Focal Cognitive Syndromes

The neurologic results of cortical lesions reflect the structural properties of the affected region. Lesions affecting primary sensorimotor cortices result in primary sensorimotor deficits that are qualitatively all-or-nothing, such as blindness (hemianopia) and paralysis (hemiparesis). Quantitatively, though, the severity of the deficit depends on the extent of the lesion (so that a hemiparetic patient may not be completely paralyzed but simply weak). Lesions affecting modality-specific association regions result in conceptually more complex disorders that are confined to a single modality, such as nonfluent aphasia (a form of motor speech disorder reflecting the language-dominant hemisphere) or prosopagnosia (a visual disorder impairing the ability to disambiguate visually similar entities, specifically faces, reflecting the “what” visual pathway in inferotemporal cortices).

Discourse Analysis in Tamil Speaking Individuals with Parkinson’s Disease

Background: Parkinson’s disease (PD) is a degenerative disorder of the central nervous system. Though PD is primarily a motor speech disorder during the initial stages, as the disease progresses, a gradual decline in linguistic aspects is observed as well. The effects of disease (PD) on cognition, word retrieval, syntax, and speech/voice processes may act together to manifest uniquely in spoken language tasks. There is a dearth of studies focusing on the discourse abilities in PD, especially in Indian context. Aim: The present study aims at investigating the micro and macrostructure discourse by using personal narrative task in Tamil speaking individuals with idiopathic PD and comparing it with the neuro-typical individuals Method: Two groups comprising 5 Tamil-speaking individuals with idiopathic PD in the age range of 60–85 years and 5 neuro-typical individuals were included in the study. To assess their discourse skills, participants were engaged in a personal narrative task. The analyses done were based on macro and micro structural aspects of discourse. The raw scores were subjected to suitable statistical analysis. Results: There was a statistical significance between the Parkinson & Normal group on Total number of words, No. of Different words, No. of content words, No. of Functional words, Free morphemes, Complex structure per utterances & Relevant piece per utterance in microstructural aspects. However there was no significant difference observed in the macrostructural aspects due to smaller sample size. Conclusion: It can be concluded that in addition to speech impairment, language structure is also affected in persons with PD. Intervening at the level of discourse is an important aspect as it enhances the quality of life. Keywords: Parkinson’s disease, Discourse, Tamil speaking, Micro analysis, Macro Analysis.

Assessment of Dysphonia in Children with Pompe Disease Using Auditory-Perceptual and Acoustic/Physiologic Methods

Bulbar and respiratory weakness occur commonly in children with Pompe disease and frequently lead to dysarthria. However, changes in vocal quality associated with this motor speech disorder are poorly described. The goal of this study was to characterize the vocal function of children with Pompe disease using auditory-perceptual and physiologic/acoustic methods. High-quality voice recordings were collected from 21 children with Pompe disease. The Grade, Roughness, Breathiness, Asthenia, and Strain (GRBAS) scale was used to assess voice quality and ratings were compared to physiologic/acoustic measurements collected during sustained phonation tasks, reading of a standard passage, and repetition of a short phrase at maximal volume. Based on ratings of grade, dysphonia was present in 90% of participants and was most commonly rated as mild or moderate in severity. Duration of sustained phonation tasks was reduced and shimmer was increased in comparison to published reference values for children without dysphonia. Specific measures of loudness were found to have statistically significant relationships with perceptual ratings of grade, breathiness, asthenia, and strain. Our data suggest that dysphonia is common in children with Pompe disease and primarily reflects impairments in respiratory and laryngeal function; however, the primary cause of dysphonia remains unclear. Future studies should seek to quantify the relative contribution of deficits in individual speech subsystems on voice quality and motor speech performance more broadly.

Rat Models of Vocal Deficits in Parkinson’s Disease

Parkinson’s disease (PD) is a progressive, degenerative disorder that affects 10 million people worldwide. More than 90% of individuals with PD develop hypokinetic dysarthria, a motor speech disorder that impairs vocal communication and quality of life. Despite the prevalence of vocal deficits in this population, very little is known about the pathological mechanisms underlying this aspect of disease. As such, effective treatment options are limited. Rat models have provided unique insights into the disease-specific mechanisms of vocal deficits in PD. This review summarizes recent studies investigating vocal deficits in 6-hydroxydopamine (6-OHDA), alpha-synuclein overexpression, DJ1-/-, and Pink1-/- rat models of PD. Model-specific changes to rat ultrasonic vocalization (USV), and the effects of exercise and pharmacologic interventions on USV production in these models are discussed.

A Novel Protocol Designed to Treat Spastic Dysarthria Secondary to Traumatic Brain Injury: A Case Study

Introduction Spastic dysarthria is a motor speech disorder produced by bilateral damage to the activation pathways of the central nervous system. Its speech characteristics reflect the effects of hypertonicity and weakness of the bulbar musculature in a way that slows movement and reduces range of motion and force. Perceptually, speech has a high-pitched, strained, hypernasal vocal quality with decreased intelligibility. Purpose The purpose is to present a case illustration describing the use of a novel treatment protocol to improve speech intelligibility in the presence of spastic dysarthria. Method An underlying framework, including principles of exercise, neuroplasticity, and motor learning with adjunctive biofeedback, is described. The protocol consisted of four sessions per week for 4 weeks with daily homework. Results The participant exhibited improvements in intelligibility, patient satisfaction, lingual, and jaw range of motion, nasality, and tongue strength. Conclusion The use of a novel protocol using biofeedback and incorporating principles of exercise science, neuroplasticity, and motor learning for the treatment of spastic dysarthria demonstrated positive outcomes.

A Modeling-Guided Case Study of Disordered Speech in Minimally Verbal Children With Autism Spectrum Disorder

Purpose Understanding what limits speech development in minimally verbal (MV) children with autism spectrum disorder (ASD) is important for providing highly effective targeted therapies. This preliminary investigation explores the extent to which developmental speech deficits predicted by Directions Into Velocities of Articulators (DIVA), a computational model of speech production, exemplify real phenotypes. Method Implementing a motor speech disorder in DIVA predicted that speech would become highly variable within and between tokens, while implementing a motor speech plus an auditory processing disorder predicted that DIVA's speech would become highly centralized (schwa-like). Acoustic analyses of DIVA's output predicted that acoustically measured phoneme distortion would be similar between the two cases, but that in the former case, speech would show more within- and between-token variability than in the latter case. We tested these predictions quantitatively on the speech of children with MV ASD. In Study 1, we tested the qualitative predictions using perceptual analysis methods. Speech pathologists blinded to the purpose of the study tallied the signs of childhood apraxia of speech that appeared in the speech of 38 MV children with ASD. K-means clustering was used to create two clusters from the group of 38, and analysis of variance was used to determine whether the clusters differed according to perceptual features corresponding to within- and between-token variability. In Study 2, we employed acoustic analyses on the speech of the child from each cluster who produced the largest number of analyzable tokens to test the predictions of differences in within-token variability, between-token variability, and vowel space area. Results Clusters produced by k-means analysis differed by perceptual features that corresponded to within-token variability. Nonsignificant differences between clusters were found for features corresponding to between-token variability. Subsequent acoustic analyses of the selected cases revealed that the speech of the child from the high-variability cluster showed significantly more quantitative within- and between-token variability than the speech of the child from the low-variability cluster. The vowel space of the child from the low-variability cluster was more centralized than that of typical children and that of the child from the high-variability cluster. Conclusions Results provide preliminary evidence that subphenotypes of children with MV ASD may exist, characterized by (a) comorbid motor speech disorder and (b) comorbid motor speech plus auditory processing disorder. The results motivate testable predictions about how these comorbidities affect speech. Supplemental Material https://doi.org/10.23641/asha.14384432

Communication Limitations in Patients With Progressive Apraxia of Speech and Aphasia

Purpose Individuals with primary progressive apraxia of speech (AOS) have AOS in which disruptions in articulation and prosody predominate the speech pattern. Many develop aphasia and/or dysarthria later in the disease course. The aim of this study was to describe the communication limitations in these patients, as measured by (a) the patient via the Communicative Participation Item Bank (CPIB) and (b) the speech-language pathologist via the American Speech-Language-Hearing Association's (ASHA) Functional Communication Measures (FCMs) and an adapted motor speech disorder (MSD) severity rating. Method Speech and language evaluations were completed for 24 patients with progressive AOS ( n = 7 with isolated AOS; n = 17 with a combination of AOS and aphasia). Descriptive comparisons were utilized to evaluate differences in communication measures among patients with various combinations of MSDs and aphasia. Differences associated with phonetic predominant or prosodic predominant AOS were also examined. Across the entire cohort, correlations were calculated between the participation ratings and other clinical assessment measures. Results The CPIB reflected greater limitations for those with aphasia and AOS compared to isolated AOS, but was not notably different when dysarthria occurred with AOS ( n = 9/24). Across the cohort, there were statistically significant correlations between the CPIB and ASHA FCM–Motor Speech and Language Expression ratings and the MSD severity rating. The CPIB did not correlate with the ASHA FCM–Language Comprehension or other speech-language measures. Conclusions Patients with neurodegenerative AOS experience reduced participation in communication that is further exacerbated by co-occurring language deficits. The study suggests measures of severity cannot be assumed to correlate with measures of participation restrictions and offers a foundation for further research examining the day-to-day sequela of progressive speech and language disorders. Supplemental Material https://doi.org/10.23641/asha.12743252