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Learning disabilities and attentional problems in boys with the fragile X syndrome.

The fragile X syndrome is a relatively common form of mental retardation that tends to affect boys more severely than girls. The syndrome is associated with a fragile site at q27 on the X chromosome and with physical features including large or prominent ears and macro-orchidism. Four boys had physical and cytogenetic features of the fragile X syndrome. However, the IQ scores of these patients extended into the normal range. All four patients demonstrated similar learning difficulties that included hyperactivity, visuomotor incoordination, language deficits, and academic delays in mathematics. The fragile X syndrome should be considered in the differential diagnosis of learning disabled children.

Attention Deficit Disorder with Hyperactivity↗

Anger management training: the effects of a structured programme on the self-reported anger experience of forensic inpatients with learning disability.

Within the current political climate, there is an increasing burden on mental health professionals to achieve accuracy in risk assessment and prediction. The accurate assessment and treatment of anger can make a valuable contribution towards alleviating this burden as part of a comprehensive treatment package. This study describes an anger management training programme provided to a group of three forensic inpatients with learning disability. An ABA single case study design was used, with anger levels assessed at weekly intervals before, during and after the programme. The results suggested that anger management training is useful with this client group, with established tools, such as the Novaco Anger Scale, the Spielberger State-Trait Anger Expression Inventory and the Modified Overt Aggression Scale, allowing the impact of the programme on anger levels to be evaluated. There is an indication that maintenance treatment is required to prevent anger levels increasing to pretest levels following treatment.

Adult↗

Executive dysfunction can explain word-list learning disability in very mild Alzheimer's disease: the Tajiri project.

Elderly people with questionable dementia (i.e. a Clinical Dementia Rating (CDR) of 0.5) have been focused on as representing the borderline zone condition between healthy people and dementia patients. Many of them are known to have pathologic traits of very mild Alzheimer's disease (AD). Although they present mild memory disorder, the underlying mechanism has not been fully investigated. Herein is reported the mechanism of learning disability in very mild AD. Eighty-six CDR 0.5 participants and 101 age- and education-matched healthy controls (CDR 0) were randomly selected from a community in the town of Tajiri, Miyagi Prefecture. The word-recall task of the Alzheimer Disease Assessment Scale-Japanese (i.e. learning and recall of 10 words) was administered. The numbers of words recalled in each trial and those never recalled throughout the trials were compared for the two CDR groups. The serial-position function was depicted for three parts (i.e. primary, middle, and recency). The CDR 0.5 group recalled significantly fewer words than the CDR 0 group. The number of never-recalled words was greater in the CDR 0.5 group. A remarkable difference was found in the middle part of the word list. The number of never-recalled words of the CDR 0.5 group was greater in the middle part. The large number of never-recalled words accounted for the poor learning performance of very mild AD participants. The results suggested that very mild AD participants have difficulty in learning and retaining words in the middle part of the word-list because of a functional decline of the central executive system.

Aged↗

Self-determination instructional strategies for youth with learning disabilities.

The concept of self-determination is rapidly gaining attention and acceptance within the disability fields. The focus on self-determination is particularly strong in the transition-from-school-to-adulthood movement. The recent Individuals With Disabilities Education Act of 1990 mandated that students with disabilities be involved in the development of their transition plans and that students' preferences and interests be taken into account as transition plans are developed. This article describes four self-determination models and examines several instructional programs and strategies to promote self-determination in students with learning disabilities. Emerging issues in self-determination are examined, and the research and policy implications of these issues are discussed. These issues include (a) redefinition of roles, (b) the importance of taking risks, (c) individual versus group orientation for self-determination, and (d) the importance of early experiences.

Curriculum↗

The use of anti-psychotic drugs with adults with learning disabilities and challenging behaviour.

The use of anti-psychotic medication with an adult population of people with learning disabilities and challenging behaviours was investigated as part of an epidemiological study covering seven district health authorities and corresponding local authorities in North West England. The study found a high rate of prescription of anti-psychotic drugs (48.1%). Chlorpromazine was the most frequently prescribed drug, followed by Thioridazine and Haloperidol. Three variables, psychiatric diagnosis, where the person was resident (hospital disturbed ward, hospital non-disturbed ward, hostel or family home) and district of origin were found to be significant determinants of prescriptions when all other variables were controlled. Of the variables reflecting individual characteristics those significantly related to prescription suggested that the socially disruptive effects of challenging behaviour were determining prescription. The results are discussed in the context of differing prescription practices across residence and district in the context of the management of socially disruptive behaviour.

Activities of Daily Living↗

Are working memory deficits in readers with learning disabilities hard to change?

This study investigated whether changes in the working memory (WM) performance of readers with learning disabilities (LD) is related to a general or domain-specific system. The study compared readers with LD, chronologically age-matched (CA-M), and reading level-matched (RL-M) children's WM performance for phonological, visual-spatial, and semantic information under initial (no probes or cues), gain (cues that bring performance to an asymptotic level), and maintenance (asymptotic conditions without cues) conditions. The main findings indicated that (a) CA-M children were superior in performance to readers with LD across initial, gain, and maintenance conditions, (b) readers with LD showed less change (as reflected in effect size scores, slopes for the quadratic curve) on both visual-spatial and verbal (phonological and semantic) WM tasks across gain and maintenance conditions than the CA-matched children, and (c) the performance of readers with LD was superior to the RL-M children's performance on initial conditions, but inferior on gain and maintenance conditions. Taken together, the results suggest that a general system moderated the changes in retrieval of phonological, visual-spatial, and semantic information in readers with LD.

Adolescent↗

Breast awareness project for women with a learning disability.

In 1995/6, while conducting a quality assurance evaluation in a residential group home for adults with a learning disability, the subject of breast screening and the importance of early detection of breast abnormalities was raised by a member of staff. At the time, the Mulberry Trust had a quality standard that women between 50 and 64 years who are supported in continuing care should attend breast screening clinics on a 3-yearly basis if they wish. A pilot scheme was devised based on the premise that breast awareness for service users should be promoted. It was decided that the scheme should include a breast examination, conducted on a monthly basis, ideally by the service users themselves, or by suitably trained staff on their behalf. The pilot scheme was implemented using available research and training was provided for qualified nursing staff within the trust. The training covered breast cancer prevention and breast awareness. After 7 months the scheme was evaluated and changes were made to policy and practice, including consultation with the trust's ethics panel regarding implementation of procedures. Training for staff in breast awareness continues and the scheme is slowly being introduced across the trust to enable all service users to be involved, both in residential homes and community settings. However, in future, the emphasis will be on identifying changes in the breast during normal care routines, such as bathing and dressing, as opposed to formal, clinical examination.

Adult↗

Neuropsychological reference groups for 9- through 14-year-old subjects with identified learning disabilities.

The neuropsychological reference groups reported in this study are based on a sample of 1,325 learning-disabled subjects aged 9 through 14 drawn from a midsize midwestern metropolitan community. The sample was composed of 1,006 males and 319 females. Separate means and standard deviations, reported for each gender and age group, were generated for 31 measures ordinarily included as part of the Halstead Neuropsychological Test Battery for Children. Several comparisons were made with other norms previously reported. The need for additional neuropsychological reference groups for children is discussed.

Journal Article↗

[Clinical correlation of hypnagogic hypersynchrony during sleep in normal children and those with learning disability].

INTRODUCTION: One of the electroencephalographic (EEG) patterns that can be mistaken for paroxysmal clinical activity, when not taken into account and especially in children, is hypnagogic hypersynchrony (HH). This consists in generalised, paroxysmal, synchronic, symmetrical, slow, high voltage waves lasting 2 8 seconds, which appear in drowsiness and in stage I. It was observed that this pattern often appeared in children with learning disability (LD). AIMS. To correlate clinical data with the presence of HH during sleep in normal children and those with LD. PATIENTS AND METHODS: We assessed 180 children between the ages of 6 12 years with normal neurological development, 130 of which suffered LD and 50 who did not have LD. EEG was performed with sleep deprivation, following the International Federation of Clinical Neurophysiology guidelines. RESULTS: The presence or absence of HH, together with its characteristics, was assessed. Of the children with LD, 35.38% displayed HH and of the children without LD, only 4% displayed HH. Since the characteristics of HH in the children with LD were different to previous descriptions, we put forward criteria with which to evaluate those differences. CONCLUSIONS: HH appeared more often in children with LD than in normal children. Qualitative, quantitative (p< 0.05) and morphological changes were found in the paroxysmal activity of HH during the stages of sleep in children with LD.

Child↗

Solving the enigma: toilet training children with learning disabilities.

Promoting continence with children with a learning difficulty has traditionally taken second place to managing their other needs. Previous studies have given the impression that children are not ready for toilet training unless they are able to experience awareness of self and respond to the stimulus of a full bladder. This has resulted in assumptions regarding the ability of some children with learning difficulties to become toilet trained and consequently they are 'labelled' as incontinent and automatically issued with nappies. This approach means that any underlying problems such as constipation are not addressed. By taking a different approach, and focusing on assessing bladder and bowel maturation the author has found that children who were once considered incapable of toilet training can become continent.

Child↗

Misconceptions in Van den Broeck's representation of misconceptions about learning disability research.

The methods and conclusions of Van den Broeck (in this issue) are evaluated from two perspectives: (a) statistical considerations and (b) theoretical models of IQ and achievement, specifically reading achievement. We consider the statistical model proposed by Van den Broeck for the regression-based discrepancy model (RDM) to be either irrelevant or conceptually inconsistent with current models of IQ and achievement. The resulting simulation produced exemplar cases that are not realistic in terms of practice. The theoretical representations of IQ and achievement were, in our understanding, inconsistent with contemporary models of either. We suggest that acceptable models support the use of the RDM as it has been proposed by us and by others as one component of the determination of the presence or absence of a learning disability.

Child↗

Familial syndrome of progressive arterial occlusive disease consistent with fibromuscular dysplasia, hypertension, congenital cardiac defects, bone fragility, brachysyndactyly, and learning disabilities.

We report on 4 of 9 sibs with a syndrome of stenosis of the renal arteries and chronic hypertension, variable stenosis or occlusion of cerebral, abdominal and probably coronary arteries due to suspected fibromuscular dysplasia, congenital cardiac abnormalities, brachydactyly and syndactyly of the hands and feet, and increased bone fragility consistent with a mild form of osteogenesis imperfecta. Three affected individuals have had mild to moderate learning disabilities. The parents and the remaining 5 sibs have normal hands and feet and no history of excessive fractures. Individual components of this syndrome may appear as isolated conditions, including fibromuscular dysplasia, brachydactyly, syndactyly, and osteogenesis imperfecta, and are autosomal dominant traits in many cases. Explanations for this familial occurrence include autosomal recessive inheritance, autosomal dominant inheritance with decreased penetrance, or parental gonadal mosaicism for a mutation involving a single gene or several contiguous genes.

Adolescent↗

Summer holiday respite provision for the families of children and young people with learning disabilities.

While the provision of respite care is a relatively recent development in the provision of services to families, it is an area of considerable growth and apparent importance. Most service development and research has focused on the provision of residential or family-based respite care though some studies have commented on the apparent demand for other kinds of relief. This study describes a pilot summer playscheme for children with learning disabilities and the reactions of mothers to its provision. The characteristics of families who used the scheme are described and their felt needs for additional respite care explored. Mothers found the scheme very useful and expressed felt needs for substantially more such provision both during the summer and at other times. The findings are discussed in the context of the importance of developing respite services which meet the varied needs of families.

Adaptation, Psychological↗

Learning disabilities in alcohol-dependent adults: a preliminary study.

To determine if neuropsychological deficits, known to precede alcohol use in those genetically predisposed to alcoholism, were present in an alcoholic population, 25 male alcoholics (mean age 41.1) were interviewed concerning alcohol usage, educational difficulties in elementary school, and family history of alcoholism. Using a regression table, discrepancies between current IQ on the Wechsler Adult Intelligence Scale-Revised (WAIS-R) (Wechsler, 1981) and achievement on the Woodcock-Johnson Psycho-Educational Battery (WJB) (Woodcock & Johnson, 1977) were calculated to determine present learning status. Forty percent of the alcoholics were found to have had special education, remedial services, or repeated grade failure concurrent with a familial history of alcoholism and current discrepancies indicative of learning disability. There were no significant differences on intelligence, years of drinking, or mean grades completed in school between this group and the rest of the subjects who did not receive services in school. Conclusions were that childhood learning disorders may be related to the development of alcoholism, particularly when alcoholism is in the family, and that special educators have a role to play in the prevention and treatment of alcoholism.

Adult↗

Mapping studies on a pericentric inversion (18) (p11.31 q21.1) in a family with both schizophrenia and learning disability.

Chromosomal abnormalities that co-occur with psychiatric disorders can be useful direct pointers to the locus of susceptibility genes. Two families with pericentric inversions of chromosome 18, inv 18(p11.3 q21.1) and psychiatric illness have previously been described. We have fine mapped the chromosomal breakpoints of the rearrangement in a clinically well, inversion carrier from one of these families where other inversion carriers suffered from chronic schizophrenia or severe learning disability. Yeast artificial chromosomes (YACs) from the Whitehead/MIT physical maps of human chromosome 18 have been positioned relative to the chromosomal breakpoints and a number of YACs that span these breakpoints have been identified. Linkage and association studies have previously suggested these regions of chromosome 18q and 18p as candidate loci harbouring genes involved in bipolar disorder and schizophrenia.

Chromosome Inversion↗

Minor physical anomalies in normal, neurotic, learning disabled, and severely disturbed children.

A high incidence of minor physical anomalies in a childhood schizophrenic population has been previously reported by Goldfarb. In the present study, 108 boys from four different clinical populations were examined, utilizing a standardized anomaly scoring system for which a high interrater reliability was obtained. The patient populations were: general pediatric ward patients (n = 31), psychoneurotic outpatients at a university child guidance clinic (n = 26), learning diabled children (n = 23), and autistic, borderline, and atypical children (n = 28) from two residential treatment centers. Both the learning disabled and residential treatment populations had higher mean anomaly scores than did the first two groups, but did not differ significantly from each other. There was a trend for patients with multiple anomalies to have had more frequent history of prenatal insults or paternal psychopathology. These results indicate that the development of these minor anatomical anomalies which are formed in the first three months of fetal development may parallel early developmental deviation of the central nervous system. The finding of high anomalies in the residential treatment groups supports the idea that some of these patients share a common etiology with the other early developmental deviations, such as speech delay or mental retardation, for which high anomaly scores have also been reported.

Abnormalities, Multiple↗

Learning disability and epilepsy in an epidemiological sample of individuals with tuberous sclerosis complex.

BACKGROUND: Intellectual impairments are a recognized feature of tuberous sclerosis complex (TSC), but the frequency and degree of intellectual impairments has not been systematically studied in large epidemiological samples using standardized measures. As such, the form of the IQ distribution (uni- or bi-modal) has not been established and the relationship between IQ and other features (e.g. epilepsy history) is poorly delineated. To address these shortcomings, we assessed the intellectual abilities of a large epidemiological sample of individuals with TSC, drawn from the 'Wessex' area of SW England and compared them with the abilities of their unaffected siblings. METHOD: Standardized tests were used to estimate the abilities of 108 (56 males, 52 females, median age = 25, range = 4-75) individuals with TSC and 29 unaffected siblings (14 males, 15 females, median age = 18, range = 6-55). Seizure history was obtained from informants and medical records. RESULTS: Estimated IQ was bi-modally distributed: 55.5% had an IQ in the normal range; 14% had mild to severe impairments: and 30.5% had profound disability (IQ < 21). Forty-four per cent of the individuals with TSC had an IQ < 70. In the subset of normally intelligent individuals with TSC, IQ was normally distributed with a mean of 93.6. This mean was significantly lower than the mean IQ of unaffected siblings (IQ = 105.6). All individuals with learning disability had a history of seizures that usually commenced before 12 months of age and that often presented as infantile spasms. Multivariate analyses indicated that a history of seizures as well as a history of infantile spasms was predictive of the degree of intellectual impairment. CONCLUSIONS: Intellectual abilities were bi-modally distributed in a representative sample of individuals with TSC. The likelihood of impairment was associated with a history of seizures, particularly infantile spasms. The genetic and brain basis of these findings requires further investigation.

Adolescent↗