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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Children and adolescents with mental retardation and developmental disabilities are thriving in their communities owing 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 mental retardation and developmental disabilities frequently present with unusual symptoms associated with psychotic disorders, leading to misdiagnosis and inappropriate treatment with antipsychotic agents. The problems due to stress on these children and their families, long-terms costs in loss of educational and vocational opportunities, and the development of serious adult psychiatric disorders are enormous. By exploring the risk factors for psychiatric diagnosis in this population and presenting illustrative cases, awareness of the indicators for pediatric practice with this population is provided in this review.
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PMID:Psychiatric disorders in children and adolescents with mental retardation and developmental disabilities. 895 67

We present a family with congenital cataract with, in some cases, mental retardation and emotional instability, but intellectual deterioration in all affected members. The latter was accompanied by psychosis in some. The inheritance is most likely autosomal dominant, affecting two generations and consisting of a congenitally blind parent and 6 of 11 of her offspring. In addition to these features, some affected individuals had dysphagia and movement disorder, especially choreiform movements. They all showed small body mass, due possibly to poor nutrition from dysphagia. The pathological findings were unique, demonstrating selective atrophy of the granule cell layer of the dentate gyrus. There was selective expression in paraffin-embedded sections of alpha B-crystallin (CRYA2) in oligodendroglia in all areas of the nervous system examined. alpha B-Crystallin is a major optic lens protein but also a heat shock protein and molecular chaperone found in brain and a number of other tissues. Because of the association of congenital cataract and the accumulation in oligodendroglia of alpha B-crystallin, the gene for this protein was sequenced for possible mutation. No mutation was found indicating other genetic locus. This family appears to have a newly recognized genetic disorder.
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PMID:A familial syndrome of congenital cataract, mental impairment, and dentate gyrus atrophy. 912 9

Several drugs are apparently effective in treating pathologic anger and aggression. Because many of the studies on aggressive populations allowed the use of concomitant medications, it is unclear whether the efficacy of each drug in a particular population is dependent on the presence of other medications, such as antipsychotic agents. Finally, one needs to be circumspect in inferring efficacy of a particular drug in aggressive patients with neuropsychiatric conditions other than the ones in which some efficacy has been established. Lithium appears to be an effective treatment of aggression among nonepileptic prison inmates, mentally retarded and handicapped patients, and among conduct-disordered children with explosive behavior. Certainly, lithium would be the treatment of choice in bipolar patients with excessive irritability and anger outbursts, and it has been shown to be effective in this population. Anticonvulsant medications are the treatment of choice for patients with outbursts of rage and abnormal EEG findings. The efficacy of these drugs in patients without a seizure disorder, however, remains to be established, with the exception perhaps of valproate and carbamazepine. In fact, dyphenylhydantoin did not appear to be effective in treating aggressive behavior in children with temper tantrums and was found to be effective in only a prison population. There is some evidence for the efficacy of carbamazepine and valproate in treating pathologic aggression in patients with dementia, organic brain syndrome, psychosis, and personality disorders. As Yudofsky et al point out in their review of the literature, although traditional antipsychotic drugs have been used widely to treat aggression, there is little evidence for their effectiveness in treating aggression beyond their sedative effect in agitated patients or their antiaggressive effect among patients whose aggression is related to active psychosis. Antipsychotic agents appear to be effective in treating psychotic aggressive patients, conduct-disordered children, and mentally retarded patients, with only modest effects in the management of pathologic aggression in patients with dementia. Furthermore, at least in one study, these drugs were found to be associated with increased aggressiveness in mentally retarded subjects. On the other hand, atypical antipsychotic agents (i.e., clozapine, risperidone, and olanzapine) may be more effective than traditional antipsychotic drugs in aggressive and violent populations, as they have shown efficacy in patients with dementia, brain injury, mental retardation, and personality disorders. Similarly, benzodiazepines can reduce agitation and irritability in elderly and demented populations, but they also can induce behavioral disinhibition. Therefore, one should be careful in using this class of drugs in patients with pathologic aggression. Beta-blockers appear to be effective in many different neuropsychiatric conditions. These drugs seem effective in reducing violent and assaultive behavior in patients with dementia, brain injury, schizophrenia, mental retardation, and organic brain syndrome. As pointed out by Campbell et al in their review of the literature, however, systematic research is lacking, and little is known about the efficacy and safety of beta-blockers in children and adolescents with pathologic aggression. Although widely used in the management of pathologic aggression, the use of this class of drugs has been limited partially by marked hypotension and bradycardia, which are side effects common at the higher doses. The usefulness of the antihypertensive drug clonidine in the treatment of pathologic aggression has not been assessed adequately, and only marginal benefits were observed with this drug in irritable autistic and conduct disorder children. Psychostimulants seem to be effective in reducing aggressiveness in brain-injured patients as well as in violent adolescents with oppositional or conduct disorders, particu
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PMID:Psychopharmacologic treatment of pathologic aggression. 919 23

Behavioral and cognitive-behavioral strategies and a broad range of group, family, couples, and milieu treatment approaches have been developed for the psychotherapy of aggressive and violent patients. These methods have been carried out in diverse settings ranging from hospitals and prisons to individual outpatient practices and have been applied across diverse populations including adults with mental retardation, dementia, and brain injury; children with attention deficit and conduct disorders and autism; recurrent violent offenders with antisocial personality disorder; and individuals with chronic psychotic disorders, mood disorders, or medical illnesses such as hypertension. Bridging these different strategies are the underlying principles of psychotherapy with aggressive and violent patients. These include ensuring the safety of clinician, patient, and potential victims as the foremost concern; developing a finely detailed assessment of aggressive and violent acts and of the antecedents, assumptions, and consequences that are attached to them; formulating well-defined goals and striving for clear communication to achieve consistency in the pursuit of these goals between therapist and patient, and among therapist and other clinicians, staff, and relevant family members or agencies; specifying ahead of time well-considered outcome measures to be used to gauge the effectiveness of treatment; and maintaining a healthy vigilance for countertransferential and similar reactions and a willingness to use consultation as an integral part of treatment.
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PMID:Psychotherapeutic approaches to aggressive and violent patients. 919 24

As the new generation of atypical antipsychotics becomes available, the limitations of the older typical agents become apparent. The new medications, which have benefits other than the alleviation of positive symptoms of schizophrenia, may also be beneficial for psychotic disorders that have responded poorly to conventional neuroleptics. This article will describe the potential use of the atypical antipsychotics, especially olanzapine, for affective mood disturbances in schizophrenia, psychotic depression and mania, first-break schizophrenia, comorbid schizophrenia and substance abuse disorders, dementia in the elderly and those with late-onset schizophrenia, and behavioral problems in patients with mental retardation or developmental delays.
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PMID:Olanzapine and the new generation of antipsychotic agents: patterns of use. 926 12

Schizencephaly is a rare disorder of brain development resulting in the formation of abnormal unilateral or bilateral clefts in the cerebral hemispheres. It is often accompanied by partial seizures, mental retardation, and hemiparesis. Two patients are described with clear psychotic symptoms with either unilateral or bilateral schizencephaly. The implications of the association between schizencephaly and psychosis in these patients for understanding the biology of the psychoses are discussed.
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PMID:Schizencephaly associated with psychosis. 932 56

Fragile X syndrome is the most common inherited form of familial mental retardation. It results from a (CGG)n trinucleotide expansion in the FMR1 gene leading to the typical Martin-Bell phenotype. Clinical features vary depending on age and sex. Expansion of a (CCG)n repeat in the FMR2 gene corresponds to the FRAXE fragile site which lies distal to FRAXA and is also associated with mental retardation, but it is less frequent and lacks a consistent phenotype. Analysis of repeat expansions in these two genes allows the molecular diagnosis of these different entities. We report here the screening of the FRAXA and FRAXE mutations in 222 unrelated mentally retarded individuals attending Spanish special schools. PCR and/or Southern blotting methods were used. We detected full mutations in the FMR1 gene in 11 boys (4.9%) and 1 boy (0.5%) with a CCG repeat expansion in the FMR2 gene. The latter shows mild mental retardation with psychotic behaviour and no remarkable physical traits. Molecular studies revealed a mosaicism for methylation in the FMR2 gene. This case supports the observation that expansions greater than 100 repeats can be partially methylated and cause the phenotype.
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PMID:Screening for FMR1 and FMR2 mutations in 222 individuals from Spanish special schools: identification of a case of FRAXE-associated mental retardation. 934 61

According to the DSM-IV, all types of psychiatric disorders can be observed in mentally retarded subjects, with prevalence estimated to be three or four times higher than in the general population. Clinical features, longitudinal course and triggering factors are influenced by the characteristics of the intellectual disability. The aim of this paper is to discuss the specificity of the psychopharmacological therapy in mental retardation (sensitivity to specific psychotropic drugs, incidence of side effects, predictive criteria for evaluating risk factors, etc.) and to propose an update in the pharmacotherapy in the most important psychiatric disorders (mood disorders, psychotic disorders, behavioral disorders, anxiety disorders, attention deficit disorders with or without hyperactivity.
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PMID:Psychiatric illness in mental retardation: an update on pharmacotherapy. 947 71

Attempts have been made to establish objective diagnostic criteria for psychiatric disorders in persons with mental retardation. This paper describes the clinical features of the most important psychiatric disorders in mentally retarded adolescents: mood disorders, psychotic disorders, severe behavioral disorders, personality disorders, anxiety disorders, and attention deficit disorder with hyperactivity. The impact of mental retardation on personality development is confirmed by the high psychopathological vulnerability of the mentally retarded. All types of mental disorders can be observed, with an incidence estimated to be at least three or four times higher than in the general population. Adolescence is a particularly important phase for the mentally retarded, since adolescent turmoil can increase the risk of psychopathology.
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PMID:Psychiatric illness in mentally retarded adolescents: clinical features. 970 28

We examined aggression and psychopathology in persons with severe or profound mental retardation. Most aggressive episodes were directed toward other clients, and ratings of aggression were positively correlated with self-injury, stereotypic behavior, and being ambulatory. In a linear regression analysis of psychopathological correlates, aggression was most consistently predicted by dependent personality and psychosis. To better describe the construct of aggression, we also developed an Aggression-psychopathology scale. Persons with mental retardation and aggression were more likely to be impulsive, attention-seeking, dependent, socially inadequate, and anxious. Intensive efforts to modify the psychopathological correlates of aggression may improve treatment planning and outcome.
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PMID:Aggression and psychopathology in persons with severe or profound mental retardation. 977 Feb 54


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