Speech treatment improves dysarthria in multisystemic ataxia: a rater-blinded, controlled pilot-study in ARSACS

Vogel, Adam P.; Stoll, Lisa H.; Oettinger, Andreas; Rommel, Natalie; Kraus, Eva-Maria; Timmann, Dagmar; Scott, Dion H.; Atay, Christina; Storey, Elsdon; Schöls, Lüdger; Synofzik, Matthis

doi:10.1007/s00415-019-09258-4

Cited by 34 publications

(42 citation statements)

References 16 publications

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“…This was not monitored formally in our study, but judging from patient reports, the suggested regime was not always maintained. Finally, Vogel et al's [15] participant group was of lower severity level than included in this study. Whilst we could not identify any relationship between level of speech impairment and post-treatment change in our study, the lower level of disability could have further contributed to greater adherence and thus possibly better outcomes in their study.…”

Section: Discussionmentioning

confidence: 94%

“…There is only one other study to date that has investigated the effects of speech treatment in a group of seven speakers with progressive ataxia [15]. This study's approach included a wider range of treatment targets, including voice and loudness production, as well as articulatory practice.…”

Section: Discussionmentioning

confidence: 99%

“…Our participants experienced a high number of interruptions and gaps in their treatment course (Table 2), which could have affected their outcomes. On the other hand, whilst Vogel et al [15] report a 100% completion rate of their protocol, no information is provided about interruptions. Sapir et al [26] do not mention adherence being an issue in their case report.…”

Section: Discussionmentioning

confidence: 99%

“…Sapir et al [26] do not mention adherence being an issue in their case report. In addition, Vogel et al [15] used a home practice App which ensured regular practice by participants. This was not monitored formally in our study, but judging from patient reports, the suggested regime was not always maintained.…”

Section: Discussionmentioning

confidence: 99%

“…Vogel et al [15] recently reported positive outcomes from a pilot study on speech treatment involving seven patients with autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS). Their treatment was home based, supported by an App that took participants through exercises addressing voice production and articulation.…”

Section: Introductionmentioning

confidence: 99%

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Feasibility and Acceptability of Lee Silverman Voice Treatment in Progressive Ataxias

2020

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Communication difficulties have considerable impact on people with progressive ataxia, yet there are currently no evidencebased treatments. LSVT LOUD® focuses on the production of healthy vocal loudness whilst also improving breath support, vocal quality, loudness and articulation in participating patients. This study aimed to investigate whether Lee Silverman Voice Treatment (LSVT LOUD®) can improve communication effectiveness in these patients. We performed a rater-blinded, singlearm study investigating LSVT LOUD® treatment in a population of patients with progressive ataxia including Friedreich's ataxia (n = 18), spinocerebellar ataxia type 6 (n = 1), idiopathic cerebellar ataxia (n = 1), and spastic paraplegia 7 (n = 1). Twenty-one patients were recruited to the study, with 19 completing treatment. Sessions were administered via Skype in the LSVT-X format, meaning two sessions per week over a period of 8 weeks. Assessments included two baseline and two post-treatment measures and focused on outcome measures covering aspects ranging from physiological function to impact and participation. Results indicate improvements in patient-perceived outcomes for 14 of the 19 participants, in both speech and psychosocial domains. Speech data furthermore demonstrate significant improvements in prolonged vowel duration, and voice quality measures. Intelligibility and naturalness evaluations showed no change post-treatment. Patients reported high acceptability of the treatment itself, as well as administration by Skype. This is the largest treatment study for people with progressive ataxia published to date. It provides an indication that LSVT LOUD® can have a positive impact on communication in this patient group and could form the basis for larger-scale trials.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Feasibility and Acceptability of Lee Silverman Voice Treatment in Progressive Ataxias

2020

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Home‐based biofeedback speech treatment improves dysarthria in repeat‐expansion SCAs

Vogel

Graf

Magee

et al. 2022

Ann Clin Transl Neurol

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CAG repeat‐expansion spinocerebellar ataxias (CAG‐SCAs) are genetically defined multisystemic degenerative diseases, resulting in motor symptoms including dysarthria with a substantial impact on daily living. Whilst speech therapy is widely recommended in ataxia, very limited evidence exists for its use. We evaluated the efficacy of a home‐delivered, ataxia‐tailored biofeedback‐driven speech therapy in CAG‐SCA in 16 individuals with SCA1, 2, 3, or 6. Treatment was delivered intensively over 20 days. Efficacy was evaluated by blinded ratings of intelligibility (primary) and acoustic measures (secondary) leveraging an intra‐individual control design. Intelligibility improved post‐treatment (Z = −3.18, p = 0.004) whilst remaining stable prior to treatment (Z = 0.53, p = 1.00).

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Responsiveness of the Scale for the Assessment and Rating of Ataxia and Natural History in 884 Recessive and Early Onset Ataxia Patients

Traschütz

Adarmes‐Gómez

Anheim

et al. 2023

Annals of Neurology

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ObjectiveThe Scale for the Assessment and Rating of Ataxia (SARA) is the most widely applied clinical outcome assessment (COA) for genetic ataxias, but presents metrological and regulatory challenges. To facilitate trial planning, we characterize its responsiveness (including subitem‐level relations to ataxia severity and patient‐focused outcomes) across a large number of ataxias, and provide first natural history data for several of them.MethodsSubitem‐level correlation and distribution‐based analysis of 1,637 SARA assessments in 884 patients with autosomal recessive/early onset ataxia (370 with 2–8 longitudinal assessments) were complemented by linear mixed effects modeling to estimate progression and sample sizes.ResultsAlthough SARA subitem responsiveness varied between ataxia severities, gait/stance showed a robust granular linear scaling across the broadest range (SARA < 25). Responsiveness was diminished by incomplete subscale use at intermediate or upper levels, nontransitions (“static periods”), and fluctuating decreases/increases. All subitems except nose‐finger showed moderate‐to‐strong correlations to activities of daily living, indicating that metric properties—not content validity—limit SARA responsiveness. SARA captured mild‐to‐moderate progression in many genotypes (eg, SYNE1‐ataxia: 0.55 points/yr, ataxia with oculomotor apraxia type 2: 1.14 points/yr, POLG‐ataxia: 1.56 points/yr), but no change in others (autosomal recessive spastic ataxia of Charlevoix‐Saguenay, COQ8A‐ataxia). Whereas sensitivity to change was optimal in mild ataxia (SARA < 10), it substantially deteriorated in advanced ataxia (SARA > 25; 2.7‐fold sample size). Use of a novel rank‐optimized SARA without subitems finger‐chase and nose‐finger reduces sample sizes by 20 to 25%.InterpretationThis study comprehensively characterizes COA properties and annualized changes of the SARA across and within a large number of ataxias. It suggests specific approaches for optimizing its responsiveness that might facilitate regulatory qualification and trial design. ANN NEUROL 2023

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Speech treatment improves dysarthria in multisystemic ataxia: a rater-blinded, controlled pilot-study in ARSACS

Cited by 34 publications

References 16 publications

Feasibility and Acceptability of Lee Silverman Voice Treatment in Progressive Ataxias

Feasibility and Acceptability of Lee Silverman Voice Treatment in Progressive Ataxias

Home‐based biofeedback speech treatment improves dysarthria in repeat‐expansion SCAs

Responsiveness of the Scale for the Assessment and Rating of Ataxia and Natural History in 884 Recessive and Early Onset Ataxia Patients

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