摘要:

ABSTRACT.The fragile‐X syndrome is the most common cause of familial intellectual disability. Recently, the gene related to the fragile‐X syndrome [the ‘fragile‐X mental retardation’‐1 (FMR‐1) gene] has been isolated. In this review, clinical and molecular aspects of the fragile‐X syndrome, current benefits and future prospect

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00342.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

Abstract.Autopsies were carried out on 60 mentally handicapped patients and the brain was examined in detail in all cases. The clinical records were studied and correlated with the pathological findings, A variety of pathological changes were found in the central nervous system, and there was a high incidence of microcephaly and gross abnormalities among the patients with epilepsy and neurological dysfunction. The incidence of Alzheimer's disease was very high among patients with Down's syndrome, but in those patients without Down's syndrome, the incidence appeared to be much the same as in the general population. The most common cause of death was respiratory disease, followed by cardiovascular disease. There was a high incidence of volvulus among the group with epilepsy. The findings are discussed, and reference made to the long‐term care of the mentally handicapped. The study demonstrates the importance of the autopsy in terms of furthering knowledge in the field of mental handica

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00343.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.A systematic screening for fragile‐X syndrome, using various clinical criteria to preselect for cytogenetic testing, was performed throughout the North East Essex Health District on 1100 people attending three different local services for people with learning disability. The selection procedure used varied from a gestalt impression to head, ear and testis measurement depending on the setting. Fifty‐nine males and five females who met the selection criteria went on to have chromosome studies. Of these, 23 males and one female were positive (more than 4% positive cells). They came from 19 families. Whilst the true prevalence of fragile‐X syndrome is not known in the district,at a minimum, it contributed 3.2% of the institutionalized males (health authority care), 4.4% of the boys and 2.1% of the girls attending special schools for severe learning disability, 7.9% of the boys attending schools for mild learning disability (Local Education Authority), and 3.5% of men attending the two adult training centres within the district (social services). These figures compare well with the yield from reported surveys in which all individuals without a known diagnosis were tested cytogeneti

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00344.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.The effectiveness of computerized handwriting instruction incorporating modelling of the target letter, corrective feedback and reinforcement contingent upon letter accuracy was examined with severely mentally retarded adults. Assisted by faded prompts on a sheet of paper, subjects wrote the letter ‘q’ while their handwriting patterns were detected by a digitized graphics tablet linked to a computer. Computerized instruction was introduced for six subjects (mean mental age = 3 years, 10 months) according to a multiple‐basetine‐across‐subjects design. Instruction produced significant improvements in letter quality which were not simply due to the effects of practice. Subjects who had extended practice before being introduced to the computer were less responsive to the information provided by the computer and did not attain the levels of performance accuracy achieved by other

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00345.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.In a study of 124 hospital residents with a learning disability aged 60 years and over, DSM‐III‐R diagnostic criteria were used to determine the prevalence of dementia (12.9%), mood disorder (8.9%) and schizophrenia (6.5%). The figure for dementia confirms the prevalence given in previous studies, but the figures for mood disorder and schizophrenia are higher. It was found that mood disorder was commoner in the age group 60–69 years (P>0.01) and dementia was commoner in the age group 70–79 years (P>0.01). There were no significant differences in the prevalence of schizophrenia w

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00346.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.The results of a community‐based study measuring the occurrence of movement disorders in a population of people with learning difficulties treated with antipsychotic medication are presented. This group was compared with an age‐ and sex‐matched group with a similar degree of handicap, who were not treated with antipsychotic medication. When medication was given within British National Formulary Guidelines, no significant increase in movement disorders in the treated group was found. The relevance of this to psychiatric practice is disc

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00347.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Sixty‐seven subjects with mild or moderate intellectual disability were assessed on a variety of measures of emotion. All of the measures were self‐report measures and all of the data is based on reports by die subjects' themselves. The battery included the Zung Self‐Rating Anxiety Scale, the Zung Depression Inventory, the General Health Questionnaire and the Eysenck‐Widi‐ers Personality Test. The results reveal an impressive amount of convergent validity in the subjects' emotiona

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00348.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Few reports have described the psychiatric co‐morbidity of Noonan syndrome. While mental retardation is a common feature of Noonan syndrome, the diagnosis of autism using operational criteria has not been reported. In this paper, the authors describe the association of Noonan syndrome with autism. They propose that the co‐morbid diagnosis of autism should be considered during the clinical assessment of persons with Noonan syndr

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00349.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.Manic episodes have seldom been reported in individuals with Down's syndrome. The case of an individual with Down's syndrome who developed a manic episode in later life is described. This manic episode was subsequently followed by a first depressive episode.

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00350.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY

摘要:

ABSTRACT.A case of erotic delusion and concurrent somatic sexual hallucination is described in a 66‐year‐old mentally handicapped woman. The history, mental state and biological investigations convey the impression of erotomania, or de Clerambault's syndrome. Treatment with Pimozide has resulted in a remarkable improvement such that the patient has been symptom free for 8 months at the time of reporting. In the authors' opinion, the case lends support to the argument that erotomania exists as a mono‐delusional disorder and is equivalent to the DSM‐III‐R classification of Paranoid Disorder, Erotom

ISSN:0964-2633    

DOI:10.1111/j.1365-2788.1994.tb00351.x

出版商:Blackwell Publishing Ltd    

年代:1994

数据来源: WILEY