ABSTRACT

This summary provides an overview of the assessment and treatment recommendations contained int he Practice Parameters for the Assessment and Treatment of Children, Adolescents and Adults with Mental Retardation and Comorbid Mental Disorders. The parameters were written to aid clinicians in the assessment and treatment of children, adolescents and adults with symptoms of mental retardation and comorbid mental disorders. Mental retardation (MR) is a heterogeneous condition defined by significantly sub-average intellectual and adaptive functioning and onset before age 18 years. With an approach underscored by principles of normalization and the availability of appropriate education and habilitation, persons with MR generally live, are educated, and work in the community. Mental disorders occur more commonly in persons with MR than in the general population. However, the disorders themselves are essentially the same. Clinical presentations can be modified by poor language skills and by life circumstances, so a diagnosis might hinge more heavily upon observable behavioral symptoms. The diagnostic assessment considers and synthesizes the biological, psychological and psychosocial context of mental disorders. Comprehensive treatment integrating various approaches, including family counseling, pharmacological, educational, habilitative, and milieu interventions is the rule. Key Words: dual diagnosis, developmental disabilities, mental illness, psychiatry, mental health, practice parameters, guidelines, children, adolescents, adults.

Principle Authors: Ludwik Szymanski, M.D., and Bryan H. King, M.D. This summary was developed by the Work Group on Quality Issues: William Bernet, M.D., Chair, John E. Dunne, former Chair, Maureen Adair, M.D., Valerie Arnold, M.D., Joe Beitchman, M.D., R. Scott Benson, M.D., Oscar Bukstein, M.D., Joan Kinlan, M.D., Jon McClellan, M.D., David Rue, M.D., and Jon Shaw, M.D. AACAP Staff: Elizabeth Sloan, L.P.C., and Kristin Kroeger.

This summary and the full text of the Practice Parameter for the Assessment and Treatment of Children, Adolescents, and Adults with Mental Retardation and Comorbid Mental Disorders is available to Academy members on the World Wide Web (www.aacap.org) and appears in the [1999] supplement of the JAACAP. The full text of these parameters was reviewed at the 1998 Annual Meeting of the American Academy of Child and Adolescent Psychiatry. Both the full text and this Summary were approved by the AACAP Council on June 25, 1999.

Reprint requests to AACAP Publications Department, 3615 Wisconsin Ave., NW, Washington, DC 20016.

© [year] by the American Academy of Child and Adolescent Psychiatry.

These parameters give the clinician direction in diagnosing and treating children, adolescents, and adults with mental retardation and comorbid mental disorders. Recommendations are based on extensive review of the scientific literature and clinical consensus among experts in the subject. These parameters are not intended to define the standard of care; nor should they be deemed inclusive of al proper methods of care or exclusive of other methods of care directed at obtaining the desired results. The ultimate judgement regarding the care of a particular patient must be made by the clinician in light of all the circumstances presented by the patient and his or her family, the diagnostic and treatment options available, and available resources. The literature review, including references, and the rationale for specific recommendations are contained in the complete document (American Academy of Child and Adolescent Psychiatry, 1999).

INTRODUCTION

In the past several decades, the patterns of service delivery for persons with MR have changed dramatically. Presently, an overwhelming majority live in the community and are expected to use community-based medical services. Yet barriers to successful community living exist. Mental disorders are more prevalent in persons with MR than in the general population and are a primary reason for failure to adapt to community living. Further, relatively few mental health clinicians receive training specific to the needs of this population. The goal of these parameters is to aid clinicians who are called upon to provide mental health services to persons who have mental disorders comorbid with MR.

The primary focus of these practice parameters is on children, adolescents, and young adults up to 21 to 22 years of age, an upper age limit of eligibility for public special education and related services in some states. Issues related to older individuals with MR are also highlighted to provide an understanding of the natural history of MR.

Mental retardation is not a single entity, but includes a heterogeneous group of individuals with a broad spectrum of levels of functioning, disabilities, and strengths. Therefore, these parameters are general in nature, and have to be adapted to a particular person's needs.

These parameters consist of two major sections:
Part I: Review of general knowledge on MR. Mental health clinicians usually are not the primary professionals responsible for the diagnosis and treatment of MR. However, the understanding of the biological basis and nature of the MR, its natural history, and the availability of and entitlement for services, is necessary for every mental health clinician working with these individuals.
Part II: Mental disorders comorbid with MR. This section describes the modifications of psychiatric diagnostic and treatment techniques that may be necessary for patients with significant cognitive impairment. It also details some variations in the clinical presentation of major mental disorders in this population and the principles of psychiatric interventions including general treatment and habilitation programs.

EXECUTIVE SUMMARY

GENERAL ASPECTS OF MENTAL RETARDATION

The diagnosis of mental retardation requires the finding of significantly subaverage current intellectual functioning, for example, IQ below 70-75 on a standardized, individually administered test; significant impairment in present adaptive functioning; and onset prior to age 18. Its presence is noted on Axis II in the multiaxial classification scheme. MR is not a single disorder but a heterogeneous condition defined by a person's functioning. Its prevalence is estimated at about 1% of the population, and about 85% of those with this condition have IQs within the mild mental retardation range. In about 35% a genetic causation is found, and in less than 10%, a malformation syndrome of unknown origin may be identified. External, prenatal, perinatal or postnatal factors including infections, trauma, toxins, delivery problems, and prematurity account for about one third. In the remainder the etiology is unknown.

The cause of the mental retardation should be identified if possible, as it may clarify the prognosis, sometimes suggest treatment, or alert the clinician to possible medical and behavioral complications that may be more common in certain conditions.

Assessment of MR

The assessment of a person with mental retardation is typically multidisciplinary. Increasingly, developmental disorders clinics are established in which psychologists may perform cognitive assessment, developmental pediatricians and clinical geneticists may complete physical diagnostic evaluations, and psychiatrists and behavioral psychologists may assess psychological and behavioral function. University Affiliated Programs (Appendix A) have been established throughout the U.S. to bring together such expertise, but available resources will influence the roles of various clinicians in the evaluation process. The comprehensive assessment includes:

• Diagnosis of mental retardation using standardized intelligence testing and evaluation of adaptive skills through testing or clinical evaluation.
• Biomedical evaluation, including family, pregnancy, perinatal, developmental, health, social, and educational history; physical and neurodevelopmental examination; and laboratory tests. Laboratory tests are usually indicated by the findings in the history and physical examination and may include chromosomal analysis (including fragile-X by DNA analysis); brain imaging (CT scan, MRI); EEG; urinary amino-acids; blood organic acids and lead level; appropriate biochemical tests for inborn errors of metabolism.
• Assessment of psychological and behavioral functioning.

Habilitation and Treatment of Persons with MR

The habilitation of persons with MR is based on the principles of normalization and community based care, with additional supports as needed. Federal legislation, for example, the Individuals with Disabilities Education Act (IDEA), entitles disabled children and adolescents to a full range of diagnostic, educational and support services from birth to age 21. Specialized treatments are also provided if necessary, as is done for persons with severe visual and auditory impairment. Additional entitlements may be provided by state laws. The parents of children and adolescents with MR are entitled by these laws to receive support services and to be active participants in treatment planning. Some parents and older patients are not aware of their rights to obtain services. The clinician has an important role in such instances to educate and, if needed, to refer to a "patient advocate" or "educational advocate." In recent practice, children and adolescents are educated in special classes in regular school or in inclusionary programs (in age appropriate regular classes, with additional supports as needed). In the United States, children with MR are now rarely if ever placed in residential institutions and separate schools. Adults with MR of all levels live in the community, in settings varying from their own apartments with supports as needed, to small (4-8 residents) group homes. They are employed in specialized settings or, increasingly, in the competitive job market. Habilitation and treatment include:

• Specific treatment of the underlying condition, if known, to prevent or to minimize brain insults that result in MR (e.g., shunting in the case of hydrocephalus).
• Early intervention, education, and ancilliary therapies (such as physical, occupational, and language therapies), family support, and other services, as needed.
• Treatment of comorbid physical conditions, such as hypothyroidism, congenital cataracts or heart defects in children with Down syndrome, treatment of seizures in persons with tuberous sclerosis, etc. Treatment of comorbid mental disorders.

ASSESSMENT OF MENTAL ILLNESS IN PERSONS WITH MR

Mental illness is frequently comorbid with mental retardation, with most prevalence estimates ranging from 30% to 70%. Virtually all categories of mental disorders have been reported in this population. An accurate psychiatric diagnosis provides the foundation for understanding the patient and for treatment planning.

The psychiatric diagnostic evaluation of persons who have MR is in principle the same as for persons who do not have retardation. The diagnostic approaches are modified, depending on the patient's cognitive level and especially communication skills. For persons who have mild MR and good verbal skills the approach does not differ much from diagnosing persons with average cognitive skills. The poorer the communication skills, the more one has to depend on information provided by caregivers familiar with the patient and on direct behavioral observations.

The assessment includes: Comprehensive History
The history taken from the patient and from several caregivers in different settings covers:

• Presenting symptoms include concrete descriptions of specific behaviors in various situations and settings, their change over time, antecedent events and the way the various caregivers handle them.
• Psychiatric review of systems includes premorbid and current behavioral and personality patterns, adaptive functioning, self care, communication, and social functioning.
• Details of previous psychiatric treatment, with particular emphasis on medication side-effects that could cause the presenting symptoms.
• Past and present educational, habilitative, work programs and living situation: their quality, consistency and appropriateness; availability of supportive services; long term plans for the patient's care.
• Parents'/caregivers' attitudes to the patient, their understanding of his/her disability, support for growth vs. overprotection.
• Review of past cognitive tests and evaluations, or request for new ones if needed.

Patient Interview

• Ample time must be allotted for the patient interview which typically takes longer than with patients without MR. Sufficient time is needed to put the patient at ease.
• The verbal examination should be adapted to the patient's communication skills and should use clear and concrete language, structure, reassurance, and support. Leading questions and questions requiring yes or no answers should be avoided and the interviewer should ensure that questions are understood.
• Patients with sensory impairments like blindness or deafness must be approached in a manner that recognizes their needs through the use of appropriate interpreters or communication devices.
• Mental status may be assessed in the context of conversation, rather than in a formal examination. It is often helpful to start the interview with a discussion of a patient's strengths and interests, rather than problems, and later focus on the patient's understanding of disability, limitations, and reasons for the referral.
• Nonverbal aspects of the interview include observations of performance on selected tasks, relatedness, expression of affect, impulse control, attention span, activity level, and the presence of unusual behaviors or seizures.

Medical Review

This review should include developmental and medical history, past etiological assessments, and coexisting general medical disorders and their treatments. The latter is particularly important, since undiagnosed medical conditions are frequent in this population and may lead to behavioral symptoms.

Diagnostic Formulation

Data from the assessments should be interpreted in light of developmental level, communication skills, associated handicaps, life experiences, education, and family and sociocultural factors. A particular behavior may suggest an underlying mental disorder if it is a part of a pattern of a defined mental disorder syndrome. The possibility of sexual or other abuse that the patient cannot report, should be considered. A DSM-IV diagnosis (in addition to MR) should be made, if the appropriate criteria are met. The diagnostic statement should include a description of the person's strengths, deficiencies, and needs including intellectual, adaptive behaviors, communication, health, and psychosocial domains. A comprehensive assessment should yield a multiaxial diagnostic formulation with appropriate differential, and supporting evidence for diagnoses should be highlighted.

SPECIFIC DIAGNOSIS OF COMMON COMORBID MENTAL DISORDERS

Pervasive Developmental Disorders (PDD)

The majority of children with PDD also have MR. However, children with MR alone do not have significant impairments in reciprocal social interaction and can engage in social communication, verbal or nonverbal (such as gestures and eye contact), appropriate to their developmental level.

Attention-Deficit/Hyperactivity Disorder

The diagnostic criteria for ADHD are based on observable behavior as reported by multiple informants and thus can be applied to nonverbal children. ADHD should be differentiated from situation-specific inattentiveness, such as at school if the academic expectations are too high, and medication side effects. In assessing "noncompliance"- not following commands of caregivers - one should consider the child's ability to understand social rules and the presence of sufficient skills to communicate opposition.

Tic Disorders and Stereotypic Movement Disorder

In Tourette's disorder, the movements, as opposed to self-stimulatory stereotypes seen in persons with severe MR, are less complex and appear involuntary. For the latter, the diagnosis of stereotypic movement disorder may be used if other mental disorders are excluded. The specifier "with self-injurious behavior," is added if bodily damage results. Self-injurious behavior (SIB) is common in certain MR syndromes, especially Lesch-Nyhan syndrome.

Mental Disorders Due to a General Medical Condition

MR, in and of itself, does not constitute a medical condition to which aberrant behavior or emotional disturbance should be ascribed. The attribution of behavioral or emotional disturbance to a general medical condition should be used only when there is evidence from history, physical examination, or laboratory findings that the disturbance is a direct consequence of a specific medical condition. For example, in the case of Down syndrome, hypothyroidism may present as symptoms of depression. In such cases, the medical disorder to which the depression should be attributed is hypothyroidism, not Down syndrome.

Schizophrenia and Other Psychotic Disorders

Schizophrenia can be diagnosed in the usual manner in verbal persons with mild MR, but rarely if at all in persons with more severe retardation. For the latter, the less specific diagnosis of psychotic disorder NOS may be made, if behavioral features such as grossly disorganized behavior and negative signs are present, but were absent in the premorbid period. Conversation with an imaginary friend should not be confused with hallucinations.

Mood Disorders

Mood disorders, especially depressive disorders, are quite common in persons with MR. In verbal persons with mild MR the complaints are simpler and concrete. History obtained from caregivers and evidence of neurovegetative signs help in assessing the mood change. Depression may also be manifest by aggressive behavior. Environmental events, such as a precipitous move to a new setting or change in care provider, may trigger a depressive episode. Medication side-effects should be considered, for example, depression resulting from beta blockers or agitation associated with akathisia from a neuroleptic drug.

Anxiety Disorders

Verbal persons with mild MR can report on subjective feelings of anxiety; in nonverbal ones symptoms such as avoidance behaviors and agitation might suggest the diagnosis. The tendency toward anxiety and social avoidance is also a part of the behavioral phenotype of fragile X syndrome.

Posttraumatic Stress Disorder (PTSD)

PTSD in persons with MR might be quite frequent and should be routinely considered in the differential diagnosis. These individuals are vulnerable to abuse, due to difficulties in reporting it and a tendency to please others.

Obsessive-compulsive Disorders (OCD)

This diagnosis may be difficult in nonverbal persons who cannot report on obsessional thoughts underlying their compulsions. Some repetitive behaviors, for example, hoarding objects, flicking lights on and off, tidying and arranging, all have been suggested as indication of OCD in persons with MR. Self-restraint, for example, insistence upon wearing a helmet or other protective device, is a behavior that has been described in persons who also exhibit SIB, and might suggest the ego-dystonic nature of self-injury. A connection between some self-injurious behaviors and obsessive-compulsive disorder has thus been postulated.

Eating Disorders

Anorexia and bulimia nervosa are relatively rare in the context of mental retardation, particularly moderate to severe MR, but MR is a predisposing factor for other eating disorders like pica and rumination. The ingestion of nonnutritive substances, pica, and the regurgitation and rechewing of food, rumination, occur with greater frequency as the severity of cognitive disability increases. When these behaviors are a focus of clinical attention, the diagnoses should be indicated.

TREATMENT

The principles of psychiatric treatment are the same as for persons without mental retardation, but modification of techniques may be necessary, according to the individual patient's developmental level, especially communication skills. Medical, habilitative, and educational interventions should be coordinated within an overall treatment program. The mental health clinicians should actively participate with other professionals in the development of the various treatment interventions.

The Behavioral Emergency

When the clinician is called to assist with a behavioral emergency, the first task is generally to ensure the safety of the patient and others. For example, in the case of severe self-injurious or aggressive behavior, if the usual attempts at redirection fail and the patient continues to pose an imminent risk, it may be necessary to temporarily employ physical restraint. In some instances this may require admission to a psychiatric hospital. The use of emergency medication may also be considered after adequate diagnostic assessment. Medical causes for an acute behavioral exacerbation must always be considered. It is not uncommon for even simple problems like constipation, infection, or even occult injury to set the stage for behavioral problems. Medication side-effects like akathisia from neuroleptics or disinhibition from sedative/hypnotics can be expressed in aggressive and self-injurious behaviors. When a temporizing measure is necessary, it is generally advisable to utilize a drug with which the patient has had a positive experience, typically a neuroleptic or benzodiazepine. The need for emergency treatment should prompt a comprehensive diagnostic assessment including the evaluation of environmental influences. Approaches should also be considered to prevent recurrence of such emergency situations for a given individual.

Psychosocial Interventions

Persons with MR may benefit from group, individual and family psychotherapy. Concrete goals should be established, with the overall aim to achieve a maximally feasible quality of life. Disruptive behaviors should not merely be suppressed, but replaced with constructive, adaptive behaviors and skills. Patients should learn to understand their own disability, focus on strengths, develop a positive self image, a realistic striving for independence, and age-appropriate social skills. The treatment techniques include focus on current reality, directiveness and structure to maintain focus, and activities adapted to chronological age. A therapist with training in developmental disorders is best equipped to accomplish these goals and to guide the patient to develop his/her own understanding. Therapists should be active, directive, and flexible, perhaps using themselves as examples, and should be prepared to give concrete advice.

Family therapy typically focuses on the parents' identification and support of their child's strengths and independence, and the provision of opportunities for success. Parents of recently diagnosed children need careful explanation of their child's condition. Concrete advice in management and resource finding is important, as well as help in obtaining educational supports to which the child is entitled under federal and local laws. Parents of adolescents and young adults need help in coming to terms with emergent sexuality, and in emotionally separating and preparing them to move to out-of-family living in the community.

Pharmacotherapy

Medication effects generally are not different from those expected in the absence of MR. The adage, "start low, go slow," reflects the observation that shifts in dose-response in certain contexts are far more likely than changes in the mechanism of action of a compound, for example, persons with Down syndrome may be exquisitely sensitive to anticholinergic drugs, and some persons with MR may be more sensitive to the disinhibiting effects of sedative/hypnotic agents.

There are several problems with pharmacotherapy frequently encountered with persons with MR:

• Some clinicians appear to prescribe medication with inadequate information, aiming, for example, for symptom suppression seemingly without consideration of the potential negative impact on habilitative function or overall quality of life. Risks/benefits for medication appear not to be fully considered (e.g., drugs which adversely affect cognition, either directly or through sedation, appear to be used without clear justification and careful monitoring).
• There is no evidence of informed consent to support the prescription or administration of psychotropic medication.
• Medication appears not to be integrated as part of a comprehensive treatment plan (e.g., there is no evidence of a behavior plan; no evidence of communication between prescribing physician and other therapists; behavioral data are not collected).
• Medication may not appear to be appropriate for the diagnosis of record (e.g., a patient with a diagnosis of a mood disorder is receiving only thioridazine).
• Drug exposure appears to be excessive or poorly justified. Medication appears to be prescribed for extended periods for nonspecific indications for which other active treatments or environmental supports are needed. Multiple representatives from the same medication class and other complex polypharmacy regimens are employed or no attempt can be seen over time to adjust medication doses to document ongoing need or the minimum dose at which a medication remains effective. In some cases treatment effectiveness is never clearly established.
• No evidence can be found for the active monitoring for emergent side-effects, particularly in nonverbal patients.

Treatment Follow-up

A common problem in the treatment of persons with MR is assessing its effectiveness, which may be viewed differently by various caregivers. Therefore, discrete treatment goals should be agreed upon by the clinician and caregivers, as well as target or "index" symptoms. Interdisciplinary collaboration of professionals and caregivers is essential. Various mental health clinicians might function in the team as direct care providers, team leaders, or consultants to other professionals. Among them, clinicians with medically and psychologically oriented training are often prepared to function as synthesizers of treatment modalities of various disciplines. Follow-up includes patient interview/observation and obtaining comprehensive interim information. If the patient is not experiencing improvement, the accuracy and completeness of the biopsychosocial diagnosis should be reviewed, as well as the consistency of implementation of treatment by the caregivers.

REFERENCES

American Academy of Child and Adolescent Psychiatry (1999), Practice Parameter for the Assessment and Treatment of Children, Adolescents, and Adults with Mental Retardation and Comorbid Mental Disorders. J Am Acad Child Adolesc. Psychiatry, XXX.