摘要:

A batiery of neuropsychological tests, developed to study patterns of age‐related differences in adults with Down's syndrume (DS), was administered to 10 DS adults over age 35 and 19 younger DS adults. Although all adults with DS are reported to develop the neuropathoiogical changes of Alzheimer's disease by the fourth decade of life, only four of the 10 old DS adults were judged to have dementia based on clinical criteria. Demented old DS adults had global neuropsychological deficits, as indicated by significant differences on all functions tested except some simple language functions. In contrast, non‐demented old adults had a selective pattern of neuropsychological reductions relative to young adults. Ability to form new long‐term memories and visuospatial construction were consistently diminished, whereas immediate memory span and language were not. The global mental decline observed in demented adults suggests a stage of disease progression that corresponds to severe dementia in pre‐ morbidly normal adults with dementia of the Alzheimer type (DAT). The prominent long‐term memory impairment with selective reductions of nonmemory functions in non‐demented adults suggests a correspondence to early and inter

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01467.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.Basal metabolic rale (BMR) was measured on 2 consecutive Jays with an open‐circuit, indirect calorimeter in 16 healthy subjects with trisomy 21 Down's syndrome (DS) aged 22‐35 years and 11 healthy control volunteers aged 20‐31 years. BMR measurements Jut mil differ on consecutive days in either the DS or control groups. Absolute BMR, BMR corrected for surface area, and HMR corrected tor lean body mass did not differ between the DS subjects and the volunteers. The per cent of predicted BMR also did not Jitter between the two groups. These results suggest that systems controlling basal metabolism are not affected by extra gcnomic material from chromoso

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01468.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.This paper reports on a study of the communicative competence of adults with a mental handicap living in hospital and community settings. One hundred and twenty subjects, half from hospital settings and half from community settings, were assessed on the Communication Assessment profile (CASP). Significant differences were found between the hospital and community groups on pragmatic competence. The communication environment was shown to be an important determinant of pragmatic skills. The implications of this finding are discussed.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01469.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.This study investigated emotional awareness, the recognition and understanding of different emotional states, among a non‐clinical population of adolescents and young adults with mental handicaps. While emotional awareness showed a high positive correlation with language comprehension, the results suggest that many of these individuals have specific emotional awareness deficits which are not in line with their language comprehension abilities. This finding suggests that the assessment of emotional awareness would be an important step in the choice of a self‐report measure to assess an individual's emotional st

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01470.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.A case of Prader Willi Syndrome who suffered from hypothyroidism is described. This patient on cytogenetic examination was found to have Mosaic 46,XX/46,XX,det(15Kq11.1q11.2) karyotype.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01471.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.This paper describes an attempt to treat Gilles de la Tourette syndrome (GTS) in a severely mentally handicapped girl, using a cuc‐controlled relaxation technique. Response to treatment followed a pattern frequently found in the general clinical literature. Namely tic frequency decreased during relaxation sessions but there was no generalization of effects outside of relaxation sessions. Pharmacological intervention, using clonidine, also did not reduce tic frequency. However, pimozide proved immediately effective in suppressing tics and this improvement was maintained at follow‐up. Medication side effects were noted and implications for long‐term treatment of GTS are disc

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01472.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.A case of a mentally handicapped female with unbalanced form of translocation deletion between chromosomes 6 and 22 is reported. Clinical features, mental disorder and abnormal dermatoglyphs of the propositus are described.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01473.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.Secondary Cystathioninuria is associated with various pathological conditions (Gjessing, 1963; Gjessing&Mauritzen, 1965; Endres&Wuttge, 1978). In many cases, Cystathioninuria has been associated with mental retardation (Harris el al., 1959; Robbet al., 1984). As far as the authors know, Cystathioninuria has not previously been described in Down's syndrome. In 1981, in the authors' institution for the mentally retarded, all patients with Down's syndrome were screened with regard to aminoaciduria, using thin layer chromatography. In the course of this process, a case of Cystathioninuria was discovered. The results are presented in detail.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01474.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.A case of Miller‐Dieker syndrome is reported. A 6‐month‐old baby girl has the characteristics of the syndrome. Post natal growth deficiency: microcephaly, profound mental retardation. Craniofacial defects: micrognathia, ear abnormalities, anteverted nares, wrinkling of forehead, bitemporal growing of high forehead. Neurological abnormalities: opisthotonos, epilepsy, initial hypotonia. The literature is rev

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01475.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY

摘要:

ABSTRACT.Urinary taurine levels were estimated in 29 mentally retarded children. These levels were then compared with those of normal healthy children. The urinary taurine levels were significantly higher in the mentally retarded subjects. There is probably an intriguing relationship between taurine levels and mental retardation.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1989.tb01476.x

出版商:Blackwell Publishing Ltd    

年代:1989

数据来源: WILEY