摘要:

ABSTRACT.Significant hearing loss and external pinna malformations are two of the most common defects evident in Down's syndrome. The external and middle ears are linked embryologically, both arising from the first and second branchial arches. Evidence indicates that the majority of hearing loss in Down's syndrome is conductive in nature, originating from malformations of the middle ear ossicles and/or the eustachian tube. Recent studies also have indicated that hearing loss is a contributing factor to the IQ and learning deficits that afflict most individuals with Down's syndrome. Therefore, an early, external diagnostic feature for predicting conductive hearing loss would be desirable. In the current study, people with Down's syndrome, people with non‐Down's mental retardation and control subjects were examined in a clinical environment for the presence of hearing loss and pinna defects. It was found that 90% of the Down's syndrome population had significant hearing loss, compared to slightly more than 50% in the non‐Down's group and no hearing loss in the controls. Also, the majority of hearing loss among individuals with Down's syndrome was conductive, while all hearing loss in the non‐Down's group was sensorineural The Down's syndrome population exhibited nearly 3.5 pinna defects per ear, with malformations of the helix being very evident. The non‐Down's population exhibited 2.5 pinna defects per ear, with concha defects being the most

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00456.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.The use of antiepileptic agents for individuals with learning disabilities (mental handicap) resident both within National Health Service facilities and the community was surveyed in the UK. There was no difference in rates of polypharmacy, but there were significant differences in choice of antiepileptic agent. In particular, individuals resident in the community were more likely to be in receipt of phenytoin, primidone and phenobarbitone, which are particularly recognized as producing adverse effects on cognition and behaviour

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00457.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Eighteen people with learning disabilities who moved from hospital to community‐based homes were compared with a matched group of 18 who did not. A week‐long time budget diary was used to record each occasion that the person left their home, the people accompanying them on each trip, their mode of transport and their destination. The diary was completed before they left the hospital and was repeated at least 1 year later. There was a strong correlation between the number of trips made from hospital wards at baseline and the number made at follow‐up in both groups. Moving from the hospital had no significant effect on the number of trips made, but it did change their nature. Movers made more trips to unsegregated facilities; they made fewer trips alone and more in groups which included both staff and resi

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00458.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.The prescribing of medicines for people with learning disabilities living in Leicestershire, England, was surveyed. Prescribing in both National Health Service (NHS) and non‐NHS settings was compare

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00459.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.The complexity of need experienced by people with learning disabilities might best be described by a multi‐axial classification. The data routinely collected for a register of people with learning disabilities were analysed to see whether factors that might discriminate between individuals could be identified. Three factors were identified. The factor scores were used in a cluster analysis. A ten‐cluster model formed from these factors made empirical sense. The present investigation indicates that a multi‐axial classification is feasible and may be useful. However, the results cannot be applied beyond the data set used for its development at the present time. Ultimately, it will be necessary to collect additional information in order to calibrate the factor s

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00460.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Mildly retarded adults, equal mental age nonretarded children, high mental age nonretarded children, equal chronological age nonretarded adults and young nonretarded adults were required to perform a priming task (letters/digits) in whichsomeprimes were masked (visual noise mask) at just below detection levels to assess automatic processing. The critical (just below detection level) prime‐mask stimulus onset asynchronies (SOAs) were established individually for each subject using the method of limits and reassessed after the experimental trials. The mean critical SOA for each of the five groups was comparable and the critical SOAs remained stable across the 120 experimental trials for all groups. The nonretarded adult subjects demonstrated semantic, categorical and orthographic priming. The mildly retarded, the equal‐MA and the high‐MA groups failed to demonstrate priming, and in fact, demonstrated superior performance for prime‐target conditions which should have been poorest. This finding was discussed in terms of the level of specificity engendered in the priming task. Under the mask procedure, the nonretarded adult groups demonstrated semantic (letter) priming and orthographic priming, suggesting that letters (not digits) function as an analog to words which were employed in earlier masked prime lexical decision tasks. The mentally retarded, the equal‐MA and the high‐MA groups again failed to demonstrate priming under the ma

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00461.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Several authors have suggested the existence of an inverse relationship between bipolar affective disorder and Down's syndrome (DS). The present authors have examined this hypothesis by a critical review of the literature. The present findings are consistent with a reduced rate of bipolar disorder in subjects with DS when compared with non‐DS mentally retarded adults and with the general population. Thus, possession of an extra copy of chromosome 21 may confer protection against bipolar disorder. This could be the result of non‐specific mechanisms or the action of a disease‐modifying gene. However, the most interesting possibility is that either dominant or recessive alleles act at a major susceptibility locus for bipolar disorder on chromosome 21. Testable predictions result from the major susceptibility locus models. In order to investigate these hypotheses further, the present authors suggest the following: (1) further studies of the prevalence of bipolar disorder in DS; and (2) the reporting of all cases of bipolar disorder in trisomy 21 with details of the meiotic origin of the non‐disjunction and details about affective disorder in relatives of the

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00462.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Prader‐Willi syndrome presents behavioural characteristics at the temperamental level which can be described as oppositional, explosive and at times antisocial. These traits may fluctuate and be driven by unknown biological factors as well environmental variables. Obsessive compulsive traits and other anxiety symptoms are also frequent. Delusional psychotic thinking is manifest in some cases, but may be latent in several such patients. A third set of manifestations is a ‘refusal‐lethargy’ syndrome of akinesis, refusal of food and drink, and soiling, which seems to be triggered by environmental circumstances but resembles the hypersomnic, lethargic depressions. These three sets of phenomena are documented through the clinical observation of nine cases and may be useful in the study of the genotype‐phenotype relationship in this and other syndromes, particularly those in which similar manifestations are observed and cyclic changes are seen. The use of drugs in this syndrome can also become more rational if this classification is used to identify clearer targets for treatment. The possibility that most manifestations of the syndrome may be an expression of a hyposerotonergic defect is

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00463.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00464.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00465.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY