Dysarthria

About: Dysarthria is a research topic. Over the lifetime, 2402 publications have been published within this topic receiving 56554 citations.

Isolated lingual involvement in Wilson's disease.

Abstract: Lingual involvement can occur in a variety of neurological disorders including pyramidal, extrapyramidal and lower motor neuron disorders. It can be seen in the form of tremor, bradykinesia, dystonia, atrophy and weakness of tongue movements and can clinically present as difficulty in swallowing and dysarthria which can be a source of great discomfort to the patient. We describe a patient who presented with isolated lingual involvement and was diagnosed to have Wilsons's disease. This case emphasizes the clinical variability in presentation of Wilson's disease and importance of early clinical diagnosis.

How does automatic speech recognition handle dysarthric speech

Abstract: This paper describes how the speech of a severely dysarthric speaker is recognized by two speech recognition systems, namely the Infovox RA system and the Prototype Swedish DragonDictate (PSDD) system. The results indicated that the PSDD system adapted to the speech of the severely dysarthric subject but with lower values than for a normal speaker. On the Infovox RA system, the dysarthric subject had a mean recognition score of 74% while the normal subject scored 97%. The results are discussed in terms of what effect the speech characteristics of the subject had on the speech recognizer. I N T R O D U C T I O N Automatic speech recognition (ASR) is a viable interface even for individuals with speech impairments such as dysarthria with which to access computers (Lariviere, MacKinnon, & Risebrough, 1993). However, it is not known how various types of recognition systems work with individuals with different degrees and types of dysarthria. This information would guide professionals in selecting a suitable recognizer for an individual and in using A S R with a wider population for a variety of applications.

Auditory pathway changes mirror overall disease progress in individuals with Friedreich ataxia.

Abstract: Friedreich ataxia (FRDA) is a neurodegenerative disease affecting both motor and sensory systems through mutations of the FXN gene. Electrophysiologic changes consistent with axonopathy in the cochlear nerve and auditory brainstem have been reported in a high proportion ([90 %) of affected individuals, as have functional effects including disruption of temporal-resolution and speech perception [1, 2]. We present longitudinal findings for two males with FRDA in whom auditory deterioration mirrored overall disease progress. Patient 1 reported walking difficulties evident from his late teens. Molecular diagnosis confirmed homozygosity for GAA expansions in intron 1 of the FXN gene (repeat lengths: 527/1058). When last examined (at 32 years old), he could walk independently but required a motorisedscooter for community mobility. He also had mild dysarthria, upper limb dysmetria, lower limb areflexia and scoliosis. Patient 2 also reported walking problems in adolescence (15 years). Molecular diagnosis confirmed homozygosity for GAA expansions in intron 1 of the FXN gene (repeat lengths: 569/884). At diagnosis he was independently ambulant, but his physical difficulties progressed rapidly, and at 22 years old he required a walker for short distances and wheelchair for community access. He also had upper limb dysmetria, dysarthria, lower limb areflexia, scoliosis and hypertrophic cardiomyopathy. Both individuals underwent annual auditory brainstem response (ABR) and binaural speech-processing (Listening in Spacialized Noise [LiSN-S]) evaluations over a 3 year period. Patient 1 was 29–32 years and Patient 2, 18–21 years old at assessment. Overall disease severity was equivalent in the two subjects at initial assessment with both achieving scores of about 65 on the Friedreich Ataxia Rating Scale (FARS) [3]. Disability levels for Patient 1 remained stable, but Patient 2 had significant functional deterioration across the study period (Fig. 1). Both subjects showed evidence of cochlear nerve axonopathy despite maintaining normal sound detection. Patient 1 presented with ABRs of normal latency and low amplitude at each test point (Fig. 2a), suggesting normal transmission efficiency but reduced neural populations in the central auditory pathways [1]. Patient 2 showed normal ABRs at first assessment, which decreased in amplitude over the study period and became unidentifiable in the final year (Fig. 2b). ‘‘Spatial listening’’ is the ability to localize sound sources (and hence improve perception) through the integration of subtle timing and level differences in the acoustic-signal reaching the two ears [4]. These interaural cues are initially processed in the lower brainstem, where neural impulses from left and right nerves meet at the G. Rance (&) Department of Audiology and Speech Pathology, The University of Melbourne, 550 Swanston Street, Parkville, Melbourne, VIC 3010, Australia e-mail: grance@unimelb.edu.au

Speech and language in bilateral perisylvian polymicrogyria: a systematic review.

Abstract: Aim We aimed to systematically review the speech production, language, and oral function phenotype of bilateral perisylvian polymicrogyria (BPP), and examine the correlation between the topography of polymicrogyria and the severity of speech, language, and oral functional impairment. Method A systematic search of MEDLINE, Embase, and PubMed databases was completed on 26th October 2017 using Medical Subject Heading terms synonymous with BPP and speech, language, or oral motor impairment. In total, 2411 papers were identified and 48 met inclusion criteria. Results Expressive and receptive language impairment and oral structural and functional deficits are frequent in BPP. Expressive deficits are frequently more severe than receptive. Only one study used formal assessments to demonstrate the presence of speech disorder, namely dysarthria. Seven studies reported an association between diffuse BPP and more severe language impairment. Interpretation Findings confirmed that language deficits are common in BPP, though assessment of the specific speech phenotype is limited. The paucity of high quality studies detailing the specific communication phenotype of BPP highlights the need for further investigation. Improving understanding of this phenotype will inform the development of targeted therapies and lead to better long-term outcomes. What this paper adds Speech, language, and oral functional impairments are common in individuals with bilateral perisylvian polymicrogyria. Posterior polymicrogyria is associated with a less severe language impairment than anterior polymicrogyria. Deeper investigation of speech is needed to understand implicated networks in this malformation.