, 2011, "On the Basics of Protecting the Health of Citizens in the Russian Federation" (Collected Legislation of the Russian Federation, 2011, No. 48, Art. 6724);

• Order of the Russian Ministry of Health No. 103n dated February 28, 2019, "On approval of the procedure and timelines for the development of clinical guidelines, their revision, the standard template of clinical guidelines, and requirements for their structure, composition, and scientific validity of the information included in the clinical guidelines."

• Order of the Russian Ministry of Health No. 804n dated October 13, 2017, "On approval of the nomenclature of medical services."

• Order of the Ministry of Health and Social Development of the Russian Federation "On the Procedure for the Use of Medicinal Products in Patients on Vital Indications" No. 494 dated August 9, 2005.

Appendix B. Physician Action Algorithms

Appendix C. Patient Information

Parents or legal representatives of a patient with intellectual disability need to be explained in an accessible form the nature, manifestations, diagnostic methods, and intervention strategies for this condition. Intellectual disability represents a group of conditions caused by congenital or early-acquired arrested or incomplete mental development with prominent deficits in cognitive abilities and adaptive behavior, making adequate social functioning of the individual difficult or entirely impossible. There is no direct treatment for intellectual disability. However, individuals with intellectual disability may exhibit the full range of mental disorders, the frequency of which is 3–4 times higher than in the general population. Recommended pharmacotherapy is aimed at halting these disorders. Evidence-based methods with proven efficacy and safety include continuous habilitation based on behavioral, psychological, and educational approaches. The efficiency of social functioning is ensured by vocational habilitation and training in simple occupations (shoemaking, sewing, bookbinding, etc.). Medical habilitation measures are carried out in medical rehabilitation departments of psychoneurological dispensaries, and for individuals with disabilities, in institutions of the Ministry of Labour and Social Protection.

Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools

Appendix G1

Title in Russian: Wechsler Intelligence Scale for Children (WISC)

Original title: Wechsler Intelligence Scale for Children, WISC

Source (official developer website):

Wechsler Intelligence Scale: Diagnostics of the Structure of Intelligence (Children's Version): Methodological Guide / Yu.I. Filimonenko, V.I. Timofeev. - Saint Petersburg: IMATON, 2016. - 106 p. - (IMATON. Professional Psychological Tools) [20]

www.imaton.com

type: rating scale

Purpose: investigation of the structure of intelligence in children aged 5 to 16 years.

Source (official developer website, validation publication): Wechsler D. Williams&Wilkins; Baltimore: 1939.

Type (underline):

• rating scale

• index

• questionnaire

• other (specify):

Purpose: the technique is designed to assess basic intellectual functions that ensure successful behavior in various situations and effectiveness in various types of activities.

Content: full methodology [20]

Key: all scoring tables [20]

Note: the test is distributed by IMATON: https://imaton.com/catalog/test-vekslera-detskii-variant/

Appendix G2

Russian title: Raven's Progressive Matrices

Original title: The Raven Progressive Matrices

Source (official developer website):

Raven J., Raven J.C., Court J.H. Manual for Raven's Progressive Matrices and Vocabulary Scales. Section 3. Standard Progressive Matrices (including Parallel and Plus versions). — "Cogito-Center". — M., 2012. [15].

www.imaton.com

Purpose: designed to differentiate subjects by their level of intellectual development

Source (official developer website, validation publication): Raven, J. (1981). Manual for Raven's Progressive Matrices and Vocabulary Scales. Research Supplement No.1: The 1979 British Standardisation of the Standard Progressive Matrices and Mill Hill Vocabulary Scales, Together With Comparative Data From Earlier Studies in the UK, US, Canada, Germany and Ireland. San Antonio, Texas: Harcourt Assessment

Raven J., Raven J.C., Court J.H. Manual for Raven's Progressive Matrices and Vocabulary Scales. Section 3. Standard Progressive Matrices (including Parallel and Plus versions). — "Cogito-Center". — M., 2012.

Type (underline):

• rating scale

• index

• questionnaire

• other (specify):

Purpose: designed to measure the general intelligence factor (Spearman's g factor). J. Raven's test evaluates the ability to perceive abstract forms, identify their relationships, and make logical decisions in the shortest possible time.

Content: full methodology [15]

Key: all scoring tables [15]

Note: the test is distributed by IMATON: https://imaton.com/catalog/buklet_cvetnye_progressivnye_matricy_ravena_klassicheskaya_forma_serii_a_ab_b/