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Fragile X syndrome--a review.

Since the discovery of the Fragile X syndrome some 20 years ago, there have been numerous articles concerning clinical features, cognitive profile and transmission. It is a syndrome of importance to all those working in the mental health field and those involved in community health, such as General Practitioners, since it is second only to Down syndrome as a genetic cause of mental handicap.

Abnormalities, Multiple↗

Nonspeaking children in schools for children with severe mental disabilities in the greater Pretoria area: implications for speech-language therapists.

The aim of this study was to describe children enrolled in registered schools for children with severe mental disabilities in the greater Pretoria area in order to compile a profile of nonspeaking children. Emphasis was placed on the prevalence of nonspeaking children as well as on their functioning in different skill areas in order to assess the need for service delivery. Two questionnaires were developed; the first for obtaining biographical data from teachers; the second for obtaining information on the communication and related abilities of children between 3-12 years. The particular teachers completed the questionnaires in conjunction with fieldworkers. Results indicated a high prevalence (38%) of nonspeaking children in schools for children with severe mental disabilities in the Pretoria area and also indicated that they were a heterogeneous group regarding communication and related abilities. This survey was the first step in determining prevalence and describing nonspeaking children in schools for children with severe mental disabilities in South Africa. Results also indicated that these children are in great need of additional service delivery with special reference to augmentative and alternative communication (AAC) strategy implementation.

Adolescent↗

[Spoken and written language abilities of remedial class children placed back into mainstream education].

Current literature emphasizes the role of the speech-language therapist with regard to language learning disabled children. A lack of research regarding the language abilities of the older language learning disabled child was, however, identified. The possibility of continued spoken and written language problems in aid class children who have been placed back into mainstream education, served as a motivation for this research project. The empirical survey was administered on twenty-eight Afrikaans and English speaking ex-aid class children. According to experimental aims the subjects' level of performance in spoken and written language and scholastic skills were analysed and described qualitatively and quantitatively, through the Test for Oral Language Production and the South African Written Language Test. The findings indicate that a significant percentage of the subjects evidenced spoken and written language problems and moderate difficulties in the school subjects Afrikaans and English. Meaningful correlations were found to exist between spoken and written language, and language skills and scholastic performance. Implications for dealing with the language learning disabled child are discussed, and continuation of speech-language intervention with aid class children who have been placed back into mainstream education, is strongly recommended. A need for further research with regard to the older language learning disabled child is expressed in order to support and expand these results.

Child↗

The effect of language knowledge on speech perception: what are we really assessing?

OBJECTIVE: The authors examined whether open-set speech perception scores are limited by knowledge of vocabulary and syntax and further considered whether remediation of vocabulary and syntax will increase open-set speech perception scores. STUDY DESIGN: This was a repeated-measures study design in the setting of a primary (elementary) school for the hearing-impaired. PATIENTS: The study population was composed of three hearing-impaired children using Nucleus 22-channel cochlear implant. INTERVENTION: Intervention used was language remediation sessions. MAIN OUTCOME MEASURES: The main outcome measures were assessment of auditory-alone speech perception benefit using open-set words and sentences and assessment of syntactic knowledge using the Test of Syntactic Ability. Outcome measures were applied before and after remediation. RESULTS: Child 1 and child 2 showed a significant postremediation improvement in their overall scores on the Test of Syntactic Ability and in their ability to perceive words learned during remediation. Child 1 and child 2 also showed a significant improvement in their scores on a modified Bamford-Kowal-Bench open-set sentence test, which specifically targeted grammatical constructs trained in remediation sessions. CONCLUSIONS: Remediation of language knowledge deficits significantly improved open-set speech perception for two children, suggesting a need to include language remediation in cochlear implant habilitation programs.

Adolescent↗

[The evaluation of aphasic deficits for the definition of a targetted logotherapeutic treatment].

The Aachener Aphasie Test (AAT) is the major German test for the diagnosis of aphasic disorders. The test is easy to use and is valid and reliable for the diagnosis of aphasia and its severity and to evaluate the recovery of the aphasic disorder after language rehabilitation. The AAT is, however, not sufficient to define cognitively sound logotherapeutic treatment. The use of tasks which are based on cognitive functional models allows the identification of specific processing levels that have been damaged by a cerebral lesion, and the definition of a focussed rehabilitation plan. In this paper, we will discuss the results of a cognitive neurolinguistic treatment in a patient who suffered of Broca's aphasia with agrammatism and phonological dyslexia.

Adult↗

A neurolinguistic longitudinal study of a pure motor aphasia.

We have made a detailed neurolinguistic study of a patient with motor aphasia. The method used for his reeducation and the variants found in his language from a linguistic point of view through the course of several months are described. During this study we have found similarities with cases studied by Marcie reached by him in the errors common in patients with motor aphasia. But, contrary to Marcies, findings, we have found phonemes and allophones outside of the phonological and/or dilectal system of the language in this area. These phonemes and allophones are described together with the environment in which they appear.

Adult↗

Visual speech aids: a British survey of use and evaluation by speech and language therapists.

One hundred and eighty speech and language therapists throughout the UK completed a postal questionnaire which asked for ratings of visual speech aids (VSAs) on a number of dimensions together with information regarding frequency of use of such aids, comments on the range of applicability of each type of aid and other demographic data relating to the diversity of client groups covered in case loads. The results (using analysis of variance) revealed a main effect of VSA type. There was also a main effect of question type; all VSAs scored poorly on some dimensions as compared with others. Furthermore there was a significant interaction between type of VSA and dimension type. This suggests that VSAs not only tend to be built for particular client groups, but that the makers tend to focus on some specific design criteria to the detriment of others. These results are discussed with respect to the future design of VSAs, and with respect to their testing and future evaluation.

Analysis of Variance↗

A narrative therapy approach to counseling: a model for working with adolescents and adults with language-literacy deficits.

PURPOSE: Remediation efforts with adolescents and adults with language-literacy deficits (LLD) may be hindered by concomitant factors such as decreased self-esteem and self-efficacy. Despite sound linguistically based remediation practices, treatment lacking integrated counseling components may fail to achieve optimal outcomes. In this tutorial, we recount a counseling approach, specifically a narrative therapy counseling approach based on constructivist theory, to be used with adolescents and adults with LLD. METHOD: A review of the literature includes research on the use of counseling in communication disorders settings and on the application of a narrative therapy counseling approach with individuals with decreased self-esteem. The key components and steps of narrative therapy are described, and applications for use with adolescents and adults with LLD are suggested. Sample vignettes of a counseling component for a 22-year-old student with LLD who is enrolled in literacy intervention are used to illustrate some of the key points of a narrative therapy counseling approach. IMPLICATIONS: A narrative therapy counseling approach that is integrated into language-literacy remediation may be an effective way of addressing concomitant factors such as decreased self-esteem in adolescents and adults with LLD. Readers of this article are challenged to seek additional counseling training and to begin to conduct research to determine the feasibility, effectiveness, and efficacy of integrating a counseling component into speech and language therapy.

Adolescent↗

Current and future therapy in muscular dystrophy; need for a common language between basic scientists and clinicians.

This paper reviews the current therapy of Duchenne dystrophy, with the only two effective means of prolonging ambulation being the provision of knee-ankle-foot orthoses, and administration of corticosteroids, and the prospects, for the future application in Duchenne dystrophy, of ongoing research in cell therapy, gene therapy, protein upregulation and pharmacological possibilities. A critical review is also provided on the inappropriate nomenclature currently used and the need for a common language between basic and clinical scientists. Amongst the major problems are equating the mdx mouse, with its mild clinical phenotype, and Duchenne dystrophy, with its clearly defined severe clinical phenotype; the use of inappropriate and often emotive terminology to describe the pathological changes, such as "rescue", "reversal", "prevention", in place of describing the actual changes in acceptable descriptive language; and the use of the term therapy, in place of experiments, both in the basic laboratory studies and also the clinical experiments of injection of cells or gene constructs into single small muscles. A missing link in the multidisciplinary efforts, in this field, is the almost total absence of mouse doctors, who can define, at a clinical level, the motor, respiratory and cardiac deficits in the dystrophic animal, and bridge the gap between the mouse scientists doing experimental studies in the laboratory and the clinicians and veterinarians involved in the care of humans and dogs with these disorders and speaking the same language in relation to the clinical aspects and the pathology.

Adrenal Cortex Hormones↗

A treatable language disorder: pharmacological treatment of pervasive developmental disorder.

Results of treatment of four patients are described. All of the patients had pervasive developmental disorder (PDD), a tic disorder, and a characteristic pattern of speech and language impairment. The patients were treated with haloperidol for their tic disorders, and concomitant with the reduction of the frequency and severity of tics was marked improvement in language. The patients averaged 3 months of language gain for each week of speech therapy directly after the initiation of haloperidol treatment for tics. Progress in speech and language therapy was extremely slow during the years prior to treatment with haloperidol. To the authors' knowledge, no language disorder has been described in the literature which shows such a predictable and marked response to pharmacological treatment. The authors hypothesize that tic disorders in individuals with PDD may be a marker for a more positive response to dopamine antagonists like haloperidol.

Child↗