Speech, voice, language and cognition in individuals with spinocerebellar ataxia (SCA)

Spinocerebellar ataxias (SCA) constitute a group of genetically defined hereditary, degenerative, progressive diseases affecting the cerebellum and its connections. Few previous investigations have focused on how SCA affects different aspects of communication. The aim of the present investigation was to characterize speech and voice in individuals with SCA and to investigate the progression of speech and voice symptoms, using both perceptual and acoustic methodology. In addition, language and cognition in individuals with SCA were studied. Thirty-two individuals with spinocerebellar degenerative disease participated in the studies. The majority had been diagnosed with SCA using molecular genetic testing and the rest were clinically diagnosed by a specialist in neurology. Matched control subjects were included in study II and III. Speech and voice in individuals with SCA were assessed perceptually by a group of four speech-language pathologists with long experience of neurogenic communication disorders. Recorded speech samples from individuals with SCA were rated using visual analogue scales, VAS. Speech samples were also used for computer-based acoustic analysis. Speech and voice were characterized by the following perceptual parameters: Equalized stress, imprecise consonants, vocal instability, monotony, strained-strangled voice, stereotypic intonation and reduced speech rate. Factor analysis resulted in two main factors; one associated with temporal aspects of speech and the other with vocal quality. Acoustic analysis confirmed the perceptual findings. Rate of speech and sequential and alternating motion rates were reduced, and duration and variability of syllables and pauses during rapid syllable repetition were increased compared to matched control subjects. Inter-stress intervals (ISIs) were also longer and more variable in subjects with SCA compared to control subjects. Perceived vocal instability was confirmed acoustically by increased coefficient of variation of fundamental frequency, CV of F0, during sustained phonation. This was also found in individuals with SCA with otherwise close to normal speech. Speech and voice were followed in nine individuals with SCA during three years and speech samples were analyzed both perceptually and acoustically. Perceived imprecision of consonants and stereotypic intonation had increased during the three years. Some acoustic measures had also changed, e g duration of syllables in rapid syllable repetition and duration of inter-stress intervals. In addition, there was a trend towards change in several other perceptual and acoustic measures, especially measures related to temporal aspects. Changes were more substantial in individuals with early disease onset, regardless of disease duration. An increase of mean dysarthria scores was also found. Language and cognition were assessed in 20 individuals with SCA and control subjects matched for age, gender, length of formal education and estimated cognitive level. Executive functions and attention were most severely affected, but memory and lexicosemantic knowledge were also impaired, especially in individuals with more severely impaired estimated global cognitive level of functioning. Cognitive impairment correlated with low age at disease onset and also with impairment of motor speech function, but not with disease duration. It was concluded that dysarthria in SCA resembles previous descriptions of ataxic dysarthria, but also includes an element of strained-strangled voice. Equalized stress was more prominent and imprecision of vowels was less common compared to previous studies. Vocal instability may be an early sign of the disease. Progression of symptoms can be seen over a three-year period, especially as increased perceived imprecision of consonants and stereotypic intonation, but also measured with a clinical dysarthria test. Cognition was impaired in individuals with SCA, especially executive functions and attention. Assessment by speech-language pathologists should include testing of cognition and language as it may have implications for treatment. Keywords: Spinocerebellar ataxia, ataxic dysarthria, perceptual analysis, acoustic analysis, cerebellar degenerative disorders, cognitive impairment, language impairment, executive dysfunction, progression of neurological disease

HiCommunication as a novel speech and communication treatment for Parkinson's disease: A feasibility study

Abstract Introduction Speech and communication problems are common in Parkinson's disease (PD) and can result in social withdrawal and reduced quality of life. Intervention may improve symptoms but transfer and maintenance remain challenging for many. Access to treatment may also be limited. Group intervention incorporating principles for experience‐dependent plasticity may address these challenges. The aim of this study was to develop and study feasibility aspects of a new intervention program for group training of speech and communication in people with PD. Materials & Methods Development and content of the program called HiCommunication is described. Core target areas are voice, articulation, word‐finding and memory. Five participants with mild‐moderate PD completed this feasibility trial. Attendance rate and possible adverse events as well as the participants' experiences were documented. A speech recording and dysarthria testing were completed to study feasibility of the assessment procedure and evaluate possible changes in voice sound level and intelligibility. Results Attendance rate was 89%. No adverse events occurred. Participants reported a positive experience and limited fatigue. Assessment was completed in approximately 30 min and was well tolerated. Four of five participants had an increased voice sound level during text‐reading postintervention and mean intelligibility improved. Conclusions Results indicate that HiCommunication is feasible for people with mild‐moderate PD. The program was appreciated and well tolerated. Positive outcomes regarding voice sound level and intelligibility were observed; however, the number of participants was very limited. The results motivate that effects of HiCommunication are further studied in a randomized controlled trial, which is ongoing

Characterization of Mild and Moderate Dysarthria in Parkinson's Disease : Behavioral Measures and Neural Correlates

PurposeAlterations in speech and voice are among the most common symptoms in Parkinson's disease (PD), often resulting in motor speech disorders such as hypokinetic dysarthria. We investigated dysarthria, verbal fluency, executive functions, and global cognitive function in relation to structural and resting-state brain changes in people with PD. MethodsParticipants with mild-moderate PD (n = 83) were recruited within a randomized controlled trial and divided into groups with varying degrees of dysarthria: no dysarthria (noDPD), mild dysarthria (mildDPD), moderate dysarthria (modDPD), and also combined mildDPD and modDPD into one group (totDPD). Voice sound level and dysphonia, verbal fluency, motor symptoms, executive functions, disease severity, global cognition, and neuroimaging were compared between groups. Gray matter volume and intensity of spontaneous brain activity were analyzed. Additionally, regressions between behavioral and neuroimaging data were performed. ResultsThe groups differed significantly in mean voice sound level, dysphonia, and motor symptom severity. Comparing different severity levels of dysarthria to noDPD, groups differed focally in resting-state activity, but not in brain structure. In totDPD, lower scores on semantic verbal fluency, a composite score of executive functions, and global cognition correlated with lower superior temporal gyrus volume. ConclusionThis study shows that severity of dysarthria may be related to underlying structural and resting-state brain alterations in PD as well as behavioral changes. Further, the superior temporal gyrus may play an important role in executive functions, language, and global cognition in people with PD and dysarthria

Speech following DBS for essential tremor : Effects of chronic and high-amplitude stimulation in the posterior subthalamic area

Deep brain stimulation (DBS) can be very effective in alleviating tremor, but adverse effects on speech are frequently reported, especially following bilateral DBS. Most of the existing literature on DBS and speech deals with the effects of DBS targeting the subthalamic nucleus or the ventral intermediate nucleus of the thalamus, which are the traditional targets for Parkinson’s disease and essential tremor, respectively. More recently, the posterior subthalamic area (PSA) has been highlighted as a particularly effective target for tremor; however, there are limited studies of PSA-DBS effects on speech. We report speech outcomes for 14 persons with essential tremor during chronic PSA-DBS and at unilateral high-amplitude PSA-stimulation. The objectives were to answer the following questions: To what extent is speech function, and in particular articulation and voice, affected by chronic PSA-DBS? How is speech affected by unilateral high-amplitude stimulation Is bilateral chronic PSA-DBS worse for speech than unilateral PSA-stimulation

Speech following DBS for essential tremor : Effects of chronic and high-amplitude stimulation in the posterior subthalamic area

Deep brain stimulation (DBS) can be very effective in alleviating tremor, but adverse effects on speech are frequently reported, especially following bilateral DBS. Most of the existing literature on DBS and speech deals with the effects of DBS targeting the subthalamic nucleus or the ventral intermediate nucleus of the thalamus, which are the traditional targets for Parkinson’s disease and essential tremor, respectively. More recently, the posterior subthalamic area (PSA) has been highlighted as a particularly effective target for tremor; however, there are limited studies of PSA-DBS effects on speech. We report speech outcomes for 14 persons with essential tremor during chronic PSA-DBS and at unilateral high-amplitude PSA-stimulation. The objectives were to answer the following questions: To what extent is speech function, and in particular articulation and voice, affected by chronic PSA-DBS? How is speech affected by unilateral high-amplitude stimulation Is bilateral chronic PSA-DBS worse for speech than unilateral PSA-stimulation

Perceptual assessment of dysarthria: Comparison of a general and a detailed assessment protocol

<p><i>Objective.</i> The present, preliminary study was designed to investigate whether the results of the use of a detailed assessment protocol <i>ad modum</i> the Mayo Clinic rating of dysarthria and that of a more general assessment protocol, corresponding to ratings of deviances of the different speech production processes, differed primarily in terms of reliability.</p> <p><i>Patients and methods.</i> Recordings of text readings of 20 patients with various degrees and types of dysarthria were assessed using both protocols by five clinicians with extensive experience in assessment of neurogenic communication disorders, and results from both assessments were compared.</p> <p><i>Results.</i> The general assessment protocol was carried out with higher intra- and inter-rater reliability compared with the detailed assessment protocol. Perceptual deviations were identified in the same domains using both protocols, although only the more detailed protocol could be used to specify particular audible symptoms. Monotony, imprecise consonants, and harsh voice were the most prominent deviations identified with the detailed protocol.</p> <p><i>Conclusion.</i> It is concluded that a general assessment protocol is sufficient to identify problem areas reliably and indicate severity of dysarthria but needs to be complemented with a short description of the most prominent audible symptoms and an assessment of intelligibility.</p

Speech following DBS for essential tremor : Effects of chronic and high-amplitude stimulation in the posterior subthalamic area

Deep brain stimulation (DBS) can be very effective in alleviating tremor, but adverse effects on speech are frequently reported, especially following bilateral DBS. Most of the existing literature on DBS and speech deals with the effects of DBS targeting the subthalamic nucleus or the ventral intermediate nucleus of the thalamus, which are the traditional targets for Parkinson’s disease and essential tremor, respectively. More recently, the posterior subthalamic area (PSA) has been highlighted as a particularly effective target for tremor; however, there are limited studies of PSA-DBS effects on speech. We report speech outcomes for 14 persons with essential tremor during chronic PSA-DBS and at unilateral high-amplitude PSA-stimulation. The objectives were to answer the following questions: To what extent is speech function, and in particular articulation and voice, affected by chronic PSA-DBS? How is speech affected by unilateral high-amplitude stimulation Is bilateral chronic PSA-DBS worse for speech than unilateral PSA-stimulation

Speech function following deep brain stimulation of the caudal zona incerta : effects of habitual and high-amplitude stimulation

Purpose: Deep brain stimulation (DBS) is often successful in alleviating motor symptoms of essential tremor (ET); however, DBS may also induce adverse speech effects. The caudal zona incerta (cZi) is a promising DBS target for tremor, but less is known about the consequences of cZi DBS for speech. This preliminary study examined how habitual cZi DBS and cZi stimulation at high amplitudes may affect speech function in persons with ET. Method: Fourteen participants with ET were evaluated: off stimulation, on habitual cZi DBS, and with unilateral cZi stimulation at increasing stimulation amplitudes. At each stimulation condition, the participants read three 16-word sentences. Two speech-language pathologists made audio-perceptual consensus ratings of overall speech function, articulation, and voice using a visual sort and rate method. Rated functions when off stimulation, on habitual cZi DBS, and at maximal-amplitude stimulation were compared using Friedman nonparametric tests. For participants with bilateral habitual DBS (n = 5), the effects of bilateral and unilateral stimulation were described in qualitative terms. Results: Habitual cZi DBS had no significant group-level effect on any of the investigated speech parameters. Maximal-amplitude stimulation had a small but significant negative effect on articulation. Participants with reduced articulatory precision (n = 9) had more medially placed electrodes than the nonaffected group (n = 5). Bilateral and unilateral left stimulation had comparable effects on speech. Conclusions: Findings from this preliminary study of cZi DBS indicate that speech is generally not affected by stimulation at habitual levels. High-amplitude cZi stimulation may, however, induce adverse effects, particularly on articulation. Instances of decreased articulatory function were associated with stimulation of more medial electrode contacts, which could suggest cerebello-rubrospinal involvement.Originally included in thesis in manuscript form.Publisher Note: This article is part of the Special Issue: Select Papers From the 2020 Conference on Motor Speech.</p

Assessment of speech impairment in patients with Parkinson's disease from acoustic quantifications of oral diadochokinetic sequences

This investigation aimed at determining whether an acoustic quantification of the oral diadochokinetic (DDK)task may be used to predict the perceived level of speech impairment when speakers with Parkinson's disease(PD) are reading a standard passage. DDK sequences with repeated [pa], [ta], and [ka] syllables were collectedfrom 108 recordings (68 unique speakers with PD), along with recordings of the speakers reading a standardizedtext. The passage readings were assessed in five dimensions individually by four speech-language pathologists ina blinded and randomized procedure. The 46 acoustic DDK measures were merged with the perceptual ratings ofread speech in the same recording session. Ordinal regression models were trained repeatedly on 80% of ratingsand acoustic DDK predictors per dimension in 10-folds, and evaluated in testing data. The models developedfrom [ka] sequences achieved the best performance overall in predicting the clinicians' ratings of passage readings. The developed [pa] and [ta] models showed a much lower performance across all dimensions. The additionof samples with severe impairments and further automation of the procedure is required for the models to be usedfor screening purposes by non-expert clinical staff

Students take charge of Learning–Using e-learning in Perceptual Assessment in Speech–Language Pathology

Perceptual assessment is the basis for diagnosis and evaluation of treatment in speech–language pathology (SLP). Students need to practise assessment skills. A web-based platform with cases and expert feedback in cleft palate disorders was developed in national collaboration. The aim of the study was to evaluate the results of individual training on assessment skills in SLP students and their perception of e-learning. Forty-five students performed tests using a pre- and post-test set-up. Perceptual assessments were demonstrated and instructions provided during teacher-led activities in ongoing fully scheduled courses; students were then individually trained in their free time. Reference samples were available. A significant improvement was found in rating and phonetic transcriptions after training. Positive comments concerned accessibility and practice time