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Long-term prognosis of epilepsy in children--a follow-up report beyond 18 years of age.

The long-term prognosis of 185 children with epilepsy, who continued to attend the Clinic for Epileptic Children, the Department of Pediatrics, the University of Tokyo, beyond the age of 18 years, was reported. The length of follow-up varied from three to 20 years, but most of them were followed longer than 10 years. The presumed etiology in these children was divided into a cryptogenic group (124, 67.0%) and a symptomatic group (61). The types of seizures were classified into grand mal (86 cases), focal seizure (27), petit mal absence (4), psychomotro seizure (5), infantile spasms (7), and so on. It may be noted that the highest frequency of grand mal was demonstrated, while the incidences of infantile spasms, myoclonic seizure, and akinetic seizure were low in the series. Only 28 children (15.1%) had complications of physical and/or mental handicaps. The follow-up study revealed that 140 patients (75.7%) had been seizure-free in the last 12 months. One hundred and fifteen of them had no seizures for five years or longer. On the other hand, electroencephalographic abnormalities generally continued for a long time after disappearance of seizures. Eightly-one of well-controlled patients were gradually decreasing the doses of anticonvulsants. As for seizure types, it is noted that focal seizure, psychomotor seizure, and infantile spasms were relatively difficult to be controlled. Except for 27 patients, most of them attended normal schools, including junior colleges or universities, and engaged in various occupations. Fifteen female patients had already married, and out of 13 babies who were born from these patients, there were one with ventricular septal defect, one with mental deficiency, and one with anencephaly, while the rest were entirely normal. Additional problems on withdrawal of anticonvulsants after a long-term seizure-free period, and what a medical system should be for treatment of epilepsy in children up to their adulthood were discussed.

Adolescent↗

Abilities and needs of children with genetic syndromes.

Children with unbalanced karyotypes or other genetic syndromes are generally supposed to be developmentally retarded and mentally handicapped. This has to be ascribed to defect oriented views, leading to negative valuations of their unusual physical condition including slow motor development. A new dialogue oriented view and working concept is presented. It accepts physical differences as biological facts, which primarily do not restrict brainpower. The development of intelligence, behaviour and personality depends largely on the environment. Impeding factors should be searched here and eliminated as far as possible with the aim to prevent secondary mental disability after a syndrome diagnosis. In general, children with genetic syndromes are able to understand language. Thus, they can be educated like other children. They should learn to accept rules and limits, answer questions and follow demands. Their communicative tools may be words, gestures, mimics or written language. While physical support has to be given if necessary, their principally open mind needs stimuli according to their chronological age. Normal mental capacities should be expected, unless the opposite is proven.

Child↗

Childhood-onset growth hormone deficiency, cognitive function and brain N-acetylaspartate.

Cognitive deficits have been reported in adults with childhood-onset growth hormone (GH) deficiency. We evaluated cognitive deficits simultaneously with parameters for neuronal integrity using (1)H magnetic resonance spectroscopy (MRS) in a cross-sectional design. We studied 11 adults (mean age 24.5 years) with childhood-onset GH deficiency, which persisted after reaching final height. All subjects were evaluated after interruption of GH supplementation for at least 3 months. We performed neuropsychological assessment (NPA) using tests evaluating memory, mental processing speed, reading ability and executive functioning. MRS was used to assess brain N-acetylaspartate (NAA)/choline ratios. Data were compared with an age-, sex- and education-matched control group (n=9, mean age 27.3 years). NPA demonstrated attenuated performance of the patients in the delayed verbal memory recall score (P<0.05) and the trail making A test (P<0.05), a measure of planning of behavior, processing speed and attention. Other neuropsychological tests were not affected. NAA/choline ratios were significantly reduced (P<0.01) in GH deficient subjects. Specific cognitive defects indicating affected memory and attention were found in patients with childhood-onset GH deficiency. These defects occur simultaneously with reduced neuronal integrity.

Adolescent↗

The middle-class family and middle-class adolescents in a state of disarray: a social-psychiatric analysis.

Because mental health professionals tend to focus their attention on the complex of factors involved in the provision of psychotherapeutic and related services, they often do not give primary attention to the serious instabilities afflicting marriage and family among the middle class and are insufficiently aware of parents' deficiencies in rearing their children well. Moreover, they frequently underestimate the increasing prevalence of character defects and disorders and the acting-out problems they cause among middle-class adolescents and youths, who are widely involved in sexual activity, drug use, and theft and whose educational performance has sharply declined. Using a social-psychiatric perspective, this paper discusses the major sociocultural factors that greatly influence the problems straining and breaking up marriage and family among the middle class. It also examines the reasons for parental inadequacies that contribute heavily to their children's becoming impulse-dominated and involved in self-impairing and socially harmful problems.

Achievement↗

Patterns of anomalies in children with malformed ears.

Sixteen children with anomalies of the auricle and/or middle ear who presented malformations of the face, mouth, upper airway, spine, limbs, heart, gastrointestinal (GI), and/or genitourinary (GU) systems, were described. While clusters of anomalies suggested syndromes such as the oculo-auriculo-vertebral syndrome of Goldenhar, hamifacial microsomia, mandibulo-facial dysostosis (Treacher Collins syndrome), Pierre Robin, Klippel-Feil, Moebius, Duane, and/or VATER syndromes, many children did not fit what are usually considered even minimal criteria for these syndromes. Several children had malformations which fit the description of more than one syndrome. The importance of investigating the children for unsuspected anomalies, especially of the GU system, was emphasized. Life threatening problems in this group consisted of airway problems, congenital heart disease, and major anomalies of the GI and GU systems. Better management of sucking, swallowing and airway problems might have decreased the early morbidity and mortality (3/16) in this group. Children with multiple defacing anomalies may not be mentally retarded so that aggressive management of their visceral anomalies and hearing problems, and early educational intervention are mandatory. Delay in development may be due to hearing loss, vestibular impairment, ataxia, the consequences of early malnutrition, and multiple hospitalizations rather than to mental retardation. A pessimistic attitude in infancy is unwarranted since it is impossible to predict which children will end up competitive individuals.

Abnormalities, Multiple↗

Developmental precursors of affective illness in a general population birth cohort.

BACKGROUND: Recent evidence suggests that neurodevelopmental impairment may be a risk factor for later affective disorder. METHODS: Associations between childhood developmental characteristics and affective disorder were examined in a prospectively studied national British birth cohort of 5362 individuals born between March 3 and March 9, 1946. Mental state examinations by trained interviewers performed at ages 36 and 43 years identified 270 case subjects with adult affective disorder (AD). Teachers' questionnaires completed at age 13 and 15 years identified 195 case subjects who had shown evidence of childhood affective disturbance (CAD). RESULTS: Female gender and low educational test scores at ages 8, 11, and 15 years were a risk factor for AD, CAD, and AD without CAD. In addition, attainment of motor milestones was later in the CAD group (odds ratio [OR] = 1.2; 95% confidence interval [CI], 1.1-1.3), followed by, and independent of, greater risk for speech defects between the ages of 6 and 15 years (OR = 2.0; 95% CI, 1.3-3.0), decreased psychomotor alertness on medical examination between ages 4 and 11 years (OR = 4.6; 95% CI, 2.2-9.7), and an excess of twitching and grimacing motor behaviors in adolescence (OR = 3.9; 95% CI, 2.5-6.1). Persistent CAD was strongly associated with persistent AD (OR = 7.8; 95% CI, 2.6-23.2). CONCLUSION: The findings give credence to the suggestion that affective disorder, especially its early-onset form, is preceded by impaired neurodevelopment.

Adolescent↗

CHARGE syndrome.

CHARGE syndrome was initially defined as a non-random association of anomalies (Coloboma, Heart defect, Atresia choanae, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness). In 1998, an expert group defined the major (the classical 4C's: Choanal atresia, Coloboma, Characteristic ears and Cranial nerve anomalies) and minor criteria of CHARGE syndrome. Individuals with all four major characteristics or three major and three minor characteristics are highly likely to have CHARGE syndrome. However, there have been individuals genetically identified with CHARGE syndrome without the classical choanal atresia and coloboma. The reported incidence of CHARGE syndrome ranges from 0.1-1.2/10,000 and depends on professional recognition. Coloboma mainly affects the retina. Major and minor congenital heart defects (the commonest cyanotic heart defect is tetralogy of Fallot) occur in 75-80% of patients. Choanal atresia may be membranous or bony; bilateral or unilateral. Mental retardation is variable with intelligence quotients (IQ) ranging from normal to profound retardation. Under-development of the external genitalia is a common finding in males but it is less apparent in females. Ear abnormalities include a classical finding of unusually shaped ears and hearing loss (conductive and/or nerve deafness that ranges from mild to severe deafness). Multiple cranial nerve dysfunctions are common. A behavioral phenotype for CHARGE syndrome is emerging. Mutations in the CHD7 gene (member of the chromodomain helicase DNA protein family) are detected in over 75% of patients with CHARGE syndrome. Children with CHARGE syndrome require intensive medical management as well as numerous surgical interventions. They also need multidisciplinary follow up. Some of the hidden issues of CHARGE syndrome are often forgotten, one being the feeding adaptation of these children, which needs an early aggressive approach from a feeding team. As the child develops, challenging behaviors become more common and require adaptation of educational and therapeutic services, including behavioral and pharmacological interventions.

Abnormalities, Multiple↗

[Pregnancy interruption from the pediatric viewpoint].

Therapeutic abortion is foremost a biological problem. The abrupt termination of the physiologic phenomenon of pregnancy is a sudden interference with the biology of the interaction between mother and fetus. Therapeutic abortion is also a psychological, a sociological and a political problem. Today therapeutic abortion is legal under certain condition and done by physicians. This review supports therapeutic abortion for prenatally determined abnormalities which have a scientifically high risk of defective offspring especially trisomy 21. Intensive genetic counselling prior to the therapeutic abortion is necessary. The last decision for the therapeutic abortion rests with the pregnant mother. The genetic medical problem is only part of the decision making since a number of familial, personal, religious and human factors are involved in the decision. The experience of the physician, his world view, his medical education, his knowledge of the psychological and physical and social anxieties of the pregnant patient also modify the decision making. The gynaecologist who carried out the therapeutic abortion needs to take these factors into account. Considering how difficult and depressing the development of severely mentally retarded persons is especially when they reach adulthood and how limited there life is by suffering, limitations and isolation therapeutic abortion is a valuable method to spare parents the stress of caring for severely mentally retarded offspring for years and decades.

Abortion, Therapeutic↗

[Evaluation of the effects of psychomotor reeducation in aged patients with dementia].

Psychomotor re-education has been attempted experimentally with a population of demented patients in a geriatric ward. It draws on the methods employed at the Bel Air Psychiatric Clinic, in particular the principle of transfer of training. To assess the effect, if any, of the therapy, two psychological rating scales devised specially for demented patients, were established by the team of psychologists at the Grenoble Teaching Hospital. These rating scales are based on the theoretical notions of J. de AJURIAGUERRA and colleagues, that is, the concepts of structural analysis, of involution and homogeneity or inhomogeneity. The psychological test rating scale proposed explores four aspects of mental activity : memory, operating mechanisms, visual defects, space perception and orientation. The rating scale is described in detail, with its references, instructions and scoring.

Aged↗

Perceptions of risks of smoking and heavy drinking during pregnancy: 1985 NHIS findings.

As part of the Health Promotion and Disease Prevention Questionnaire administered in the 1985 National Health Interview Survey, nearly 20,000 respondents ages 18-44 answered questions about their awareness of the risks of smoking and heavy drinking during pregnancy. In reference to smoking, interviewers asked about miscarriage, stillbirth, prematurity, and low birth weight; in reference to heavy drinking, they asked about miscarriage, mental retardation, low birth weight, and birth defects, as well as fetal alcohol syndrome. For each of these adverse outcomes, a majority of subjects acknowledged increased risk because of smoking or heavy drinking during pregnancy. The range was 66-80 percent of respondents for the four questions on smoking, with the perceived association to smoking strongest for low birth weight. Approximately 84 percent of respondents associated heavy drinking with increased risk for each of the suggested pregnancy outcomes. Smoking seemed to be perceived to pose a lesser risk to pregnancy than heavy drinking. This relative lack of awareness of the pregnancy risks of smoking was more apparent among respondents with less education and more pronounced among blacks than whites. Women were more likely than men to express some opinion on these pregnancy-related questions and were more cognizant than men of the risks. On this limited survey, Americans ages 18-44 were not very knowledgeable about fetal alcohol syndrome. Among the 55 percent who had heard of fetal alcohol syndrome, fewer than one in four correctly identified it as a set of birth defects when offered three possible definitions. It will be interesting to correlate responses to these "knowledge" questions with NHIS data still forthcoming on reported actual smoking and drinking behavior among women respondents who were recently pregnant.

Adolescent↗

The role of the psychologist in adult congenital heart disease.

In addition to monitoring and treating the cardiac disease, patients benefit from health professionals recognizing and managing the potential psychosocial consequences of growing up with congenital heart disease. Working groups from Europe and North America have emphasized the benefit of inclusion of specialized mental health care for adult congenital heart disease (ACHD) patients. This article reviews the evidence that ACHD patients have special and unique psychosocial needs and outlines ways in which psychologists can be integrated into multidisciplinary ACHD care teams. There are three professional domains in which clinical health psychologists can contribute to an ACHD team: provision of clinical services, multidisciplinary research, and professional education. Considerations for incorporating psychology into ACHD teams are presented.

Adolescent↗

The person and diabetes in psychosocial context.

This review spans life with diabetes from the first year through the succeeding period of relatively good health, into the phase when the patient needs to accommodate for more symptoms and impaired functioning. Focusing on autonomy, vulnerability to fears, and vocational choices, this article compares effects of diabetes on children and youths with effects on those with onset in later life. A concept of biopsychosocial homeostasis helps to explain: (1) why patients turn to a physician when they do; (2) how the physician may often save the patient time and money by helping that patient to change psychological and social components of well-being and functioning--and highlights the importance of these; (3) how to treat depressive disorder; (4) how to discuss worst fears and encourage patients to play with their unpleasant imaginings; and (5) how to reduce hypoglycemia. Repeated hypoglycemic episodes have the cumulative deleterious effect of undercutting the patient's and others' sense that the patient is dependable, with impaired performance and even serious occupational and marital repercussions. Educational tactics are suggested for the patient with IDDM who finds it harder to identify early levels of hypoglycemia; that patient must learn to respond to specific clues of mild hypoglycemia, stemming from defective thinking and emotional and sexual functioning. There is no substitute for a full history and an empathic understanding of the patient's imaginings, relationships, and circumstances.

Adaptation, Psychological↗

Schizophrenia: all in the mind or locked in the brain?

This paper questions whether a natural science language can be transposed either into the care of individuals affected by mental illness or the educational curricula of those preparing to care for them. The importance of biological research into schizophrenia is not denied. However, it is suggested that paradigms which depend upon ownership (of knowledge) may be less worthwhile to schizophrenic people than an approach which rests upon a philosophy of being. In this sense, a consideration of the place of consciousness in investigations into brain function is stated to be relevant: issues of mind and brain are central to discussions about schizophrenia. It is not denied that the laboratory-bench may ultimately unravel genetic susceptibility to schizophrenia. However, a biology of persons - however persuasive its language - can lead, in the case of schizophrenia, to formulations of human defect. Forms of care which proceed from determinism can lead, as they did in the past, to the curtailment of individual aspirations for both carers and patients.

Biological Psychiatry↗

Obstetrician-gynecologists' practice and knowledge regarding spina bifida.

The purpose was to assess practicing obstetrician-gynecologists' knowledge about the prenatal diagnosis and postnatal prognosis of spina bifida. Written questionnaires designed to assess practicing obstetrician-gynecologists' knowledge of spina bifida were mailed to 1000 randomly selected American College of Obstetricians and Gynecologists Fellows. More than 50% did not identify many of the sonographic features indicative of an open neural tube defect in the fetus and more than one third overestimated the risks of stillbirth, whereas more than two thirds overestimated the risk for premature delivery in a pregnancy complicated by fetal spina bifida. Just more than 50% correctly estimated the 1-year survival rate and just less than 50% correctly estimated survival at 6 years. Sixty-six percent overestimated the incidence of mental retardation associated with spina bifida. Maternal-fetal medicine specialists returning the survey exhibited a much better understanding of the prenatal issues and prognostic and outcome factors related to spina bifida. There are gaps in obstetrician-gynecologists' knowledge about the diagnostic features of and prognosis for fetal spina bifida. It is important for them to take advantage of continuing medical education opportunities to learn more about the management of pregnancies complicated by fetal spina bifida and about the prognosis for affected individuals.

Adult↗

Beliefs about causation of schizophrenia: do Indian families believe in supernatural causes?

BACKGROUND: Beliefs about the causation of schizophrenia could influence the attitudes patients' families adopt towards the patient and may also influence their help-seeking behaviour. Indian families have been typically described as often believing in causes like supernatural forces and therefore seeking help from magico-religious healers. In the changing mental health scenario in India, this impression needs verification. METHOD: Key relatives living with 254 chronic schizophrenia patients were interviewed and asked to name the causes they believed were behind the illness. A list of possible causes was provided for the families to select from, and relatives were also encouraged to mention other possible causes, not featured in the list. The possible causes identified and the factors related to attributions made were analysed. RESULTS: A supernatural cause was named by only 12% of the families and as the only cause by 5%. Psychosocial stress was most commonly cited cause, followed by personality defect and heredity. A small number of families (14%) could not name any cause and 39% named more than one cause. Patient gender and education, duration of illness and the key relative's education and the nature of relationship were related to the type of causal attributions made. CONCLUSION: Families living with patients suffering chronic schizophrenia receiving treatment in urban India rarely subscribe to the idea of supernatural causation of the illness. The causal attributions made by them are fairly rational and understandable, given the relative lack of exposure to proper information about the illness.

Adult↗

Effects of prenatal alcohol exposure at school age. I. Physical and cognitive development.

Alcohol is a potent teratogen associated with dysmorphology, growth retardation, and neurological damage in children with the full fetal alcohol syndrome (FAS); alcohol is also associated with growth retardation and behavioral alterations in neonates prenatally exposed to various dosages. Questions remain about the long-term consequences of prenatal alcohol exposure. This study reports on the follow-up of a subsample of 68 children, the majority of whom were low income and black (mean age: 5 years, 10 months) who were first evaluated as neonates. Physical and cognitive outcomes of 25 children of women who drank throughout pregnancy [absolute alcohol (AA)/week: mean = 11.80 oz), even after receiving an educational intervention to stop drinking, were compared with outcomes of children in two contrast groups: a) women (n = 22) who stopped drinking (AA/week: mean = 11.46 oz) in the second trimester after an educational intervention but resumed postpartum; and b) women who did not drink during pregnancy and who drank little postnatally (n = 21). Children were compared for alcohol-related birth defects (ARBDs), growth (height, weight, and head circumference), and cognitive, academic, and adaptive measures. Neonatal and current physical measures were correlated to determine predictability of neonatal status. When the effects of age and gender were controlled, children in the continued-to-drink group showed significantly more ARBDs and had smaller head circumferences than those in the other two groups. When current drinking reported by caretakers was controlled, the children who were exposed throughout pregnancy also showed significant and consistent deficits in several areas of intellectual functioning including sequential processing (short-term memory and encoding) and overall mental processing. Alcohol-exposed children displayed significant deficits in preacademic skills when compared with children of nondrinkers, with both alcohol groups deficient in premath and reading skills. There were no differences in adaptive behavior at follow-up. These data suggest that alcohol exposure throughout pregnancy is correlated with persistent physical differences as well as identifiable deficits in sequential memory processes and specific academic skills. However, even when alcohol use is limited to the first part of pregnancy, significant deficits in academic skills and growth parameters are measurable.

Adult↗

Some etiologic and prognostic factors in early infantile autism and psychosis.

Fifty infants and young preschool children seen in a pediatric developmental service and diagnosed as having "autism" all had evidence of organic disease of the brain and three fourths had mental deficiency of varying degrees. They did not differ in any respect from a comparison group of patients with central nervous system dysfunction unassociated with the symptom complex of autism. Both groups of patients had a high incidence of low birthweight, complications of pregnancy and the neonatal period, seizure disorders, and a variety of specific disease entities associated with developmental defects. Follow-up of 40 of the 45 survivors for a mean of five years showed that none of the patients had had treatment directed to their psychotic symptoms. However, three fourths had established social responses appropriate to their level of function; those who did not generally were over 3 years of age at the time of their first examination or had initial DQs of 35 or less. The degree of mental deficiency was as great or greater at follow-up than it was initially.

Adolescent↗

[Analysis of temporal evolution in alcohol consumption during pregnancy in the Spanish autonomic communities].

BACKGROUND AND OBJECTIVE: The fetal alcohol syndrome (FAS) is considered as the main preventable cause of congenital defects and mental deficiency. We analyzed whether the consumption of alcohol during gestation has changed in Spain. SUBJECTS AND METHOD: We analyzed the evolution over the last 24 years, as well as the geographical distribution according to Spanish regions, of maternal alcohol consumption during pregnancy. We used data from the ECEMC, a registry which includes 1,820,862 liveborns, of whom 30,836 were malformed. A similar number of healthy controls was collected. We established 5 increasing levels of alcohol consumption, for sporadic and diary intakes, and 3 levels of maternal scholarship. RESULTS: Except for the lowest and highest intakes, alcohol consumption levels showed a decreasing trend over the time. These tendencies were similar in almost all Spanish regions. When comparing the intake between regions, the differences were statistically significant. There was a correlation between the maternal scholarship and the alcohol intake, showing that the higher the education the lower the amount of alcohol consumption. CONCLUSIONS: The awareness of the alcohol effects is related to the maternal cultural level. Maternal consumption of alcohol varies across Spanish regions, though its trends are similar.

Alcohol Drinking↗