The Use of the Voice Trainer App for Vocal Control in People with a Degenerative Ataxia: A Pilot Intervention Study.

Dysarthria is disabling in persons with degenerative ataxia. There is limited evidence for speech therapy interventions. In this pilot study, we used the Voice trainer app, which was originally developed for patients with Parkinson's disease, as a feedback tool for vocal control. We hypothesized that patients with ataxic dysarthria would benefit from the Voice trainer app to better control their loudness and pitch, resulting in a lower speaking rate and better intelligibility. This intervention study consisted of five therapy sessions of 30 min within 3 weeks using the principles of the Pitch Limiting Voice Treatment. Patients received real-time visual feedback on loudness and pitch during the exercises. Besides, they were encouraged to practice at home or to use the Voice trainer in daily life. We used observer-rated and patient-rated outcome measures. The primary outcome measure was intelligibility, as measured by the Dutch sentence intelligibility test. Twenty-one out of 25 included patients with degenerative ataxia completed the therapy. We found no statistically significant improvements in intelligibility (p = .56). However, after the intervention, patients were speaking slower (p = .03) and the pause durations were longer (p < .001). The patients were satisfied about using the app. At the group level, we found no evidence for an effect of the Voice trainer app on intelligibility in degenerative ataxia. Because of the heterogeneity of ataxic dysarthria, a more tailor-made rather than generic intervention seems warranted.

Design of the PERSPECTIVE study: PERsonalized SPEeCh Therapy for actIVE conversation in Parkinson's disease (randomized controlled trial).

OBJECTIVE: To evaluate the effectiveness of personalized and home-based speech therapy on quality of life, intelligibility, and social participation for people with Parkinson's disease (PD) who have a reduced intelligibility of speech. BACKGROUND: Speech problems in PD have a profound negative impact on social interaction and quality of life. Evidence for speech therapy in PD is growing, but more work remains needed to explore its full potential. Efficacy exists for highly intensive standardized speech treatment programs, but not all patients can comply with this rather intense intervention, especially the more severely affected ones. Here, we aim to study the effectiveness of personalized and home-based (remote) speech therapy in PD on quality of life and speech. The intervention will be supported by a dedicated speech training app. We expect that this approach will improve speech intelligibility and quality of life in patients irrespective of disease stage. METHODS: We will perform a single blind, randomized controlled trial, comparing 8 weeks of speech therapy to no intervention using a waiting list design. A total of 215 PD patients with problems in intelligibility will be recruited by 12 highly experienced speech therapists. All patients will be measured at baseline and after 8 weeks (primary endpoint). Additionally, the experimental group will be re-assessed one more time, after a wash-out period of 24 weeks. The control group will receive deferred treatment after 8 weeks, but without additional follow-up assessments. Our primary outcome is quality of life (as measured with PDQ-39). Secondary outcomes include speech and voice quality, intelligibility, severity of voice and speech complaints, and caregiver burden. RESULTS: The inclusion of participants has started on March 1, 2019, and is expected to be finalized on April 1, 2021. We expect to have the first results in January 2022. CONCLUSIONS: We will investigate the effectiveness of speech therapy in PD. Particular strengths of our study include a randomized and single-blinded design, the personalized treatment approach, the inclusion of PD patients irrespective of disease stage or severity of the speech complaint, the long-term follow-up, the adequate power, and the use of a patient-relevant primary endpoint. This will allow us to draw firm conclusions about the effectiveness of personalized and remote speech therapy for PD patients in all disease stages. TRIAL REGISTRATION: ClinicalTrials.gov NCT03963388 . Registered on May 24, 2019.

Botulinum toxin-A injections vs radiotherapy for drooling in ALS.

OBJECTIVES: Botulinum neurotoxin (BoNT) injections in the salivary glands and radiotherapy (RT) on these glands are commonly used to alleviate severe drooling in patients with amyotrophic lateral sclerosis (ALS). This study compares BoNT type A with RT based on patient-rated evaluations. MATERIALS & METHODS: A prospective randomized controlled pilot study to compare RT (n = 10; on the parotid and the posterior part of the submandibular glands) with BoNT-A treatment (n = 10; in the parotid glands only, because of the risk of increasing oropharyngeal weakness) in patients with ALS. The primary outcome was the drooling status (burden of drooling), and our secondary interests were the degree of salivation, global change of drooling after treatment, and level of satisfaction with the treatment and negative experiences. RESULTS: There were no statistically significant between-treatment differences for the drooling status after treatment. Only at twelve weeks more saliva reduction was achieved by RT (P = 0.02). Patients treated with RT also described more transient negative experiences (like pain in mandible) directly after treatment. Subgroup analysis showed that patients with very severe dysphagia (no oral intake) were less satisfied and experienced a lower global change of drooling after treatment. CONCLUSIONS: This pilot study showed no significant difference in the burden of drooling between the treatments. However, with RT more saliva reduction was achieved, including negative experiences directly after treatment, but without the risk of decreasing oropharyngeal function. In addition, patients with very severe dysphagia do not seem to benefit from either treatment.

Orofaryngeale slikstoornissen bij de ziekte van Parkinson.

OROPHARYNGEAL SWALLOWING DISORDERS IN PARKINSON'S DISEASE: Parkinson's disease is one of the most common and best studied neurodegenerative diseases. The typical motor features, like hypokinesia and rigidity are also seen in chewing and swallowing, but this 'hypokinetic dysphagia' is a complaint that generally occurs in the later stages of the disease. However, consequences as choking on liquid or food and very slow eating and drinking can contribute to a decrease of the quality of life and in combination with decreased coughing capacity cause aspiration pneumonia. Hypokinetic dysphagia can also contribute to drooling, but hypomimia is the best predictor of that complaint. Several validated questionnaires are available in Dutch to assess dysphagia complaints and their severity. The behavioural treatment consists of compensatory techniques, like adapting head posture to avoid liquid aspiration. But also training exercises can be helpful to overcome hypokinesia, similar to talking louder to overcome hypokinetic speech. Medical treatment, either with levodopa or deep brain surgery in general does not provide clinical improvement of swallowing.

Orofaryngeale slikstoornissen bij neurodegeneratieve aandoeningen.

OROPHARYNGEAL DYSPHAGIA IN NEURODEGENERATIVE DISEASES: Neurodegenerative diseases are progressive degenerations of the brain with increasing movement disorders, like gait, speech and swallowing disorders combined with cognitive disorders, like dementia. Neurodegenerative diseases have in common that aspiration pneumonia is the most frequent cause of death in the end-stage of the disease. This article provides an overview of the prevalence, characteristics and treatments of oropharyngeal dysphagia resulting from multiple system atrophy (MSA), progressive supranuclear palsy (PSP), Huntington's disease (HD), multiple sclerosis (MS) and dementia. The prevalence of dysphagia ranges from 32% in MS, 57% in patients with dementia to 73% in patients with MSA and almost 100% in patients with PSP or HD. Treatment is aimed at swallowing with less effort, swallowing more safely and/or optimal nutritional intake. When cognitive decline and behavioural changes increase, passive compensations like food adaptations and comfortable mealtime conditions become the principal interventions.

Diurnal and nocturnal drooling in Parkinson's disease.

Drooling as symptom of Parkinson's disease (PD) has thus far been poorly defined. This uncertainty is reflected by high variations in published prevalence rates. The aim of this study was to investigate the prevalence of saliva loss versus accumulation of saliva as a possible preliminary stage, and diurnal drooling versus nocturnal drooling. In addition, we evaluated the association between drooling severity and the severity of facial and oral motor disorders. We collected age, disease duration, UPDRS III and Hoehn & Yahr stage from 104 consecutive outpatients with PD. Diurnal and nocturnal drooling was evaluated with a validated questionnaire (ROMP-saliva). A speech pathologist, blinded for drooling severity, rated facial expression, involuntary mouth opening and difficulty with nose breathing and also interviewed patients about sleeping position and nose-breathing during the night. Thirty patients (29%) had no complaints with saliva control ('non-droolers'), 45 patients (43%) experienced accumulation of saliva or only nocturnal drooling ('pre-droolers'), and 29 (28%) had diurnal drooling (24 of which also drooled during the night; 'droolers'). The droolers had longer disease duration (10 vs. 7 years, p = 0.01) and drooling was independently associated with involuntary mouth opening (OR = 2.0; 95% CI 1.02-3.99) and swallowing complaints (OR = 1.2; 95% CI 1.03-1.31). Diurnal drooling-defined as dribbling of saliva while awake-is present in about 28% of PD patients. This is less than usually reported. Diurnal drooling typically appeared later in the disease course. The association with oral motor behavior should encourage the development of behavioral treatment approaches.

Prevalence of oropharyngeal dysphagia in Parkinson's disease: a meta-analysis.

Dysphagia is a potentially harmful feature, also in Parkinson's disease (PD). As published prevalence rates vary widely, we aimed to estimate the prevalence of oropharyngeal dysphagia in PD in a meta-analysis. We conducted a systematic literature search in February 2011 and two independent reviewers selected the papers. We computed the estimates of the pooled prevalence weighted by sample size. Twelve studies were suitable for calculating prevalence rates. Ten studies provided an estimate based on subjective outcomes, which proved statistically heterogeneous (p < 0.001), with a pooled prevalence estimate with random effect analysis of 35% (95% CI 28-41). Four studies provided an estimate based on objective measurements, which were statistically homogeneous (p = 0.23), with a pooled prevalence estimate of 82% (95% CI 77-87). In controls the pooled subjective prevalence was 9% (95% CI 2-17), while the pooled objective prevalence was 23% (95% CI 13-32). The pooled relative risk was 3.2 for both subjective outcomes (95% CI 2.32-4.41) and objective outcomes (95% CI 2.08-4.98). Clinical heterogeneity between studies was chiefly explained by differences in disease severity. Subjective dysphagia occurs in one third of community-dwelling PD patients. Objectively measured dysphagia rates were much higher, with 4 out of 5 patients being affected. This suggests that dysphagia is common in PD, but patients do not always report swallowing difficulties unless asked. This underreporting calls for a proactive clinical approach to dysphagia, particularly in light of the serious clinical consequences.

Prevalence and definition of drooling in Parkinson's disease: a systematic review.

Drooling (saliva loss) is a frequently reported symptom in patients with Parkinson's disease (PD), but an accurate estimate of the prevalence of drooling is lacking. The aim of this study was to systematically review the prevalence of drooling in published research papers. A systematic PubMed and CINAHL search was done, including studies published until January 2009. Eight studies were found, presenting prevalence rates of drooling based on responses of PD patients to questionnaires. The statistical heterogeneity was highly significant (P < 0.0001), with prevalence rates ranging from 32 to 74%. The pooled prevalence estimate with random effect analysis was of 56% (95% CI 44-67) for PD patients and 14% (95% CI 3-25) for healthy controls; the pooled relative risk (RR) with random effect analysis was 5.5 (95% CI 2.1-14.4). All studies reported data of community dwelling idiopathic PD patients, with a mean age around 65 years and mild PD in 50-60% of the cases. Heterogeneity was mainly caused by differences in definition or frequency of drooling. The highest prevalence rates included nocturnal drooling where others noted only diurnal drooling. Analysis of the data of two studies showed that drooling is reported frequently by 22-26% of the patients. Prevalence rates were lower in milder PD patients. The summarized findings demonstrate that drooling can be present in half of all PD patients. In about a quarter of PD patients, drooling appears to be a frequently occurring problem. We recommend to report drooling in future studies with more detailed consideration of severity, frequency and nocturnal versus diurnal complaints.

Multidisciplinary care for patients with Parkinson's disease.

Parkinson's disease (PD) is a chronic and progressive neurodegenerative disorder with a complex phenotype, featuring a wide variety of both motor and non-motor symptoms. Current medical management is usually monodisciplinary, with an emphasis on drug treatment, sometimes supplemented with deep brain surgery. Despite optimal medical management, most patients become progressively disabled. Allied health care may provide complementary benefits to PD patients, even for symptoms that are resistant to pharmacotherapy or surgery. This notion is increasingly supported by scientific evidence. In addition, the role of allied health care is now documented in recent clinical practice guidelines that are available for physiotherapy, occupational therapy and speech-language therapy. Unfortunately, adequate delivery of allied health care is threatened by the insufficient expertise among most therapists, and the generally low patient volumes for each individual therapist. Moreover, most allied health interventions are used in isolation, with insufficient collaboration and communication with other disciplines involved in the care for PD patients. Clinical experience suggests that optimal management requires a multidisciplinary approach, with multifactorial health plans tailored to the needs of each individual patient. Although the merits of specific allied health care interventions have been scientifically proven for other chronic disorders, only few studies have tried to provide a scientific basis for a multidisciplinary care approach in PD. The few studies published so far were not yet convincing. We conclude by providing recommendations for current multidisciplinary care in PD, while highlighting the need for future clinical trials to evaluate the cost-effectiveness of a multidisciplinary team approach.

Allied health care interventions and complementary therapies in Parkinson's disease.

Allied health care and complementary therapies are used by many patients with Parkinson's disease (PD). For allied health care, supportive scientific evidence is gradually beginning to emerge, and interventions are increasingly integrated in the treatment programs for PD patients. To evaluate whether such multidisciplinary programs are justifiable, we review the literature of allied health care and complementary therapies in PD. According to the level of available evidence, we provide recommendations for clinical practice. Finally, we discuss the need for an improved organization of allied health care, and identify topics for future research to further underpin the pros and cons of allied health care and complementary therapies in PD.

Dysphagia in facioscapulohumeral muscular dystrophy.

Dysphagia is not considered a symptom of facioscapulohumeral muscular dystrophy (FSHD). In this study, the authors found that dysphagia does occur in patients with advanced FSHD showing mild involvement of the jaw and lingual muscles. Dysphagia is seldom life threatening in these patients. The authors conclude that dysphagia should not be considered an exclusion criterion for FSHD.

Ptosis aggravates dysphagia in oculopharyngeal muscular dystrophy.

BACKGROUND: Ptosis and dysphagia are important features in oculopharyngeal muscular dystrophy (OPMD). OBJECTIVE: Retroflexion of the head is a well known compensatory mechanism for ptosis, but generally retroflexion has a negative effect on swallowing. We hypothesised that severity of ptosis is related to degree of retroflexion and that this compensation is responsible for deteriorating dysphagia. METHODS: Nine OPMD patients were examined in the conditions "head position adapted to ptosis" and "head position slightly flexed". Ptosis was quantified by photogrammetry and retroflexion of the head by digital photographs. The severity of dysphagia was measured using visual analogue scales (VAS) and by calculating swallowing volumes and oropharyngeal swallow efficiency (OPSE) based on videofluoroscopy. RESULTS: Statistical analyses show a significant relationship between ptosis and degree of retroflexion. The degree of retroflexion of the head correlated significantly with VAS scores and with the maximum swallowing volume. The slightly flexed head position significantly improved VAS scores as well as swallowing volumes and OPSE. CONCLUSION: In OPMD patients, ptosis significantly correlates with retroflexion of the head, which has a negative effect on swallowing. Subjective and objective reduction of swallowing problems was found when patients were instructed to eat and drink with a slightly flexed head position.