[Communication and language problems in children with nemaline myopathy]. / Dificultades de comunicacion y lenguaje en niños con miopatia nemalinica.

INTRODUCTION: Nemaline myopathy is a rare disease with an incidence of 1 in every 50,000 live births. It is the most prevalent of the congenital myopathies, a heterogeneous set of neuromuscular disorders present at birth or manifesting at a very early age, which affect the skeletal muscles and give rise to weakness, hypotonia and psychomotor retardation, although cognitive development remains normal. AIM: To review the studies conducted to date on the communication difficulties and dysphagia of children with nemaline myopathy and their possible management based on speech therapy. DEVELOPMENT: All the children presented dysphagia, with severe feeding problems during the first three years of life that nevertheless are somewhat mitigated as time goes by. In 50% of cases a gastrostomy will be used, although some oral ingestion is maintained in many of them. Nemaline myopathy gives rise to a clearly dysarthric pattern. The weakness of the muscles involved in ventilation and of the face, with a limited ability to close the mouth, leads to hypophonia, nasality and marked unintelligibility. Studies conducted on treatments based on speech therapy suggest that, in the most disabling cases of dysarthria, alternative systems of communication should be used in the first years of life so as to eliminate the frustration caused by the lack of meaningful expression. Later, communicators based on written language can be used. In the remaining cases, the aim must be to improve speech intelligibility. CONCLUSIONS: Speech therapy can contribute to improve the quality of life of these children with two types of treatment: management of their dysphagia and improvement of their communication problems through speech or technical aids. These is insufficient scientific evidence of the effectiveness of these treatments.

TITLE: Dificultades de comunicacion y lenguaje en niños con miopatia nemalinica.Introduccion. La miopatia nemalinica es una enfermedad rara con incidencia de 1 de cada 50.000 nacimientos vivos. Es la mas prevalente de las miopatias congenitas, un conjunto heterogeneo de trastornos neuromusculares presentes en el nacimiento o de muy temprana manifestacion, que afectan a la musculatura esqueletica, y que provocan debilidad, hipotonia y retraso psicomotor, pero con desarrollo cognitivo normal. Objetivo. Revisar los estudios sobre los problemas de comunicacion y disfagia de niños con miopatia nemalinica y los posibles abordajes desde la logopedia. Desarrollo. Todos los niños presentan disfagia, con graves problemas para la alimentacion durante los tres primeros años que se mitigan con el tiempo. El 50% usara gastrostomia, aunque muchos de ellos mantengan alguna ingesta oral. La miopatia nemalinica provoca un patron de habla claramente disartrico. La debilidad de la musculatura de la ventilacion y de la cara, con limitacion para cerrar la boca, conlleva hipofonia, nasalidad y acusada ininteligibilidad. Los estudios sobre tratamientos logopedicos sugieren que, ante la disartria mas incapacitante, deberian usar sistemas alternativos de comunicacion, en los primeros años de vida, para eliminar la frustracion de la falta de expresion util, y beneficiarse de comunicadores basados en el lenguaje escrito mas adelante. En el resto, el objetivo debe ser mejorar la inteligibilidad del habla. Conclusiones. La logopedia puede aportar dos tipos de tratamientos para mejorar la calidad de vida de estos niños: el abordaje de la disfagia y la mejora de las dificultades de expresion a traves del habla o de ayudas tecnicas. No hay suficiente evidencia cientifica de la eficacia de estos tratamientos.

[Non-speech oral motor treatment efficacy for children with developmental speech sound disorders]. / Eficacia de los programas de ejercicios de motricidad oral para el tratamiento logopedico de las dificultades de habla.

INTRODUCTION: In the treatment of speech disorders by means of speech therapy two antagonistic methodological approaches are applied: non-verbal ones, based on oral motor exercises (OME), and verbal ones, which are based on speech processing tasks with syllables, phonemes and words. In Spain, OME programmes are called 'programas de praxias', and are widely used and valued by speech therapists. AIM: To review the studies conducted on the effectiveness of OME-based treatments applied to children with speech disorders and the theoretical arguments that could justify, or not, their usefulness. DEVELOPMENT: Over the last few decades evidence has been gathered about the lack of efficacy of this approach to treat developmental speech disorders and pronunciation problems in populations without any neurological alteration of motor functioning. The American Speech-Language-Hearing Association has advised against its use taking into account the principles of evidence-based practice. The knowledge gathered to date on motor control shows that the pattern of mobility and its corresponding organisation in the brain are different in speech and other non-verbal functions linked to nutrition and breathing. CONCLUSIONS: Neither the studies on their effectiveness nor the arguments based on motor control studies recommend the use of OME-based programmes for the treatment of pronunciation problems in children with developmental language disorders.

TITLE: Eficacia de los programas de ejercicios de motricidad oral para el tratamiento logopedico de las dificultades de habla.Introduccion. En el tratamiento logopedico de las dificultades de habla se practican dos enfoques metodologicos antagonicos: los no verbales, basados en ejercicios de motricidad oral (EMO), y los verbales, que se basan en tareas de procesamiento de habla con silabas, fonemas y palabras. En España, los programas de EMO se llaman 'programas de praxias', estan muy difundidos y son apreciados por los logopedas. Objetivo. Revisar los estudios sobre la eficacia de los tratamientos basados en EMO aplicados a niños con trastornos de habla y los argumentos teoricos que podrian justificar o no su utilidad. Desarrollo. Durante las ultimas decadas se han acumulado pruebas sobre la falta de eficacia de este enfoque en el tratamiento de los trastornos evolutivos del habla y en las dificultades de pronunciacion de poblaciones sin alteracion neurologica de la funcion motriz. La American Speech-Language-Hearing Association ha desaconsejado su uso atendiendo a los principios de practica basada en la evidencia. Los conocimientos acumulados sobre el control motor demuestran que el patron de movilidad y su correspondiente organizacion cerebral son diferentes en el habla y en otras funciones no verbales ligadas a la alimentacion y la respiracion. Conclusiones. Ni los estudios sobre su eficacia ni los argumentos a partir de estudios del control motor aconsejan el uso de los programas basados en EMO para el tratamiento de las dificultades de pronunciacion en niños con trastornos evolutivos del lenguaje.

[The value of phonological analysis in speech therapy]. / Utilidad del análisis fonológico en la terapia del lenguaje.

INTRODUCTION: Phonological analysis is a system for assessing children's speech that was introduced by Ingram in 1976. Despite being acknowledged as one of the essential elements for understanding speech therapy intervention in speech disorders, it is not a wholly generalised practice due to the difficulties involved and the shortage of standardised instruments for performing it. DEVELOPMENT: Here, we examine the impact of phonological analysis on speech therapy intervention and the history of the instruments that have been developed over the years to perform it, both in English and in Spanish. The basic procedure for carrying out this kind of analysis is explained and we also present a software application that allows it to be performed automatically. CONCLUSIONS: We recommend making phonological analysis to assess children with language disorders a more widespread practice and also increasing the planning of adequate intervention programmes.

[A proposal for intervention in dysorthographic disorders by attending to the semiology of the mistakes]. / Una propuesta de intervención en trastornos disortográficosatendiendo a la semiología de los errores.

INTRODUCTION: A careful analysis of the mistakes made by children with learning disabilities in writing reveals a number of qualitatively distinct errors, each of which is directly related to one of the fundamental processes underlying the ability to spell correctly, that is to say, perceptive-linguistic, metalinguistic, operative memory and long-term memory processes, among others. The objective of this study is to describe our proposal for a methodology for treating children with orthography learning difficulties that takes these processes into account. DEVELOPMENT: A thorough analysis of the orthographic mistakes and their semiological classification is taken as our starting point, and we then put forward a sequence of instruction for each kind of error that tackles the deficient psycholinguistic processes, offsets the weaker points of the processing with the stronger points, and aims to get the child to learn a large number of words, thus favouring the metacognitive and self-regulatory processes applied to writing. CONCLUSIONS: Teaching pupils with dyslexic and dysorthographic problems to spell and write correctly entails important methodological difficulties. In our clinical practice we have attempted to develop teaching processes that fit the semiological characteristics of the spelling errors by trying to modify both the pupil's actual academic performance and the psycholinguistic processes that underlie such errors.

Una propuesta de intervención en trastornos disortográficos atendiendo a la semiología de los errores / A proposal for intervention in dysorthographic disorders by attending to the semiology of the mistakes

Introducción. Cuando realizamos un análisis minuciosode los errores que cometen los niños con problemas de aprendizajede la escritura, podemos observar errores cualitativamente diversos,cada uno de ellos directamente relacionado con uno de los procesosbásicos imprescindibles para la ortografía: procesos perceptivo-lingüísticos,metalingüísticos, de memoria operativa, de memoria a largoplazo, etc. El objetivo de este trabajo es la exposición de una propuestametodológica para el tratamiento de niños con dificultades deaprendizaje de la ortografía que considere estos procesos. Desarrollo.Se parte de un análisis minucioso de los errores ortográficos y suclasificación semiológica, y se propone una secuencia de instrucciónpara cada tipo de error que afronta los procesos psicolingüísticosdeficitarios, compensa los puntos débiles del procesamiento con lospuntos fuertes y persigue el aprendizaje de un gran número de palabras,de manera que se favorecen los procesos metacognitivos yautorregulatorios aplicados a la escritura. Conclusiones. La enseñanzade la ortografía a alumnos con problemas disléxicos y disortográficosplantea dificultades metodológicas importantes. En nuestrapráctica clínica hemos intentado desarrollar procesos de enseñanzaque se adapten a las características semiológicas de los erroresortográficos intentando influir tanto en el rendimiento académicoreal como en los procesos psicolingüísticos que subyacen a esoserrores

Introduction. A careful analysis of the mistakes made by children with learning disabilities in writing reveals anumber of qualitatively distinct errors, each of which is directly related to one of the fundamental processes underlying theability to spell correctly, that is to say, perceptive-linguistic, metalinguistic, operative memory and long-term memoryprocesses, among others. The objective of this study is to describe our proposal for a methodology for treating childrenwith orthography learning difficulties that takes these processes into account. Development. A thorough analysis of theorthographic mistakes and their semiological classification is taken as our starting point, and we then put forward a sequenceof instruction for each kind of error that tackles the deficient psycholinguistic processes, offsets the weaker points of theprocessing with the stronger points, and aims to get the child to learn a large number of words, thus favouring themetacognitive and self-regulatory processes applied to writing. Conclusions. Teaching pupils with dyslexic and dysorthographicproblems to spell and write correctly entails important methodological difficulties. In our clinical practice wehave attempted to develop teaching processes that fit the semiological characteristics of the spelling errors by trying to modifyboth the pupil’s actual academic performance and the psycholinguistic processes that underlie such errors

[Verbal dyspraxia: its clinical characteristics and treatment with speech therapy]. / Dispraxia verbal: caracteristicas clinicas y tratamiento logopédico.

INTRODUCTION: Verbal dyspraxia is a specific developmental language disorder that is characterised by a deficit at different levels of speech processing: in phonological planning, in phonetic planning and in the implementation of the speech motor programme. DEVELOPMENT: In this study we review the criteria for differential diagnosis, the tests that are useful to achieve this and the possible lines of speech therapy treatment.

[Speech therapy intervention in phonological disorders from the psycholinguistic paradigm of speech processing]. / Intervención logopédica en los trastornos fonológicos desde el paradigma psicolingüístico del procesamiento del habla.

The aim of this study is to present a survey of speech therapy intervention in phonological disorders (PD). We will examine the concepts of normal phonological development and those involved in PD in order to understand how they have been dealt with, historically, in speech therapy intervention. Lastly, we will describe how evaluation and intervention are carried out from the speech processing paradigm. Phonetic phonological skills allow people to decode the phonic strings they hear so as to be able to gain access to their phonological form and meaning. These abilities also enable them to encode these strings from lexical representations to pronounce words. The greater part of their development takes place during approximately the first four years of life. Speech processing difficulties affect the phonetic phonological skills and occur throughout almost all language pathologies, although the effect they exert is not always the same. This can range from a lack of the capacity to speak to important problems of intelligibility or mild problems with certain phonemes. Their influence on learning to read and write has been shown in recent decades. Speech therapy intervention began from a model based on articulatory phonetics. In the 70s a linguistic model based on the process of speech simplification and phonological analysis was added and this gave rise to a marked improvement in the systems used for evaluation and intervention. At present we have assumed a psycholinguistic model that links the perceptive skills with productive ones and top-down or bottom-up processing (from lexical representations to perception or production of phonemes and vice-versa).