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

Chlorpromazine and fluphenazine decanoate were employed to treat aggressive behavior and emesis in a 22-year-old patient with Cornelia de Lange syndrome. Institution of the above neuroleptics, following an unsuccessful trial of diazepam, produced reductions of 95.1 per cent and 37.9 per cent for aggressive behavior and emesis, respectively. Four follow-up observations, occurring at 3-week intervals, revealed maintenance of low rates of aggressive behavior and continued reductions in emesis (mean reduction of 81.5 per cent). These findings are important for their a) inclusion of direct behavioral observation as the data base, and b) initial promising effects of neuroleptics for a rare mental retardation syndrome.
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PMID:Pharmacological treatment of aggressive behavior and emesis in the Cornelia de Lange Syndrome. 51 49

The Lesch--Nyhan syndrome is a heritable disorder of the metabolism of uric acid in which behavioral manifestations are prominent and among the most provocative. The mutated or variant gene that determines this disorder is carried on the X chromosome. The disease is expressed exclusively in males. The molecular expression of the abnormal gene is in the completely defective activity of the enzyme hypoxanthine guanine phosphoribosyl transferase. As a result these patients overproduce uric acid and may develop early in life many of the clinical findings we associate with gout. They have in addition a variety of neurological abnormalities including mental retardation, spastic cerebral palsy, and involuntary, choreoathetoid movements. Involved patients have unusual, compulsive, aggressive behavior. Its most prominent but by no means exclusive feature is self-mutilation. The central feature in the management of this behavior is physical restraint. A number of practical procedures have been learned which facilitate the care and feeding of these patients. Promising new findings suggest that behavioral modification using extinction techniques and pharmacologic methods utilizing agents designed to increase the effective cerebral content of serotonin may each have a place in the management of behavior in this syndrome.
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PMID:Behavior in the Lesch--Nyhan syndrome. 108 51

The current status of pharmacological treatments of self-injurious behavior (SIB) and aggression in persons with mental retardation and autism was reviewed in the literature. Much of the existing literature is derived from anecdotal clinical experience, with a relative lack of well-controlled studies to determine the efficacy of different treatments. Although all psychotropics have been used to manage SIB and aggression, particularly promising are the data on the use of opioid antagonists like naltrexone. Beta-blockers may also have some role, but more controlled, systematic studies are needed. Use of neuroleptics is on the decline because of their adverse effects, such as tardive dyskinesia and possible impairment of cognitive functions. We assert that the behavioral problems of SIB and aggression are at times manifestations of different psychiatric syndromes. They present in a modified, atypical form in the developmentally disabled population because of cognitive limitations. Further understanding and classification of the psychopathology associated with this behavior is essential for its successful treatment.
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PMID:Self-injurious behavior in the developmentally disabled: pharmacologic treatment. 129 22

At the present time, neuroleptics are indicated for the treatment of acute psychotic states as well as Tourette's syndrome in children and adults. Neuroleptics may have a useful role in the attenuation of problem behaviors, such as stereotypies, hyperactivity, self-injury, and aggressive outbursts in infantile autism, pervasive developmental disorder NOS, and mental retardation, but they do not improve the underlying condition. Neuroleptics are not the agents of first choice for treatment of hyperactivity or aggression in children who do not have major developmental handicaps. Common and troublesome side effects associated with neuroleptic use in children and adolescents include sedation, extrapyramidal symptoms, and withdrawal dyskinesias; therefore, close monitoring is required. Neuroleptics should be used cautiously and only as an adjunct to other nonpharmacologic interventions.
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PMID:Neuroleptics in pediatric psychiatry. 134 40

Fifty habitually aggressive men were assessed for self-directed aggressive behavior (SDAB) and other-directed aggressive behavior (QDAB). Subjects displaying SDAB were compared with subjects exhibiting exclusively ODAB. The former were found to engage in more frequent acts of verbal aggression, physical aggression against objects, and physical aggression against others, as well as in more severe acts of verbal aggression and physical aggression against others. They were also more likely to receive diagnoses of mental retardation, organic personality disorder, intermittent explosive disorder, or autism. Findings are consistent with the presence of a neurologically based behavioral dyscontrol in the SDAB subjects.
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PMID:Self-directed and other-directed aggressive behavior in a forensic sample. 144 Jul 47

Although this article focuses on psychopharmacology, pharmacotherapy is only part of a comprehensive treatment program. Treatment should be individualized to the patient's condition and level of intellectual functioning (e.g., conduct disorder, mental retardation). Clinicians should be acquainted with the Food and Drug Administration's regulations and the Physician's Desk Reference's guidelines. Psychoactive agents should be prescribed judiciously under careful clinical and laboratory monitoring, especially when given on a long-term basis. Knowledge of potential short- and long-term side effects is imperative to minimize impairment (cognitive, sedation) and to maximize achievement of adaptive behaviors. Aggressiveness is a low-frequency behavior and therefore difficult to assess. Aggressiveness with an explosive affective component and rage seems to be more responsive to pharmacotherapy than aggressiveness alone. Children who present with covert conduct disorder symptoms, such as stealing and lying, might not be as responsive to psychoactive agents as the conduct disorder with explosive characteristics. The neuroleptics are considered the standard drugs for the treatment of aggression but sedation and concern over tardive dyskinesia have led investigators to explore and study other classes of drugs. Lithium carbonate has been studied in short-term clinical trials and has been shown to be an effective alternative to the neuroleptics. Carbamazepine and propranolol seem to be promising agents but require further critical assessment in children and adolescents. Stimulants should be considered the first choice of treatment in coexisting conduct disorder and ADHD or in milder forms of aggression. In conclusion, there is a need for systematic investigation of the effectiveness and safety of psychoactive agents in children and adolescents with aggressiveness, explosiveness, and rage outbursts. There is some supportive evidence that some patients with these target symptoms are good responders to certain drugs. Future research should compare pharmacotherapy to psychosocial treatment and the combination of both.
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PMID:The pharmacologic treatment of conduct disorders and rage outbursts. 154 49

Roberts/pseudothalidomide syndrome is a rare autosomal recessive disorder, involving growth and mental retardation, midline craniofacial abnormalities and tetraphocomelia. Cytogenetic studies have confirmed reproducible chromosomal abnormalities throughout the syndrome's wide range of clinical presentations. A family with two affected siblings is presented. One child was stillborn; the other, though severely affected by all of the physical characteristics of this disorder, has developed normally both at school and in his social-personal skills. This report supports the idea that Roberts syndrome and pseudothalidomide syndrome are the same condition, and emphasizes that normal intelligence and positive social-personal adjustment are possible, even with all of the stigmata of Roberts syndrome. Aggressive medical intervention is suggested, as well as forthright parental counselling when discussing the possible outcome for these patients.
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PMID:Roberts/pseudothalidomide syndrome and normal intelligence: approaches to diagnosis and management. 161 13

The behaviour profiles of 176 mentally retarded individuals from two reception centres and nine group homes were assessed. The correlations between behaviour and age, sex, degree of mental retardation, etiology of mental retardation and medical diagnosis were assessed using the Revised Child Behaviour Profile. The severity of behaviour disturbance did not vary with age or medical diagnosis. The moderately retarded subjects presented with more severe behaviour problems, such as aggression, than the severely mentally retarded subjects. The variable most predictive of behavioural problems was etiology of the disorder. Individuals with Down's syndrome had significantly fewer behaviour disturbances and those with autism and pervasive developmental disorder had significantly more behaviour disturbances than other subjects. A psychiatric disorder was found in 10.2% of the sample. The implications of these findings are discussed with respect to public policy.
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PMID:Behaviour problems of the mentally retarded. 179 May 15

Twelve patients with histologically defined mitochondrial myopathy are described. There were 9 males and 3 females. The age of onset ranged from birth to 35 years with a median of 14 years. The most common clinical picture was that of ophthalmoplegia, ptosis and muscle weakness found in 10 patients. One presented with exercise intolerance due to muscular aches and pains, and the other besides his muscular weakness had mental retardation and an aggressive behavior. The clinical presentation and differential diagnosis of these patients are discussed.
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PMID:[Mitochondrial myopathy: report of 12 cases with histochemical study of the skeletal muscle]. 180 26

Inhalation of thinner by youngsters and adolescents is an increasing drug abuse problem in Mexico. It presents serious repercussions upon socio-economic, cultural, legal and health (neurologic and psychiatric) problems. We report a comparative study in humans and rats which demonstrate the embryotoxic and craneo encephalic teratologic effects in the children and brood of progenitors who have chronically inhaled thinner (in the case of pregnant women, before, at the beginning and throughout pregnancy). Inhaled thinner passes directly to the blood stream and crosses the placentary barrier freely reaching the embryo. It may cause craneal bone and partial or total encephalon agenesia, added to macro and microscopic lesions secondary to direct aggression to the neuroepithelial germ cells. Abortions and premature labor with weight and size underdeveloped products and placentary hemorrhages occur. Usually these die, but if they survive they show trascendental mental retardation, as well as neurologic and psychiatric sequels.
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PMID:[Teratologic cranio-encephalic effects of chronic thinner inhalation in progenitors, in rats and humans]. 182 90


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