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1.
J Med Case Rep ; 18(1): 319, 2024 Jul 04.
Article in English | MEDLINE | ID: mdl-38961428

ABSTRACT

BACKGROUND: Myasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG), necessitating ventilation. Achieving a safe and timely diagnosis of myasthenic crisis with atypical, isolated presentation is a considerable challenge particularly in elderly patients, where myasthenia gravis can present with isolated dysarthria in rare instances, giving a clinical impression of lacunar stroke. CASE PRESENTATION: We present a compelling case of a 73-year-old Caucasian female presenting with abrupt onset of isolated dysarthria. Despite initial treatment for a presumed lacunar stroke, subsequent evaluations led to her diagnosis of a myasthenic crisis. Within 72 h of admission, the patient developed dysphagia and shortness of breath, requiring supplemental oxygen. The case highlights the sequential progression of events from the atypical presentation of isolated dysarthria and its course to the management of a myasthenic crisis. CONCLUSION: Our reported case focuses on the discussion of myasthenia that mimicked a lacunar stroke and was finally diagnosed at a critical time of medical crisis. This case highlights the imperative notion that isolated dysarthria in elderly individuals warrants vigilant monitoring for possible myasthenia gravis, given the low incidence of lacunar stroke presenting with only dysarthria.


Subject(s)
Dysarthria , Myasthenia Gravis , Stroke, Lacunar , Humans , Myasthenia Gravis/diagnosis , Myasthenia Gravis/complications , Aged , Dysarthria/etiology , Female , Diagnosis, Differential , Stroke, Lacunar/diagnosis , Stroke, Lacunar/complications , Cholinesterase Inhibitors/therapeutic use , Deglutition Disorders/etiology , Deglutition Disorders/diagnosis , Dyspnea/etiology
2.
Mol Genet Metab ; 142(3): 108510, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38843620

ABSTRACT

BACKGROUND: Information about dysarthria and dysphagia in mitochondrial diseases (MD) is scarce. However, this knowledge is needed to identify speech and swallowing problems early, to monitor the disease course, and to develop and offer optimal treatment and support. This study therefore aims to examine the prevalence and severity of dysarthria and dysphagia in patients with MD and its relation to clinical phenotype and disease severity. Secondary aim is to determine clinically relevant outcome measures for natural history studies and clinical trials. METHODS: This retrospective cross-sectional medical record study includes adults (age ≥ 18 years) diagnosed with genetically confirmed MD who participated in a multidisciplinary admission within the Radboud center for mitochondrial medicine between January 2015 and April 2023. Dysarthria and dysphagia were examined by administering the Radboud dysarthria assessment, swallowing speed, dysphagia limit, test of mastication and swallowing solids (TOMASS), and 6-min mastication test (6MMT). The disease severity was assessed using the Newcastle mitochondrial disease scale for adults (NMDAS). RESULTS: The study included 224 patients with MD with a median age of 42 years of whom 37.5% were male. The pooled prevalence of dysarthria was 33.8% and of dysphagia 35%. Patients with MD showed a negative deviation from the norm on swallowing speed, TOMASS (total time) and the 6MMT. Furthermore, a significant moderate relation was found between the presence of dysarthria and the clinical phenotypes. There was a statistically significant difference in total time on the TOMASS between the clinical phenotypes. Finally, disease severity showed a significant moderate relation with the severity of dysarthria and a significant weak relation with the severity of dysphagia. CONCLUSION: Dysarthria and dysphagia occur in about one-third of patients with MD. It is important for treating physicians to pay attention to this subject because of the influence of both disorders on social participation and wellbeing. Referral to a speech and language therapist should therefore be considered, especially in patients with a more severe clinical phenotype. The swallowing speed, TOMASS and 6MMT are the most clinically relevant tests to administer.


Subject(s)
Deglutition Disorders , Dysarthria , Mitochondrial Diseases , Humans , Deglutition Disorders/etiology , Deglutition Disorders/physiopathology , Dysarthria/etiology , Dysarthria/physiopathology , Male , Female , Mitochondrial Diseases/complications , Mitochondrial Diseases/physiopathology , Adult , Middle Aged , Retrospective Studies , Cross-Sectional Studies , Aged , Severity of Illness Index , Prevalence , Deglutition , Young Adult , Phenotype
3.
J Voice ; 2024 Jun 17.
Article in English | MEDLINE | ID: mdl-38890016

ABSTRACT

PURPOSE: This research aims to identify acoustic features which can distinguish patients with Parkinson's disease (PD patients) and healthy speakers. METHODS: Thirty PD patients and 30 healthy speakers were recruited in the experiment, and their speech was collected, including three vowels (/i/, /a/, and /u/) and nine consonants (/p/, /pÊ°/, /t/, /tÊ°/, /k/, /kÊ°/, /l/, /m/, and /n/). Acoustic features like fundamental frequency (F0), Jitter, Shimmer, harmonics-to-noise ratio (HNR), first formant (F1), second formant (F2), third formant (F3), first bandwidth (B1), second bandwidth (B2), third bandwidth (B3), voice onset, voice onset time were analyzed in our experiment. Two-sample independent t test and the nonparametric Mann-Whitney U (MWU) test were carried out alternatively to compare the acoustic measures between the PD patients and healthy speakers. In addition, after figuring out the effective acoustic features for distinguishing PD patients and healthy speakers, we adopted two methods to detect PD patients: (1) Built classifiers based on the effective acoustic features and (2) Trained support vector machine classifiers via the effective acoustic features. RESULTS: Significant differences were found between the male PD group and the male health control in vowel /i/ (Jitter and Shimmer) and /a/ (Shimmer and HNR). Among female subjects, significant differences were observed in F0 standard deviation (F0 SD) of /u/ between the two groups. Additionally, significant differences between PD group and health control were also found in the F3 of /i/ and /n/, whereas other acoustic features showed no significant differences between the two groups. The HNR of vowel /a/ performed the best classification accuracy compared with the other six acoustic features above found to distinguish PD patients and healthy speakers. CONCLUSIONS: PD can cause changes in the articulation and phonation of PD patients, wherein increases or decreases occur in some acoustic features. Therefore, the use of acoustic features to detect PD is expected to be a low-cost and large-scale diagnostic method.

4.
Psychol Res Behav Manag ; 17: 2205-2232, 2024.
Article in English | MEDLINE | ID: mdl-38835654

ABSTRACT

Purpose: Speech disorders profoundly impact the overall quality of life by impeding social operations and hindering effective communication. This study addresses the gap in systematic reviews concerning machine learning-based assistive technology for individuals with speech disorders. The overarching purpose is to offer a comprehensive overview of the field through a Systematic Literature Review (SLR) and provide valuable insights into the landscape of ML-based solutions and related studies. Methods: The research employs a systematic approach, utilizing a Systematic Literature Review (SLR) methodology. The study extensively examines the existing literature on machine learning-based assistive technology for speech disorders. Specific attention is given to ML techniques, characteristics of exploited datasets in the training phase, speaker languages, feature extraction techniques, and the features employed by ML algorithms. Originality: This study contributes to the existing literature by systematically exploring the machine learning landscape in assistive technology for speech disorders. The originality lies in the focused investigation of ML-speech recognition for impaired speech disorder users over ten years (2014-2023). The emphasis on systematic research questions related to ML techniques, dataset characteristics, languages, feature extraction techniques, and feature sets adds a unique and comprehensive perspective to the current discourse. Findings: The systematic literature review identifies significant trends and critical studies published between 2014 and 2023. In the analysis of the 65 papers from prestigious journals, support vector machines and neural networks (CNN, DNN) were the most utilized ML technique (20%, 16.92%), with the most studied disease being Dysarthria (35/65, 54% studies). Furthermore, an upsurge in using neural network-based architectures, mainly CNN and DNN, was observed after 2018. Almost half of the included studies were published between 2021 and 2022).

5.
Eur J Paediatr Neurol ; 51: 62-70, 2024 May 29.
Article in English | MEDLINE | ID: mdl-38851145

ABSTRACT

RESEARCH PURPOSE: GLUT1 deficiency syndrome (GLUT1DS) is a rare genetic disorder caused by a mutation in the SLC2A1 gene that limits the transport of glucose across the blood-brain barrier. Speech disorders and dysarthria are typical findings in patients with GLUT1DS, but have never been deeply phenotyped. The aim of the present study was to characterize speech abilities in a sample of patients with GLUT1DS. RESULTS: 30 patients with GLUT1DS were recruited. We reported impairments in different speech and oromotor domains: the speech was characterized by dysarthria, inaccurate articulation of consonants, abnormal nasal resonance, errors in intonation and prosody and low intelligibility. We observed difficulties in motor planning and programming. Moreover, we observed a significant difference between the dysarthric level of impairment with genotype groups. CONCLUSIONS: The presence of a speech disorder in patients with GLUT1DS represents a core feature of the syndrome. Our findings suggest that patients with GLUT1DS would benefit from a comprehensive neurocognitive assessment to detect strengths and weaknesses of the speech profile. Understanding the speech and language phenotype in GLUT1DS is critical for planning early intervention to positively influence the global development of patients with GLUT1DS.

6.
Brain Commun ; 6(3): fcae177, 2024.
Article in English | MEDLINE | ID: mdl-38846538

ABSTRACT

Up to half of all people with multiple sclerosis experience communication difficulties due to dysarthria, a disorder that impacts the motor aspects of speech production. Dysarthria in multiple sclerosis is linked to cerebellar dysfunction, disease severity and lesion load, but the neuroanatomical substrates of these symptoms remain unclear. In this study, 52 participants with multiple sclerosis and 14 age- and sex-matched healthy controls underwent structural and diffusion MRI, clinical assessment of disease severity and cerebellar dysfunction and a battery of motor speech tasks. Assessments of regional brain volume and white matter integrity, and their relationships with clinical and speech measures, were undertaken. White matter tracts of interest included the interhemispheric sensorimotor tract, cerebello-thalamo-cortical tract and arcuate fasciculus, based on their roles in motor and speech behaviours. Volumetric analyses were targeted to Broca's area, Wernicke's area, the corpus callosum, thalamus and cerebellum. Our results indicated that multiple sclerosis participants scored worse on all motor speech tasks. Fixel-based diffusion MRI analyses showed significant evidence of white matter tract atrophy in each tract of interest. Correlational analyses further indicated that higher speech naturalness-a perceptual measure of dysarthria-and lower reading rate were associated with axonal damage in the interhemispheric sensorimotor tract and left arcuate fasciculus in people with multiple sclerosis. Axonal damage in all tracts of interest also correlated with clinical scales sensitive to cerebellar dysfunction. Participants with multiple sclerosis had lower volumes of the thalamus and corpus callosum compared with controls, although no brain volumetrics correlated with measures of dysarthria. These findings indicate that axonal damage, particularly when measured using diffusion metrics, underpin dysarthria in multiple sclerosis.

7.
Front Hum Neurosci ; 18: 1383714, 2024.
Article in English | MEDLINE | ID: mdl-38812472

ABSTRACT

The purpose of this article is to review the scientific literature concerning speech in Parkinson's disease (PD) with reference to the DIVA/GODIVA neurocomputational modeling framework. Within this theoretical view, the basal ganglia (BG) contribute to several different aspects of speech motor learning and execution. First, the BG are posited to play a role in the initiation and scaling of speech movements. Within the DIVA/GODIVA framework, initiation and scaling are carried out by initiation map nodes in the supplementary motor area acting in concert with the BG. Reduced support of the initiation map from the BG in PD would result in reduced movement intensity as well as susceptibility to early termination of movement. A second proposed role concerns the learning of common speech sequences, such as phoneme sequences comprising words; this view receives support from the animal literature as well as studies identifying speech sequence learning deficits in PD. Third, the BG may play a role in the temporary buffering and sequencing of longer speech utterances such as phrases during conversational speech. Although the literature does not support a critical role for the BG in representing sequence order (since incorrectly ordered speech is not characteristic of PD), the BG are posited to contribute to the scaling of individual movements in the sequence, including increasing movement intensity for emphatic stress on key words. Therapeutic interventions for PD have inconsistent effects on speech. In contrast to dopaminergic treatments, which typically either leave speech unchanged or lead to minor improvements, deep brain stimulation (DBS) can degrade speech in some cases and improve it in others. However, cases of degradation may be due to unintended stimulation of efferent motor projections to the speech articulators. Findings of spared speech after bilateral pallidotomy appear to indicate that any role played by the BG in adult speech must be supplementary rather than mandatory, with the sequential order of well-learned sequences apparently represented elsewhere (e.g., in cortico-cortical projections).

8.
Clin Linguist Phon ; : 1-22, 2024 May 01.
Article in English | MEDLINE | ID: mdl-38691845

ABSTRACT

Speakers with motor speech disorders (MSD) present challenges in speech production, one of them being the difficulty to adapt their speech to different modes. However, it is unclear whether different types of MSD are similarly affected when it comes to adapting their speech to various communication contexts. This study investigates the encoding of speech modes in individuals with AoS following focal brain damage and in individuals with hypokinetic dysarthria (HD) secondary to Parkinson's disease. Participants with mild-to-moderate MSD and their age-matched controls performed a delayed production task of pseudo-words in two speech modes: normal and whispered speech. While overall accuracy did not differ significantly across speech modes, participants with AoS exhibited longer response latencies for whispered speech, reflecting difficulties in the initiation of utterances requiring an unvoiced production. In contrast, participants with HD showed faster response latencies for whispered speech, indicating that this speech mode is easier to encode/control for this population. Acoustic durations followed these same trends, with participants with AoS showing greater lengthening for whispered speech as compared to controls and to participants with HD, while participants with HD exhibited milder lengthening. Contrary to the predictions of speech production models, suggesting that speech mode changes might be particularly difficult in dysarthria, the present results suggest that speech mode adaptation rather seems particularly costly for participants with AoS.

9.
J Commun Disord ; 109: 106428, 2024.
Article in English | MEDLINE | ID: mdl-38744198

ABSTRACT

PURPOSE: This study examines whether there are differences in the speech of speakers with dysarthria, speakers with apraxia and healthy speakers in spectral acoustic measures during production of the central-peninsular Spanish alveolar sibilant fricative /s/. METHOD: To this end, production of the sibilant was analyzed in 20 subjects with dysarthria, 8 with apraxia of speech and 28 healthy speakers. Participants produced 12 sV(C) words. The variables compared across groups were the fricative's spectral amplitude difference (AmpD) and spectral moments in the temporal midpoint of fricative execution. RESULTS: The results indicate that individuals with dysarthria can be distinguished from healthy speakers in terms of the spectral characteristics AmpD, standard deviation (SD), center of gravity (CoG) and skewness, the last two in context with unrounded vowel, while no differences in kurtosis were detected. Participants with AoS group differ significantly from healthy speaker group in AmpD, SD and CoG and Kurtosis, the first one followed unrounded vowel and the latter two followed by rounded vowels. In addition, speakers with apraxia of speech group returned significant differences with respect to speakers with dysarthria group in AmpD, CoG and skewness. CONCLUSIONS: The differences found between the groups in the measures studied as a function of the type of vowel context could provide insights into the distinctive manifestations of motor speech disorders, contributing to the differential diagnosis between apraxia and dysarthria in motor control processes.


Subject(s)
Apraxias , Dysarthria , Speech Acoustics , Humans , Dysarthria/physiopathology , Dysarthria/etiology , Apraxias/physiopathology , Male , Female , Middle Aged , Adult , Aged , Phonetics , Speech Production Measurement
10.
J Neural Transm (Vienna) ; 131(7): 791-797, 2024 Jul.
Article in English | MEDLINE | ID: mdl-38592459

ABSTRACT

INTRODUCTION: Hypokinetic dysarthria (HD) is a common motor speech symptom of Parkinson's disease (PD) which does not respond well to PD treatments. We investigated short-term effects of transcranial direct current stimulation (tDCS) on HD in PD using acoustic analysis of speech. Based on our previous studies we focused on stimulation of the right superior temporal gyrus (STG) - an auditory feedback area. METHODS: In 14 PD patients with HD, we applied anodal, cathodal and sham tDCS to the right STG using a cross-over design. A protocol consisting of speech tasks was performed prior to and immediately after each stimulation session. Linear mixed models were used for the evaluation of the effects of each stimulation condition on the relative change of acoustic parameters. We also performed a simulation of the mean electric field induced by tDCS. RESULTS: Linear mixed model showed a statistically significant effect of the stimulation condition on the relative change of median duration of silences longer than 50 ms (p = 0.015). The relative change after the anodal stimulation (mean = -5.9) was significantly lower as compared to the relative change after the sham stimulation (mean = 12.8), p = 0.014. We also found a correlation between the mean electric field magnitude in the right STG and improvement of articulation precision after anodal tDCS (R = 0.637; p = 0.019). CONCLUSIONS: The exploratory study showed that anodal tDCS applied over the auditory feedback area may lead to shorter pauses in a speech of PD patients.


Subject(s)
Parkinson Disease , Transcranial Direct Current Stimulation , Humans , Parkinson Disease/therapy , Parkinson Disease/complications , Parkinson Disease/physiopathology , Pilot Projects , Male , Female , Aged , Middle Aged , Cross-Over Studies , Dysarthria/etiology , Dysarthria/therapy , Dysarthria/physiopathology , Speech/physiology , Treatment Outcome , Temporal Lobe/physiopathology
11.
Cureus ; 16(3): e56098, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38618373

ABSTRACT

Metronidazole-induced acute cerebellitis is an exceptionally rare condition resulting from severe adverse reactions to metronidazole, a medication generally employed in the management of infections caused by anaerobic microbes. Although neuropathy has been linked to metronidazole use, reports of acute cerebellitis are infrequent. The neurological effects associated with metronidazole can include weakness, dysarthria, postural instability, seizures, giddiness, vertigo, ataxia, confusion, encephalopathy, headaches, and tremors. The onset of cerebellitis can vary, occurring as early as one day or after several weeks of metronidazole treatment. This article presents a case of a young girl who presented to us with weakness in both upper and lower limbs, dysarthria, and postural instability after exposure to 12 grams of metronidazole (suicidal, 30 tablets of 400 mg). With the above-mentioned complaints, the patient was advised of magnetic resonance imaging of the brain, which showed the features of cerebellitis.

12.
Cerebellum ; 2024 Apr 20.
Article in English | MEDLINE | ID: mdl-38642239

ABSTRACT

Cerebellar pathology engenders the disturbance of movement that characterizes Friedreich ataxia (FRDA), yet the impact of cerebellar pathology on cognition in FRDA remains unclear. Numerous studies have unequivocally demonstrated the role of the cerebellar pathology in disturbed cognitive, language and affective regulation, referred to as Cerebellar Cognitive Affective Syndrome (CCAS), and quantified by the CCAS-Scale (CCAS-S). The presence of dysarthria in many individuals with ataxia, particularly FRDA, may confound results on some items of the CCAS-S resulting in false-positive scores. This study explored the relationship between performance on the CCAS-S and clinical metrics of disease severity in 57 adults with FRDA. In addition, this study explored the relationship between measures of intelligibility and naturalness of speech and scores on the CCAS-S in a subgroup of 39 individuals with FRDA. We demonstrated a significant relationship between clinical metrics and performance on the CCAS-S. In addition, we confirmed the items that returned the greatest rate of failure were based on Verbal Fluency Tasks, revealing a significant relationship between these items and measures of speech. Measures of speech explained over half of the variance in the CCAS-S score suggesting the role of dysarthria in the performance on the CCAS-S is not clear. Further work is required prior to adopting the CCAS-S as a cognitive screening tool for individuals with FRDA.

13.
IEEE J Transl Eng Health Med ; 12: 382-389, 2024.
Article in English | MEDLINE | ID: mdl-38606392

ABSTRACT

Acoustic features extracted from speech can help with the diagnosis of neurological diseases and monitoring of symptoms over time. Temporal segmentation of audio signals into individual words is an important pre-processing step needed prior to extracting acoustic features. Machine learning techniques could be used to automate speech segmentation via automatic speech recognition (ASR) and sequence to sequence alignment. While state-of-the-art ASR models achieve good performance on healthy speech, their performance significantly drops when evaluated on dysarthric speech. Fine-tuning ASR models on impaired speech can improve performance in dysarthric individuals, but it requires representative clinical data, which is difficult to collect and may raise privacy concerns. This study explores the feasibility of using two augmentation methods to increase ASR performance on dysarthric speech: 1) healthy individuals varying their speaking rate and loudness (as is often used in assessments of pathological speech); 2) synthetic speech with variations in speaking rate and accent (to ensure more diverse vocal representations and fairness). Experimental evaluations showed that fine-tuning a pre-trained ASR model with data from these two sources outperformed a model fine-tuned only on real clinical data and matched the performance of a model fine-tuned on the combination of real clinical data and synthetic speech. When evaluated on held-out acoustic data from 24 individuals with various neurological diseases, the best performing model achieved an average word error rate of 5.7% and a mean correct count accuracy of 94.4%. In segmenting the data into individual words, a mean intersection-over-union of 89.2% was obtained against manual parsing (ground truth). It can be concluded that emulated and synthetic augmentations can significantly reduce the need for real clinical data of dysarthric speech when fine-tuning ASR models and, in turn, for speech segmentation.


Subject(s)
Speech Perception , Speech , Humans , Speech Recognition Software , Dysarthria/diagnosis , Speech Disorders
14.
Front Neurol ; 15: 1337960, 2024.
Article in English | MEDLINE | ID: mdl-38660095

ABSTRACT

Poststroke seizure is a potential complication of stroke, which is the most frequent acute symptomatic seizure in adults. Patients with stroke may present with an abnormal or aggressive behavior accompanied by altered mental status and symptoms, such as hemiparesis, dysarthria, and sensory deficits. Although stroke manifestations that mimic seizures are rare, diagnosing poststroke seizures can be challenging when accompanied with negative postictal symptoms. Differential diagnoses of poststroke seizures include movement disorders, syncope, and functional (nonepileptic) seizures, which may present with symptoms similar to seizures. Furthermore, it is important to determine whether poststroke seizures occur early or late. Seizures occurring within and after 7 d of stroke onset were classified as early and late seizures, respectively. Early seizures have the same clinical course as acute symptomatic seizures; they rarely recur or require long-term antiseizure medication. Conversely, late seizures are associated with a risk of recurrence similar to that of unprovoked seizures in a patient with a focal lesion, thereby requiring long-term administration of antiseizure medication. After diagnosis, concerns regarding treatment strategies, treatment duration, and administration of primary and secondary prophylaxis often arise. Antiseizure medication decisions for the initiation of short-term primary and long-term secondary seizure prophylaxis should be considered for patients with stroke. Antiseizure drugs such as lamotrigine, carbamazepine, lacosamide, levetiracetam, phenytoin, and valproate may be administered. Poststroke seizures should be diagnosed systematically through history with differential diagnosis; in addition, classifying them as early or late seizures can help to determine treatment strategies.

15.
Clin Case Rep ; 12(3): e8628, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38464574

ABSTRACT

Key Clinical Message: Legionnaires' disease, often presenting as pneumonia, can uncommonly manifest with extrapulmonary symptoms such as cerebellar involvement and rhabdomyolysis. This case emphasizes the successful resolution of dysarthria and renal dysfunction with prompt Legionella treatment, underscoring the importance of vigilance for diverse manifestations in Legionella infections. Abstract: Legionnaires' disease usually presents with pneumonia and a few extrapulmonary manifestations, such as neurological, musculoskeletal, and cutaneous manifestations. However, cerebellar involvement and rhabdomyolysis as an association with Legionella are not frequently encountered. We present a case of Legionella-induced rhabdomyolysis requiring hemodialysis and dysarthria that resolved with Legionella treatment.

16.
Front Hum Neurosci ; 18: 1353150, 2024.
Article in English | MEDLINE | ID: mdl-38454907

ABSTRACT

Deep brain stimulation (DBS) is an effective surgical therapy for carefully selected patients with medication refractory essential tremor (ET). The most popular anatomical targets for ET DBS are the ventral intermedius nucleus (VIM) of the thalamus, the caudal zona incerta (cZI) and the posterior subthalamic area (PSA). Despite extensive knowledge in DBS programming for tremor suppression, it is not uncommon to experience stimulation induced side effects related to DBS therapy. Dysarthria, dysphagia, ataxia, and gait impairment are common stimulation induced side effects from modulation of brain tissue that surround the target of interest. In this review, we explore current evidence about the etiology of stimulation induced side effects in ET DBS and provide several evidence-based strategies to troubleshoot, reprogram and retain tremor suppression.

17.
Cerebellum ; 2024 Mar 07.
Article in English | MEDLINE | ID: mdl-38448793

ABSTRACT

The progression of multisystem neurodegenerative diseases such as ataxia significantly impacts speech and communication, necessitating adaptive clinical care strategies. With the deterioration of speech, Alternative and Augmentative Communication (AAC) can play an ever increasing role in daily life for individuals with ataxia. This review describes the spectrum of AAC resources available, ranging from unaided gestures and sign language to high-tech solutions like speech-generating devices (SGDs) and eye-tracking technology. Despite the availability of various AAC tools, their efficacy is often compromised by the physical limitations inherent in ataxia, including upper limb ataxia and visual disturbances. Traditional speech-to-text algorithms and eye gaze technology face challenges in accuracy and efficiency due to the atypical speech and movement patterns associated with the disease.In addressing these challenges, maintaining existing speech abilities through rehabilitation is prioritized, complemented by advances in digital therapeutics to provide home-based treatments. Simultaneously, projects incorporating AI driven solutions aim to enhance the intelligibility of dysarthric speech through improved speech-to-text accuracy.This review discusses the complex needs assessment for AAC in ataxia, emphasizing the dynamic nature of the disease and the importance of regular reassessment to tailor communication strategies to the changing abilities of the individual. It also highlights the necessity of multidisciplinary involvement for effective AAC assessment and intervention. The future of AAC looks promising with developments in brain-computer interfaces and the potential of voice banking, although their application in ataxia requires further exploration.

18.
IBRO Neurosci Rep ; 16: 361-367, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38425546

ABSTRACT

Background: Deep Brain Stimulation (DBS) in the Subthalamic Nucleus (STN) or the Globus Pallidus Interna (GPI) is well-established as a surgical technique for improving global motor function in patients with idiopathic Parkinson's Disease (PD). Previous research has indicated speech deterioration in more than 30% of patients after STN-DBS implantation, whilst speech outcomes following GPI-DBS have received far less attention. Research comparing speech outcomes for patients with PD receiving STN-DBS and GPI-DBS can inform pre-surgical counseling and assist with clinician and patient decision-making when considering the neural targets selected for DBS-implantation. The aims of this pilot study were (1) to compare perceptual and acoustic speech outcomes for a group of patients with PD receiving bilateral DBS in the STN or the GPI with DBS stimulation both ON and OFF, and (2) examine associations between acoustic and perceptual speech measures and clinical characteristics. Methods: Ten individuals with PD receiving STN-DBS and eight individuals receiving GPI-DBS were audio-recorded reading a passage. Three listeners blinded to neural target and stimulation condition provided perceptual judgments of intelligibility and overall speech severity. Speech acoustic measures were obtained from the recordings. Acoustic and perceptual measures and clinical characteristics were compared for the two neural targets and stimulation conditions. Results: Intelligibility and speech severity were not significantly different across neural target or stimulation conditions. Generally, acoustic measures were also not statistically different for the two neural targets or stimulation conditions. Acoustic measures reflecting more varied speech prosody were associated with improved intelligibility and lessened severity. Convergent correlations were found between UPDRS-III speech scores and perceptual measures of intelligibility and severity. Conclusion: This study reports a systematic comparison of perceptual and acoustic speech outcomes following STN-DBS and GPI-DBS. Statistically significant differences in acoustic measures for the two neural targets were small in magnitude and did not yield group differences in perceptual measures. The absence of robust differences in speech outcomes for the two neural targets has implications for pre-surgical counseling. Results provide preliminary support for reliance on considerations other than speech when selecting the target for DBS in patients with PD.

19.
J Med Internet Res ; 26: e56417, 2024 Apr 25.
Article in English | MEDLINE | ID: mdl-38509662

ABSTRACT

BACKGROUND: Dysarthria is a common poststroke speech disorder affecting communication and psychological well-being. Traditional speech therapy is effective but often poses challenges in terms of accessibility and patient adherence. Emerging smartphone-based therapies may offer promising alternatives for the treatment of poststroke dysarthria. OBJECTIVE: This study aimed to assess the efficacy and feasibility of smartphone-based speech therapy for improving speech intelligibility in patients with acute and early subacute poststroke dysarthria. This study also explored the impact of the intervention on psychological well-being, user experience, and overall feasibility in a clinical setting. METHODS: Participants were divided into 2 groups for this randomized, evaluator-blinded trial. The intervention group used a smartphone-based speech therapy app for 1 hour per day, 5 days per week, for 4 weeks, with guideline-based standard stroke care. The control group received standard guideline-based stroke care and rehabilitation. Speech intelligibility, psychological well-being, quality of life, and user acceptance were assessed using repeated measures ANOVA. RESULTS: In this study, 40 patients with poststroke dysarthria were enrolled, 32 of whom completed the trial (16 in each group). The intervention group showed significant improvements in speech intelligibility compared with the control group. This was evidenced by improvements from baseline (F1,30=34.35; P<.001), between-group differences (F1,30=6.18; P=.02), and notable time-by-group interactions (F1,30=6.91; P=.01). Regarding secondary outcomes, the intervention led to improvements in the percentage of correct consonants over time (F1,30=5.57; P=.03). In addition, significant reductions were noted in the severity of dysarthria in the intervention group over time (F1,30=21.18; P<.001), with a pronounced group effect (F1,30=5.52; P=.03) and time-by-group interaction (F1,30=5.29; P=.03). Regarding quality of life, significant improvements were observed as measured by the EQ-5D-3L questionnaire (F1,30=13.25; P<.001) and EQ-VAS (F1,30=7.74; P=.009) over time. The adherence rate to the smartphone-based app was 64%, with over half of the participants completing all the sessions. The usability of the app was rated high (system usability score 80.78). In addition, the intervention group reported increased self-efficacy in using the app compared with the control group (F1,30=10.81; P=.003). CONCLUSIONS: The smartphone-based speech therapy app significantly improved speech intelligibility, articulation, and quality of life in patients with poststroke dysarthria. These findings indicate that smartphone-based speech therapy can be a useful assistant device in the management of poststroke dysarthria, particularly in the acute and early subacute stroke stages. TRIAL REGISTRATION: ClinicalTrials.gov NCT05146765; https://clinicaltrials.gov/ct2/show/NCT05146765.


Subject(s)
Dysarthria , Feasibility Studies , Smartphone , Speech Therapy , Stroke , Humans , Dysarthria/therapy , Dysarthria/etiology , Speech Therapy/methods , Male , Female , Pilot Projects , Middle Aged , Stroke/complications , Aged , Quality of Life , Stroke Rehabilitation/methods , Mobile Applications , Treatment Outcome
20.
Front Immunol ; 15: 1308068, 2024.
Article in English | MEDLINE | ID: mdl-38524138

ABSTRACT

Background: Autoimmune nodopathy (AN) has emerged as a novel diagnostic category that is pathologically different from classic chronic inflammatory demyelinating polyneuropathy. Clinical manifestations of AN include sensory or motor neuropathies, sensory ataxia, tremor, and cranial nerve involvement. AN with a serum-positive contactin-1 (CNTN1) antibody usually results in peripheral nerve demyelination. In this study, we reported a rare case of AN with CNTN1 antibodies characterized by the presence of CNTN1 antibodies in both serum and cerebrospinal fluid, which is associated with cerebellar dysarthria. Methods: A 25-year-old man was admitted to our hospital due to progressive dysarthria with limb tremors. The patient was initially diagnosed with peripheral neuropathy at a local hospital. Three years after onset, he was admitted to our hospital due to dysarthria, apparent limb tremor, and limb weakness. At that time, he was diagnosed with spinocerebellar ataxia. Eight years post-onset, during his second admission, his condition had notably deteriorated. His dysarthria had evolved to typical distinctive cerebellar characteristics, such as tremor, loud voice, stress, and interrupted articulation. Additionally, he experienced further progression in limb weakness and developed muscle atrophy in the distal limbs. Magnetic resonance imaging (MRI), nerve conduction studies (NCS), and autoimmune antibody tests were performed. Results: The results of the NCS suggested severe demyelination and even axonal damage to the peripheral nerves. MRI scans revealed diffuse thickening of bilateral cervical nerve roots, lumbosacral nerve roots, cauda equina nerve, and multiple intercostal nerve root sheath cysts. Furthermore, anti-CNTN1 antibody titers were 1:10 in the cerebrospinal fluid (CSF) and 1:100 in the serum. After one round of rituximab treatment, the patient showed significant improvement in limb weakness and dysarthria, and the CSF antibodies turned negative. Conclusion: Apart from peripheral neuropathies, cerebellar dysarthria (central nervous system involvement) should not be ignored in AN patients with CNTN1 antibodies.


Subject(s)
Dysarthria , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Male , Humans , Adult , Dysarthria/complications , Tremor/complications , Contactin 1 , Ataxia
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