Psychiatric Disorders in Children and Adolescents with Mental Retardation and Developmental Disabilities Anne Desnoyers Hurley, Ph.D. Tufts University School of Medicine Abstract
Children and adolescents with mental retardation and developmental disabilities (MR/DD) are thriving in their communities due to special education programs that provide full inclusion in school and community life. Many youngsters, however, do not reach their full potential because of the limitations imposed by untreated psychiatric disorders. Although striking behavioral symptoms may be present, care providers often mistake them for typical aberrant behavior associated with developmental disabilities. When this occurs, these children do not receive proper psychiatric care, and may suffer restrictive behavioral programming and exclusion from community living. On the other hand, children and adolescents with MR/DD frequently present with unusual symptoms associated with psychotic disorders, leading to misdiagnosis and inappropriate treatment with antipsychotic agents. The stress on these children and their families, and long-‐terms costs in loss of educational and vocational opportunities, as well as the development of serious adult psychiatric disorders, is enormous. By exploring the risk factors that affect psychiatric diagnosis in this population, and presenting illustrative cases, awareness of the indicators for pediatric practice with this population will be provided. Citation: Hurley, A.D. (1996). Psychiatric disorders in children and adolescents with mental retardation and developmental disabilities. Current Opinion in Pediatrics, 8, 361-‐365. Contact:
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Psychiatric Disorders in Children and Adolescents with Mental Retardation and Developmental Disabilities Children and adolescents with mental retardation and developmental disabilities (MR/DD) are thriving in their communities due to the introduction of a special education programs that not only meets educational needs but provides full inclusion in school and community life. Many youngsters, however, do not reach their full potential or enjoy extensive social networks because of the limitations imposed by untreated psychiatric disorders. Although striking behavioral symptoms may be present, care providers often mistake them for typical aberrant behavior associated with developmental disabilities, and referral to psychiatric consultants and viable treatments are not offered. The costs of mental illness in this population are high and can result in institutional placements out of the home, separating the child from his or her family, and possibly introducing early feelings of abandonment and rejection. In schools settings, restrictive behavioral plans may be developed, or the child may be excluded from regular classes and interaction with non-‐disable peers, jeopardizing the development of peer relationships and self-‐esteem. As adulthood approaches, untreated psychiatric disorders become more associated with serious aberrant behavior, or engagement in high risk behavior such as substance abuse or sexual promiscuity. Thus, it is imperative that pediatricians are alert to the signs of psychiatric disorder in children and adolescents with MR/DD in order that referral for appropriate services be accomplished. The occurrence of psychiatric disorders in children and adolescents with MR/DD is an enormous problem. Studies of the rates of psychiatric disorder in this population vary widely, but there is a consensus that the rate is quite high due to several risk factors. First, these children have lessened coping abilities due to cognitive impairment and 2
developmental delay. When faced with difficulties, they have limited abilities to reason, rationalize, intellectualize, and find appropriate strategies to meet their goals. Second, the deficits in the neurobiological substrate that cause the developmental disability may as well cause abnormalities in emotional and behavioral functioning. For example, many neurological disorders are associated with behavioral and psychiatric syndromes, such as the association between Fragile X and attention deficit hyperactivity disorder (ADHD) (1,2,3), and between autism spectrum disorder and mood disorders (4,5,6) .
Third, serious psychosocial risk factors are present in the population. Despite
supports and intervention in schools, the vast majority of children and adolescents suffer social rejection from peers. Ridicule and taunting is still quite common, although schools have instituted disability awareness programs. Further, as children age, they are less prone to socialize with the child who has a significant handicap, and invitations to parties and social events typically decrease. These stressors become severe in early adolescence, with moves to junior high and high school settings. More disturbing is the high rate of child abuse in this population. Major studies have concluded that children and adolescents with disabilities are at great risk for victimization (7,8,9,10,11,). Further, once victimized, they are less able to articulate the situation than their peers, leading to delay in identification of the problem, less supports, and further social isolation.. There are also several case reports documenting the occurrence of psychiatric disorder after victimization (12,13). Studies of Prevalence Rates Studies of the rate of psychiatric disorders in children and adolescents with MR/DD have shown significantly high rates compared to non-‐disabled peers. Reiss (14) recently summarized 36 studies of children and adults and reported rates from under 10% to over 3
35%. He noted that low prevalence rates were generally found in surveys that relied on retrospective review of case files, while higher rates were found in surveys using professional interviewers and more elaborate scientific methods. Reiss himself conducted a case-‐file retrospective review finding a prevalence of 10% for mental health problems in children with MR/DD among 5,637 public school case files in Illinois. (15) The Isle of Wight study found 50% of children with neurological and developmental disabilities, ages 9 to 11, to have behavioral disorders, and this research project incorporated the examination of a normative population, rating scales completed by parents and teachers, and an individual diagnostic interview, providing the most rigorous methods (16). For children with developmental disabilities, parents rated 30% to have a behavioral disorder, teachers 42%, and psychiatric interview found 50%, compared to a finding of 7% in the general population. This study concluded that children with neurological impairments and developmental disabilities were at significant risk for behavioral and mental health problems. Gillberg, Persson, Grufman & Themner (17) followed 149 youngsters in a 4-‐year birth cohort at ages 13-‐17 and found a 57% rate of behavioral disorder among those with mild mental retardation and 64% among those with severe mental retardation. Gath & Gumley (18) studied 346 children with Down syndrome in a health care region in England and found 38% with behavioral disorder; a matched group of MR/DD children without Down syndrome found 49% to have behavioral disorder. McQueen, Spence, Garner, Pereira, & Winsor (19) studied 221 children with intelligence quotients below 55, in a 4-‐ year birth cohort, in the maritime provinces of Canada. In this study, 32% had behavioral disorders and 9% were identified with psychiatric disorder. Thus, there is no question that the rate of such disorders is significant and deleteriously effects the lives of these children and their families. Under-‐Identification of Psychiatric Disorders in Children and Adolescents with 4
Mental Retardation and Developmental Disabilities There are two major factors contributing to the under-‐ identification of psychiatric disorders in children and adolescents with MR/DD. Although certainly very familiar with children who have serious handicaps, most medical and mental health providers are prone to see aberrant behavioral symptoms as behavior associated with the disability itself. For example, the high rate of overactivity among children with developmental delays can easily be missed as symptoms of Attention Deficit Hyperactivity Disorder (ADHD) in a particular child with MR/DD. Professionals are apt to be struck by the disability itself, eclipsing interpretation of symptoms and signs as possibly part of a treatable psychiatric disorder. Reiss and his colleagues named this phenomenon diagnostic overshadowing, and provided an analogue experimental study to support their contention (20). They posited that the professional is so "overwhelmed" by the disability itself, it "overshadows" the symptoms and signs of psychiatric disorder. Their work has recently been further extended to individuals with physical disabilities as well, in a study of diagnostic response of rehabilitation counselors to difficulties in their clients (21). The second set of factors explaining the under-‐diagnosis of psychiatric disorders is associated with striking differences in presentation of psychiatric disorders in children and adults with MR/DD. The present body of knowledge regarding psychiatric disorders in children is formulated by study of children with normal intelligence, experiencing a normal course of developmental growth in all areas. For example, it is assumed the child or adolescent can, at least to some extent, participate in the diagnostic interview, the cornerstone of psychiatric diagnosis. Children and adolescents with MR/DD have difficulty complying with the standard diagnostic interview (22). Because of overall cognitive limitations, questions posed during the diagnostic interview may not be understood, or they may be answered improperly. For example, such children may not understand questions about internal feeling states, and may answer that they feel “fine” because they 5
cannot articulate feelings of dysphoria. Children and adolescents with MR/DD also are prone to experience an overall breakdown of coping mechanisms, regressing to a previous developmental level of functioning, or becoming nearly totally dysfunctional. This phenomenon, termed cognitive disintegration (22), is thought to be due to neurobiological inefficiency of the central nervous system, resulting in a loss of integrity when experiencing stress. Loss of continence, for example, is a common manifestation of psychological stress, but parents may see this difficulty as a “behavioral” issue, and pediatricians may embark on a complex medical evaluation, ignoring psychiatric disorder in the differential diagnosis. It is also often observed that previously existing aberrant behaviors, such as self-‐ injurious behavior, may increase in frequency or severity dramatically when suffering from a psychiatric disorder. The salience of very unusual behavior typically distracts family members, teachers, and pediatric clinicians from considering an underlying psychiatric disorder as a possible cause and seeing the problem as “behavioral.” For example, an increase in self-‐injurious behavior in a child with moderate mental retardation and autism would typically be addressed as "behavioral" rather than exploring the possibility that a treatable mental illness exists to explain the change. The family and teachers working with such a child would also be concerned and directed at preventing the self-‐abuse immediately, by suppressing it with a strong behavioral approach. Developmental delay itself can also causes serious problems to appear simplistic and unimportant. For example, a seriously anxious child with mental retardation may feel profoundly depressed, but express it as having an upset stomach, and may also giggle or seem silly at the same time. 6
Illustrative Case Report A case report illustrating considerable delay in diagnosis was reported by Rosenberg, Johnson and Sahl (23). They treated a 13-‐year old boy with moderate mental retardation. His history of disruptive and aggressive behavior began at age three, but he had not been referred for psychiatric treatment until age 12. Due to his impulsivity, hyperactivity, oppositional behavior, lying, stealing, and fighting, he was referred for an evaluation, and possible psychiatric hospitalization, with an initial working diagnosis of ADHD. Treatment with antidepressant medication precipitated a manic episode with frequent seclusion. He was noted to giggle and laugh, showed preoccupation with sexuality, sexual aggression, and he urinated on the floor. His speech became grandiose, pressured and racing thoughts with psychomotor agitation were seen, as well as shifts into overt symptoms of depression, tearfulness, and flat affect. He was finally stabilized on Lithium Carbonate 1,320 mg daily, with a blood level of 0.98 mEq/L. In this case, it would have been fruitful for the boy to have been referred earlier in his life. Illustrative Case Report Jones and Berney (24) reported four cases of early onset rapid cycling bipolar disorder in children with mental retardation. When all four children came to the attention of psychiatric caretakers, there was a reluctance to consider a serious psychiatric diagnosis and instead to consider conduct disorder for two patients, a neurotic disorder for one patient, and schizophrenia for only one patient. One case, an 11-‐year old girl with a family history of bipolar disorder, was referred for psychiatric care at age 4 and placed in special education programs. Her initial depressive episode lasted 6 weeks at age 11, followed by two years of episodes of high level of activity, reduced need for sleep and incoherent, continuous chatter. These periods lasted for 10 days and occurred every 6 weeks. She also soiled and was incontinent, and although she had several admissions to her local hospital, the behavior was thought to be merely manipulative. At age 14, she was referred to a 7
psychiatric unit. There, she was diagnosed with bipolar disorder, and she responded moderately to a regimen of Lithium and carbamazepine in combination with lorazepam at times of florid symptoms. Overdiagnosis/Misdiagnosis of Psychotic Disorders Although psychiatric disorders may be easily missed, on the other hand, in the presence of behavior that is quite unusual, disruptive, or bizarre, psychiatric care may be immediately sought, but with resulting misdiagnosis of a psychotic disorder. Children and adolescents with MR/DD may, for example, report hallucinations and delusions when experiencing stress, leading even experienced clinicians to either palliatively treat the “psychosis” with antipsychotic medication or misdiagnosis of a psychotic disorder, when in fact, the person is suffering from another difficulty, such as a mood disorder or post traumatic stress disorder. (25) This has led, in part, to the dramatic history of over-‐ medication with antipsychotic agents for people with MR/DD. Studies and reports have found rates between 30 to 50% of adults with MR/DD receiving psychotropic drugs, and antipsychotics have typically been the most used agents (26, 27, 28 ). The overuse of such agents suggests that the common psychiatric disorders, such as depression, anxiety, and attention deficit hyperactivity disorder (ADHD), are not considered.. Illustrative Case Report A case series illustrating misdiagnosis was reported by Warren, Holroyd, and Folstein (29). Five patients were referred for evaluation of possible Alzheimer-‐like dementia in a specialized clinic for Down syndrome and Alzheimer Disease, established because of the association of this disorder with Down syndrome as these patients advance in age (30). All patients reported in this paper suffered from depression and were successfully treated. One patient was a 17-‐year old girl who had regressed in her self care 8
skills, became mute, lost interest in their activities, became incontinent, fearful, lost 15 lbs. of weight, and displayed irregular sleep. She also engaged in inappropriate laughter and crying, as well as reporting visual hallucinations. She was successfully treated with 75 mg nortriptyline and 14 ECT treatments. Her symptoms were quite dramatic, but not inconsistent with a mood disorder, as isolation, and disturbance in sleep and appetite are hallmarks of a major depressive episode. Mistaken identification of Hallucinations and Delusions: Monologue, Imaginary Friends, and Fantasy Although treatable psychiatric disorders may be under-‐diagnosed, aberrant behavior is also falsely diagnosed as major psychiatric disorders when counseling interventions or behavioral work, combined with appropriate educational supports, would address problem areas. It is common for misdiagnosis to occur when reported phenomena are not true hallucinations or delusions, but are instead self-‐talk, imaginary friends, or fantasy manifesting itself due to the developmental delay of the patient. Young children frequently talk out loud to themselves, either engaged in play, or as assistance or self-‐ instruction when completing a task. Beyond the age of 6, such self-‐talk is discouraged and becomes internalized. (31) Among children and adolescents with MR/DD, such behavior is often retained, even into adulthood. The development of "private speech" may not be effectively reached by many individuals with mental retardation. When upset or under severe stress, the character of the self-‐talk may change. It routinely becomes more emotional and reflects the difficulties. Parents or teachers reporting such behavior, however, see it as quite disturbing and it may be identified as “psychotic.” (25) Many children have imaginary friends, but give up this coping mechanism as they age. (32) There is also usually an acute awareness that this is “play.” Children and adolescents with MR/DD may not be as aware of this as “play” and such coping mechanisms may persist into adulthood to compensate for social rejection and lack of peer 9
relationships. When upset, the character of the conversations may be appropriately angry and emotional, with parents reporting bizarre imaginary fights or yelling for hours in a bedroom when no one is present. These may be framed as "pseudo-‐hallucinations" and "pseudo -‐delusions."(25) Illustrative Case Report The following case report illustrates a typical situation in which aberrant behavior was thought to be indicative of a psychotic disorder. Fisher, Piazza, and Page (33) treated an 8-‐year old boy with autism spectrum disorder who had received extensive inpatient treatment and pharmacotherapy with haloperidol, 1.5 mg daily with no improvement. He was transferred to an intensive behavioral program and the psychiatrist gradually increased the haloperidol to 4.2 mg daily because the boy was observed talking to unseen people and inanimate objects ("James, don't you be peeing on the floor."). After a month of no improvement, drug therapy was discontinued and behavioral treatment was instituted. A contingency management plans using a token economy program for appropriate speech, and an overcorrection procedure were used. The “psychotic speech” dramatically decreased with the contingency management and 8-‐month follow-‐up data showed continuing positive levels of behavior. This boy responded to supportive behavioral and educational interventions for children with autism spectrum disorder, and the original diagnosis of atypical psychosis appears unwarranted. The success of behavioral methods suggested a more functional relationship between the behavior/symptoms and environmental variables. Conclusions and Recommendations Children and Adolescents with MR/DD have great opportunities available to them with national changes in special education, stressing full inclusion, community membership and quality of life. A major barrier to success is the high rate of untreated psychiatric 10
disorders, and failure to accurately recognize problems has led to institutional placements, loss of community opportunities, and inappropriate treatment with antipsychotic medication. Pediatric providers may, like other professionals, not recognize difficulties as being possibly caused by a psychiatric disorder, due to diagnostic overshadowing, or the eclipse of aberrant behavior by perception of the disability itself as the cause (20). Children and Adolescents with MR/DD most often present with symptoms of psychiatric illness that are different from those experienced by non-‐disabled youngsters. Because of limitations in cognitive and verbal abilities, children and adolescents with MR/DD cannot articulate internal feeling states and thoughts well. These disabilities also limit the utility of the mental status exam and psychiatric diagnostic interview. Further, maladaptive behavior, impairments in the neurobiological substrate, developmental delay, and psychosocial factors may cause the outward manifestations of psychiatric disorders to be different from those ordinarily expected among non-‐disabled children and adolescents. (22,25) In this paper, case examples were given to illustrate the presentation of this psychiatric disorder among children and adolescents with MR/DD. The patients did not verbalize internal feeling states related to the symptomatic picture. The cases studies also illustrated a high proportion of unusual features, such as hallucinations, delusions, gross neglect of self-‐care, and catatonic withdrawal. These difficulties occur in children and adolescents with MR/DD when experiencing any extreme distress and may be a general "breakdown" of coping systems. Whereas the appearance of such behavior in the average child might suggest a psychotic disorder, with the MR/DD population, that it not the case. Instead, health care providers should adopt a false negative strategy, treating a psychotic illness last after thoroughly ruling out the more common disorders such as mood disorders, anxiety disorders and ADHD. (25) Pediatricians must be aware of the high rate of psychiatric disorders in children and adolescents with MR/DD. They must think flexibly about symptom presentation, and refer 11
to a specialist when necessary. Presently, however, most mental health clinicians do not receive specific training in mental retardation or developmental disabilities. The case reports used in this paper were examples from the practice of specialists in psychiatry and mental health, and unfortunately, many mental health specialists are not adequately trained in MR/DD. A panel of the American Psychiatric Association found few residency programs offer intensive training in mental retardation or developmental disabilities (34). A similar situation exists in clinical psychology, and Nezu reported a survey of graduate clinical and counseling psychology programs conducted by Phelms and Hammer who found 75% of clinical and 67% of counseling programs did not include mental retardation or developmental disabilities in the curriculum(35). Nezu also found that the Journal of Consulting & Clinical Psychology published only 11 articles on mental retardation in all the journals from 1972 to 1992, which included 3,431 papers (Nezu, 1994). Thus, many of the difficulties in either under-‐ or over-‐diagnosing psychiatric disorders in children and adolescents with MR/DD will occur during the referral process to specialists. Pediatricians, must, therefore, be educated themselves about the issues, and sensitive to the impact of delayed development on symptom presentation.
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