RESUMO
BACKGROUND: Brain-computer interfaces can enable communication for people with paralysis by transforming cortical activity associated with attempted speech into text on a computer screen. Communication with brain-computer interfaces has been restricted by extensive training requirements and limited accuracy. METHODS: A 45-year-old man with amyotrophic lateral sclerosis (ALS) with tetraparesis and severe dysarthria underwent surgical implantation of four microelectrode arrays into his left ventral precentral gyrus 5 years after the onset of the illness; these arrays recorded neural activity from 256 intracortical electrodes. We report the results of decoding his cortical neural activity as he attempted to speak in both prompted and unstructured conversational contexts. Decoded words were displayed on a screen and then vocalized with the use of text-to-speech software designed to sound like his pre-ALS voice. RESULTS: On the first day of use (25 days after surgery), the neuroprosthesis achieved 99.6% accuracy with a 50-word vocabulary. Calibration of the neuroprosthesis required 30 minutes of cortical recordings while the participant attempted to speak, followed by subsequent processing. On the second day, after 1.4 additional hours of system training, the neuroprosthesis achieved 90.2% accuracy using a 125,000-word vocabulary. With further training data, the neuroprosthesis sustained 97.5% accuracy over a period of 8.4 months after surgical implantation, and the participant used it to communicate in self-paced conversations at a rate of approximately 32 words per minute for more than 248 cumulative hours. CONCLUSIONS: In a person with ALS and severe dysarthria, an intracortical speech neuroprosthesis reached a level of performance suitable to restore conversational communication after brief training. (Funded by the Office of the Assistant Secretary of Defense for Health Affairs and others; BrainGate2 ClinicalTrials.gov number, NCT00912041.).
Assuntos
Esclerose Lateral Amiotrófica , Interfaces Cérebro-Computador , Disartria , Fala , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Lateral Amiotrófica/complicações , Esclerose Lateral Amiotrófica/reabilitação , Calibragem , Auxiliares de Comunicação para Pessoas com Deficiência , Disartria/reabilitação , Disartria/etiologia , Eletrodos Implantados , Microeletrodos , Quadriplegia/etiologia , Quadriplegia/reabilitaçãoRESUMO
It is debated whether primary progressive apraxia of speech (PPAOS) and progressive agrammatic aphasia (PAA) belong to the same clinical spectrum, traditionally termed non-fluent/agrammatic variant primary progressive aphasia (nfvPPA), or exist as two completely distinct syndromic entities with specific pathologic/prognostic correlates. We analysed speech, language and disease severity features in a comprehensive cohort of patients with progressive motor speech impairment and/or agrammatism to ascertain evidence of naturally occurring, clinically meaningful non-overlapping syndromic entities (e.g. PPAOS and PAA) in our data. We also assessed if data-driven latent clinical dimensions with aetiologic/prognostic value could be identified. We included 98 participants, 43 of whom had an autopsy-confirmed neuropathological diagnosis. Speech pathologists assessed motor speech features indicative of dysarthria and apraxia of speech (AOS). Quantitative expressive/receptive agrammatism measures were obtained and compared with healthy controls. Baseline and longitudinal disease severity was evaluated using the Clinical Dementia Rating Sum of Boxes (CDR-SB). We investigated the data's clustering tendency and cluster stability to form robust symptom clusters and employed principal component analysis to extract data-driven latent clinical dimensions (LCD). The longitudinal CDR-SB change was estimated using linear mixed-effects models. Of the participants included in this study, 93 conformed to previously reported clinical profiles (75 with AOS and agrammatism, 12 PPAOS and six PAA). The remaining five participants were characterized by non-fluent speech, executive dysfunction and dysarthria without apraxia of speech or frank agrammatism. No baseline clinical features differentiated between frontotemporal lobar degeneration neuropathological subgroups. The Hopkins statistic demonstrated a low cluster tendency in the entire sample (0.45 with values near 0.5 indicating random data). Cluster stability analyses showed that only two robust subgroups (differing in agrammatism, executive dysfunction and overall disease severity) could be identified. Three data-driven components accounted for 71% of the variance [(i) severity-agrammatism; (ii) prominent AOS; and (iii) prominent dysarthria]. None of these data-driven LCDs allowed an accurate prediction of neuropathology. The severity-agrammatism component was an independent predictor of a faster CDR-SB increase in all the participants. Higher dysarthria severity, reduced words per minute and expressive and receptive agrammatism severity at baseline independently predicted accelerated disease progression. Our findings indicate that PPAOS and PAA, rather than exist as completely distinct syndromic entities, constitute a clinical continuum. In our cohort, splitting the nfvPPA spectrum into separate clinical phenotypes did not improve clinical-pathological correlations, stressing the need for new biological markers and consensus regarding updated terminology and clinical classification.
Assuntos
Afasia Primária Progressiva , Apraxias , Afasia Primária Progressiva não Fluente , Humanos , Afasia de Broca/patologia , Disartria , Apraxias/patologia , Idioma , FalaRESUMO
The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) is a neurodegenerative syndrome primarily defined by the presence of apraxia of speech (AoS) and/or expressive agrammatism. In addition, many patients exhibit dysarthria and/or receptive agrammatism. This leads to substantial phenotypic variation within the speech-language domain across individuals and time, in terms of both the specific combination of symptoms as well as their severity. How to resolve such phenotypic heterogeneity in nfvPPA is a matter of debate. 'Splitting' views propose separate clinical entities: 'primary progressive apraxia of speech' when AoS occurs in the absence of expressive agrammatism, 'progressive agrammatic aphasia' (PAA) in the opposite case, and 'AOS + PAA' when mixed motor speech and language symptoms are clearly present. While therapeutic interventions typically vary depending on the predominant symptom (e.g. AoS versus expressive agrammatism), the existence of behavioural, anatomical and pathological overlap across these phenotypes argues against drawing such clear-cut boundaries. In the current study, we contribute to this debate by mapping behaviour to brain in a large, prospective cohort of well characterized patients with nfvPPA (n = 104). We sought to advance scientific understanding of nfvPPA and the neural basis of speech-language by uncovering where in the brain the degree of MRI-based atrophy is associated with inter-patient variability in the presence and severity of AoS, dysarthria, expressive agrammatism or receptive agrammatism. Our cross-sectional examination of brain-behaviour relationships revealed three main observations. First, we found that the neural correlates of AoS and expressive agrammatism in nfvPPA lie side by side in the left posterior inferior frontal lobe, explaining their behavioural dissociation/association in previous reports. Second, we identified a 'left-right' and 'ventral-dorsal' neuroanatomical distinction between AoS versus dysarthria, highlighting (i) that dysarthria, but not AoS, is significantly influenced by tissue loss in right-hemisphere motor-speech regions; and (ii) that, within the left hemisphere, dysarthria and AoS map onto dorsally versus ventrally located motor-speech regions, respectively. Third, we confirmed that, within the large-scale grammar network, left frontal tissue loss is preferentially involved in expressive agrammatism and left temporal tissue loss in receptive agrammatism. Our findings thus contribute to define the function and location of the epicentres within the large-scale neural networks vulnerable to neurodegenerative changes in nfvPPA. We propose that nfvPPA be redefined as an umbrella term subsuming a spectrum of speech and/or language phenotypes that are closely linked by the underlying neuroanatomy and neuropathology.
Assuntos
Afasia Primária Progressiva , Apraxias , Afasia Primária Progressiva não Fluente , Humanos , Afasia de Broca/patologia , Estudos Prospectivos , Disartria , Fala , Estudos Transversais , Apraxias/patologia , Afasia Primária Progressiva/patologia , Afasia Primária Progressiva não Fluente/complicaçõesRESUMO
Mitochondrial membrane protein-associated neurodegeneration (MPAN) is an ultraorphan neurogenetic disease from the group of neurodegeneration with brain iron accumulation (NBIA) disorders. Here we report cross-sectional and longitudinal data to define the phenotype, to assess disease progression and to estimate sample sizes for clinical trials. We enrolled patients with genetically confirmed MPAN from the Treat Iron-Related Childhood-Onset Neurodegeneration (TIRCON) registry and cohort study, and from additional sites. Linear mixed-effect modelling (LMEM) was used to calculate annual progression rates for the Unified Parkinson's Disease Rating Scale (UPDRS), Barry-Albright Dystonia (BAD) scale, Schwab and England Activities of Daily Living (SE-ADL) scale and the Pediatric Quality of Life Inventory (PedsQL). We investigated 85 MPAN patients cross-sectionally, with functional outcome data collected in 45. Median age at onset was 9â years and the median diagnostic delay was 5â years. The most common findings were gait disturbance (99%), pyramidal involvement (95%), dysarthria (90%), vision disturbances (82%), with all but dysarthria presenting early in the disease course. After 16â years with the disease, 50% of patients were wheelchair dependent. LMEM showed an annual progression rate of 4.5 points in total UPDRS. The total BAD scale score showed no significant progression over time. The SE-ADL scale and the patient- and parent-reported PedsQL showed a decline of 3.9%, 2.14 and 2.05 points, respectively. No patient subpopulations were identified based on longitudinal trajectories. Our cross-sectional results define the order of onset and frequency of symptoms in MPAN, which will inform the diagnostic process, help to shorten diagnostic delay and aid in counselling patients, parents and caregivers. Our longitudinal findings define the natural history of MPAN, reveal the most responsive outcomes and highlight the need for an MPAN-specific rating approach. Our sample size estimations inform the design of upcoming clinical trials.
Assuntos
Distonia , Distúrbios Distônicos , Doenças Neurodegenerativas , Criança , Humanos , Disartria , Estudos de Coortes , Atividades Cotidianas , Estudos Transversais , Diagnóstico Tardio , Qualidade de Vida , Mutação/genética , Doenças Neurodegenerativas/genética , Fenótipo , Proteínas de Membrana/genética , Membranas MitocondriaisRESUMO
Speech disorders are associated with different degrees of functional and structural abnormalities. However, the abnormalities associated with specific disorders, and the common abnormalities shown by all disorders, remain unclear. Herein, a meta-analysis was conducted to integrate the results of 70 studies that compared 1843 speech disorder patients (dysarthria, dysphonia, stuttering, and aphasia) to 1950 healthy controls in terms of brain activity, functional connectivity, gray matter, and white matter fractional anisotropy. The analysis revealed that compared to controls, the dysarthria group showed higher activity in the left superior temporal gyrus and lower activity in the left postcentral gyrus. The dysphonia group had higher activity in the right precentral and postcentral gyrus. The stuttering group had higher activity in the right inferior frontal gyrus and lower activity in the left inferior frontal gyrus. The aphasia group showed lower activity in the bilateral anterior cingulate gyrus and left superior frontal gyrus. Across the four disorders, there were concurrent lower activity, gray matter, and fractional anisotropy in motor and auditory cortices, and stronger connectivity between the default mode network and frontoparietal network. These findings enhance our understanding of the neural basis of speech disorders, potentially aiding clinical diagnosis and intervention.
Assuntos
Afasia , Córtex Auditivo , Disfonia , Gagueira , Humanos , Disartria , Funções Verossimilhança , Distúrbios da FalaRESUMO
BACKGROUND: Technology to restore the ability to communicate in paralyzed persons who cannot speak has the potential to improve autonomy and quality of life. An approach that decodes words and sentences directly from the cerebral cortical activity of such patients may represent an advancement over existing methods for assisted communication. METHODS: We implanted a subdural, high-density, multielectrode array over the area of the sensorimotor cortex that controls speech in a person with anarthria (the loss of the ability to articulate speech) and spastic quadriparesis caused by a brain-stem stroke. Over the course of 48 sessions, we recorded 22 hours of cortical activity while the participant attempted to say individual words from a vocabulary set of 50 words. We used deep-learning algorithms to create computational models for the detection and classification of words from patterns in the recorded cortical activity. We applied these computational models, as well as a natural-language model that yielded next-word probabilities given the preceding words in a sequence, to decode full sentences as the participant attempted to say them. RESULTS: We decoded sentences from the participant's cortical activity in real time at a median rate of 15.2 words per minute, with a median word error rate of 25.6%. In post hoc analyses, we detected 98% of the attempts by the participant to produce individual words, and we classified words with 47.1% accuracy using cortical signals that were stable throughout the 81-week study period. CONCLUSIONS: In a person with anarthria and spastic quadriparesis caused by a brain-stem stroke, words and sentences were decoded directly from cortical activity during attempted speech with the use of deep-learning models and a natural-language model. (Funded by Facebook and others; ClinicalTrials.gov number, NCT03698149.).
Assuntos
Infartos do Tronco Encefálico/complicações , Interfaces Cérebro-Computador , Aprendizado Profundo , Disartria/reabilitação , Próteses Neurais , Fala , Adulto , Disartria/etiologia , Eletrocorticografia , Eletrodos Implantados , Humanos , Masculino , Processamento de Linguagem Natural , Quadriplegia/etiologia , Córtex Sensório-Motor/fisiologiaRESUMO
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.
Assuntos
Transtornos de Deglutição , Disartria , Doenças Mitocondriais , Humanos , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/fisiopatologia , Disartria/etiologia , Disartria/fisiopatologia , Masculino , Feminino , Doenças Mitocondriais/complicações , Doenças Mitocondriais/fisiopatologia , Adulto , Pessoa de Meia-Idade , Estudos Retrospectivos , Estudos Transversais , Idoso , Índice de Gravidade de Doença , Prevalência , Deglutição , Adulto Jovem , FenótipoRESUMO
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.
Assuntos
Disartria , Aplicativos Móveis , Treinamento da Voz , Humanos , Projetos Piloto , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Disartria/terapia , Disartria/reabilitação , Adulto , Fonoterapia/métodos , Inteligibilidade da Fala/fisiologia , Resultado do TratamentoRESUMO
Dysarthria is a common manifestation across cerebellar ataxias leading to impairments in communication, reduced social connections, and decreased quality of life. While dysarthria symptoms may be present in other neurological conditions, ataxic dysarthria is a perceptually distinct motor speech disorder, with the most prominent characteristics being articulation and prosody abnormalities along with distorted vowels. We hypothesized that uncertainty of vowel predictions by an automatic speech recognition system can capture speech changes present in cerebellar ataxia. Speech of participants with ataxia (N=61) and healthy controls (N=25) was recorded during the "picture description" task. Additionally, participants' dysarthric speech and ataxia severity were assessed on a Brief Ataxia Rating Scale (BARS). Eight participants with ataxia had speech and BARS data at two timepoints. A neural network trained for phoneme prediction was applied to speech recordings. Average entropy of vowel tokens predictions (AVE) was computed for each participant's recording, together with mean pitch and intensity standard deviations (MPSD and MISD) in the vowel segments. AVE and MISD demonstrated associations with BARS speech score (Spearman's rho=0.45 and 0.51), and AVE demonstrated associations with BARS total (rho=0.39). In the longitudinal cohort, Wilcoxon pairwise signed rank test demonstrated an increase in BARS total and AVE, while BARS speech and acoustic measures did not significantly increase. Relationship of AVE to both BARS speech and BARS total, as well as the ability to capture disease progression even in absence of measured speech decline, indicates the potential of AVE as a digital biomarker for cerebellar ataxia.
Assuntos
Ataxia Cerebelar , Disartria , Humanos , Disartria/etiologia , Disartria/complicações , Ataxia Cerebelar/diagnóstico , Ataxia Cerebelar/complicações , Incerteza , Qualidade de Vida , Ataxia/diagnóstico , Ataxia/complicações , BiomarcadoresRESUMO
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.
Assuntos
Doença de Parkinson , Estimulação Transcraniana por Corrente Contínua , Humanos , Doença de Parkinson/terapia , Doença de Parkinson/complicações , Doença de Parkinson/fisiopatologia , Projetos Piloto , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Estudos Cross-Over , Disartria/etiologia , Disartria/terapia , Disartria/fisiopatologia , Fala/fisiologia , Resultado do Tratamento , Lobo Temporal/fisiopatologiaRESUMO
Hypokinetic dysarthria (HD) is a difficult-to-treat symptom affecting quality of life in patients with Parkinson's disease (PD). Levodopa may partially alleviate some symptoms of HD in PD, but the neural correlates of these effects are not fully understood. The aim of our study was to identify neural mechanisms by which levodopa affects articulation and prosody in patients with PD. Altogether 20 PD patients participated in a task fMRI study (overt sentence reading). Using a single dose of levodopa after an overnight withdrawal of dopaminergic medication, levodopa-induced BOLD signal changes within the articulatory pathway (in regions of interest; ROIs) were studied. We also correlated levodopa-induced BOLD signal changes with the changes in acoustic parameters of speech. We observed no significant changes in acoustic parameters due to acute levodopa administration. After levodopa administration as compared to the OFF dopaminergic condition, patients showed task-induced BOLD signal decreases in the left ventral thalamus (p = 0.0033). The changes in thalamic activation were associated with changes in pitch variation (R = 0.67, p = 0.006), while the changes in caudate nucleus activation were related to changes in the second formant variability which evaluates precise articulation (R = 0.70, p = 0.003). The results are in line with the notion that levodopa does not have a major impact on HD in PD, but it may induce neural changes within the basal ganglia circuitries that are related to changes in speech prosody and articulation.
Assuntos
Levodopa , Doença de Parkinson , Humanos , Levodopa/efeitos adversos , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico por imagem , Doença de Parkinson/tratamento farmacológico , Fala/fisiologia , Imageamento por Ressonância Magnética/métodos , Qualidade de Vida , Distúrbios da Fala/diagnóstico por imagem , Distúrbios da Fala/etiologia , Disartria/etiologia , Disartria/complicações , Antiparkinsonianos/efeitos adversosRESUMO
INTRODUCTION: The purpose of this study was to identify course of the corticobulbar tract and factors associated with the occurrence of facial paresis (FP) in lateral medullary infarction (LMI). METHODS: Patients diagnosed with LMI who were admitted to tertiary hospital were retrospectively investigated and divided into two groups based on the presence of FP. FP was defined as grade 2 or more by the House-Brackmann scale. Differences between the two groups were analyzed with respect to anatomical location of the lesions, demographic data (age, sex), risk factors (diabetes, hypertension, smoking, prior stroke, atrial fibrillation, and other cardiac risk factors for stroke), large vessel involvement on magnetic resonance angiography, other symptoms and signs (sensory symptoms, gait ataxia, limb ataxia, dizziness, Horner syndrome, hoarseness, dysphagia, dysarthria, nystagmus, nausea/vomiting, headache, neck pain, diplopia, and hiccup). RESULTS: Among 44 LMI patients, 15 patients (34%) had FP, and all of them had ipsilesional central-type FP. The FP group tended to involve upper (p < 0.0001) and relative ventral (p = 0.019) part of the lateral medulla. Horizontally large lesion was also related to the presence of FP (p = 0.044). Dysphagia (p = 0.001), dysarthria (p = 0.003), and hiccups (p = 0.034) were more likely to be accompanied by FP. Otherwise, there were no significant differences. CONCLUSION: The results of present study indicate that the corticobulbar fibers innervating the lower face decussate at the upper level of the medulla and ascend through the dorsolateral medulla, where the concentration of the fibers is densest near the nucleus ambiguus.
Assuntos
Transtornos de Deglutição , Paralisia Facial , Síndrome Medular Lateral , Acidente Vascular Cerebral , Humanos , Paralisia Facial/diagnóstico por imagem , Paralisia Facial/etiologia , Disartria/complicações , Disartria/patologia , Estudos Retrospectivos , Imageamento por Ressonância Magnética/efeitos adversos , Bulbo/diagnóstico por imagem , Infarto , Síndrome Medular Lateral/complicações , Síndrome Medular Lateral/diagnóstico por imagemRESUMO
BACKGROUND There is a lack of accurate models to predict amyotrophic lateral sclerosis (ALS) disease course and outcomes. As a result, risk assessment and counseling, the timing of interventions, and their stratification in clinical trials are difficult. This study aimed to evaluate the association between symptoms at presentation and mortality. MATERIAL AND METHODS A single veterans hospital reviewed the electronic records of 105 veterans with ALS who were periodically followed in our ALS clinic between 2010 and 2021. A survival decision tree (≤3 or >3 years) was generated based on the statistical median survival of our data. The variables known to influence survival when alive were compared to patients who died. RESULTS The (mean±SD) age at onset was 62±11 years, M/F ratio 101: 4, and 90% were non-Hispanic whites. The initial score for the Amyotrophic Lateral Sclerosis Functional Rating Scale-Revised (ALSFRS-R) was 31±8.3. Dysarthria and shortness of breath (SOB) were present on initial presentation in 52 (49.5%) and 32 (30.5%) patients, respectively. Deaths occurred in 80 (76.2%) patients during the study period. The main cause of death was respiratory disease (failure and pneumonia, n=43 53.75%). Patients survived for >3 years on initial presentation with normal respiration and speech, compared to ≤3 years of survival in patients with dysarthria and SOB, irrespective of age. CONCLUSIONS This study suggests that for veterans with ALS, the main predictors of shorter survival were respiratory status and speech disorder on initial presentation to the clinic.
Assuntos
Esclerose Lateral Amiotrófica , Veteranos , Humanos , Fala , Disartria , Progressão da DoençaRESUMO
BACKGROUND: Patent foramen ovale (PFO) is a known cause of ischemic stroke in young adults and combined oral contraceptives (COCs) are widely used by women of reproductive age. If young women with PFO are taking COCs, they may be subjected to a synergistic increase in the occurrence of stroke, though reports of ischemic stroke in this population are rare. We report a woman of reproductive age who was taking COC suffered repetitive ischemic strokes before a patent foramen ovale (PFO) was detected and closed, which may raise concerns in this field. CASE PRESENTATION: A 31-year-old woman presented to the emergency department with sudden-onset right upper- and lower-limb weakness and dysarthria for 1 hour, whose only risk factor of stroke was oral contraceptive use. On admission, she was alert with left gaze deviation, dysarthria, and right-sided hemiplegia. Her symptoms improved after receiving the revascularization therapy. About 24 hours later, her left eye experienced sudden painless vision loss. Then the PFO with a substantial right-to-left shunt was detected and then she received a trans-catheter closure of the defect. Over 3 months of follow-up, there were no signs of stroke, but visual loss persisted. CONCLUSION: This case of disabling stroke raises concerns regarding optimal management in primary and secondary prevention of stroke in young women on COCs with additional risk factors of stroke.
Assuntos
Forame Oval Patente , AVC Isquêmico , Acidente Vascular Cerebral , Adulto Jovem , Humanos , Feminino , Adulto , Forame Oval Patente/complicações , Forame Oval Patente/terapia , Disartria/complicações , Acidente Vascular Cerebral/etiologia , Fatores de Risco , AVC Isquêmico/complicações , Recidiva , Resultado do TratamentoRESUMO
OBJECTIVE: To identify and agree on what outcome domains should be measured in research and clinical practice when working with stroke survivors who have dysarthria. DESIGN: Delphi process, two rounds of an online survey followed by two online consensus meetings. SETTING: UK and Australia. PARTICIPANTS: Stroke survivors with experience of dysarthria, speech and language therapists/pathologists working in stroke and communication researchers. METHODS: Initial list of outcome domains generated from existing literature and with our patient and public involvement group to develop the survey. Participants completed two rounds of this survey to rate importance. Outcomes were identified as 'in', 'unclear' or 'out' from the second survey. All participants were invited to two consensus meetings to discuss these results followed by voting to identify critically important outcome domains for a future Core Outcome Set. All outcomes were voted on in the consensus meetings and included if 70% of meeting participants voted 'yes' for critically important. RESULTS: In total, 148 surveys were fully completed, and 28 participants attended the consensus meetings. A core outcome set for dysarthria after stroke should include four outcome domains: (a) intelligibility of speech, (b) ability to participate in conversations, (c) living well with dysarthria, (d) skills and knowledge of communication partners (where relevant). CONCLUSIONS: We describe the consensus of 'what' speech outcomes after stroke are valued by all stakeholders including those with lived experience. We share these findings to encourage the measurement of these domains in clinical practice and research and for future research to identify 'how' best to measure these outcomes.
Assuntos
Técnica Delphi , Disartria , Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Humanos , Disartria/etiologia , Disartria/reabilitação , Acidente Vascular Cerebral/complicações , Feminino , Masculino , Avaliação de Resultados em Cuidados de Saúde , Pessoa de Meia-Idade , Austrália , Consenso , Idoso , Inquéritos e Questionários , Reino UnidoRESUMO
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.
Assuntos
Disartria , Estudos de Viabilidade , Smartphone , Fonoterapia , Acidente Vascular Cerebral , Humanos , Disartria/terapia , Disartria/etiologia , Fonoterapia/métodos , Masculino , Feminino , Projetos Piloto , Pessoa de Meia-Idade , Acidente Vascular Cerebral/complicações , Idoso , Qualidade de Vida , Reabilitação do Acidente Vascular Cerebral/métodos , Aplicativos Móveis , Resultado do TratamentoRESUMO
BACKGROUND: Research is beginning to shed light on the practices employed by speech-language therapists (SLTs) for the management of acquired dysarthria. However, studies that explore SLTs' satisfaction with the effectiveness of current therapies and their interest in new treatment methods for this population have not been carried out. One potential new method is neuromuscular electrical stimulation (NMES): the pool of evidence for its use in rehabilitation is increasing, yet it has not been widely explored for use with dysarthria. AIM: To extend the understanding of acquired dysarthria management practices employed by SLTs across the globe and determine their satisfaction with current therapy options. To explore their interest in using NMES with this population. METHODS AND PROCEDURES: A cross-sectional international online survey was developed and disseminated to SLTs working with adults with acquired dysarthria through international professional associations. The survey collected information on demographic characteristics, dysarthria management practices, satisfaction with treatment effectiveness and interest in and knowledge of NMES. Survey responses were analysed using descriptive and inferential statistics, and quantitative content analysis. OUTCOMES AND RESULTS: A total of 211 SLTs (North America, 48.8%; Europe, 36%; Asia, 8.1%; Oceania, 5.7%; Africa, 0.9%; South America, 0.5%) completed the survey in full. Management practices varied considerably. There was a clear preference for informal assessments, mainly oral-motor examinations, focusing on body functions and structures. The majority of respondents rejected the use of non-speech oral motor exercises as a clinical or carryover exercise. Variable satisfaction with current speech subsystem treatments was noted; however, overall, there was a general dissatisfaction. Whilst a strong interest in the use of NMES for dysarthria was evidenced, it was noted that most SLTs lacked fundamental knowledge of NMES principles and application. CONCLUSION: SLTs' management practices and satisfaction with acquired dysarthria treatments differed substantially. Investigations of the potential use of NMES for dysarthria treatment are of interest. WHAT THIS PAPER ADDS: What is already known on the subject Recent country-specific surveys have explored speech-language therapists' (SLTs') assessment and intervention practices for acquired dysarthria. These studies indicate that although clinical management for this speech disorder mainly involves informal assessment tools and impairment-focused treatment, communication beyond the impairment, such as the activity and participation domains, is also frequently assessed and treated. What this paper adds to existing knowledge The majority of SLTs are dissatisfied with the overall benefits of current acquired dysarthria treatment. Phonatory, respiration and speech rate therapies are perceived to be more effective than prosody, articulation and resonance treatments. Despite a general lack of theoretical knowledge, most SLTs are interested in neuromuscular electrical stimulation treatment for acquired dysarthria. What are the potential or actual clinical implications of this work? New, evidence-based treatments are needed for SLTs to be confident in the effectiveness of their acquired dysarthria treatment.
Assuntos
Disartria , Terapia da Linguagem , Adulto , Humanos , Disartria/terapia , Terapia da Linguagem/métodos , Fala , Estudos Transversais , Fonoterapia/métodos , InternacionalidadeRESUMO
BACKGROUND: In the past few years there has been a growing interest in the employment of verbal productions as digital biomarkers, namely objective, quantifiable behavioural data that can be collected and measured by means of digital devices, allowing for a low-cost pathology detection, classification and monitoring. Numerous research papers have been published on the automatic detection of subtle verbal alteration, starting from written texts, raw speech recordings and transcripts, and such linguistic analysis has been singled out as a cost-effective method for diagnosing dementia and other medical conditions common among elderly patients (e.g., cognitive dysfunctions associated with metabolic disorders, dysarthria). AIMS: To provide a critical appraisal and synthesis of evidence concerning the application of natural language processing (NLP) techniques for clinical purposes in the geriatric population. In particular, we discuss the state of the art on studying language in healthy and pathological ageing, focusing on the latest research efforts to build non-intrusive language-based tools for the early identification of cognitive frailty due to dementia. We also discuss some challenges and open problems raised by this approach. METHODS & PROCEDURES: We performed a scoping review to examine emerging evidence about this novel domain. Potentially relevant studies published up to November 2021 were identified from the databases of MEDLINE, Cochrane and Web of Science. We also browsed the proceedings of leading international conferences (e.g., ACL, COLING, Interspeech, LREC) from 2017 to 2021, and checked the reference lists of relevant studies and reviews. MAIN CONTRIBUTION: The paper provides an introductory, but complete, overview of the application of NLP techniques for studying language disruption due to dementia. We also suggest that this technique can be fruitfully applied to other medical conditions (e.g., cognitive dysfunctions associated with dysarthria, cerebrovascular disease and mood disorders). CONCLUSIONS & IMPLICATIONS: Despite several critical points need to be addressed by the scientific community, a growing body of empirical evidence shows that NLP techniques can represent a promising tool for studying language changes in pathological aging, with a high potential to lead a significant shift in clinical practice. WHAT THIS PAPER ADDS: What is already known on this subject Speech and languages abilities change due to non-pathological neurocognitive ageing and neurodegenerative processes. These subtle verbal modifications can be measured through NLP techniques and used as biomarkers for screening/diagnostic purposes in the geriatric population (i.e., digital linguistic biomarkers-DLBs). What this paper adds to existing knowledge The review shows that DLBs can represent a promising clinical tool, with a high potential to spark a major shift to dementia assessment in the elderly. Some challenges and open problems are also discussed. What are the potential or actual clinical implications of this work? This methodological review represents a starting point for clinicians approaching the DLB research field for studying language in healthy and pathological ageing. It summarizes the state of the art and future research directions of this novel approach.
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Demência , Processamento de Linguagem Natural , Idoso , Humanos , Disartria , Demência/diagnóstico , Envelhecimento , BiomarcadoresRESUMO
Amyotrophic lateral sclerosis (ALS) commonly results in the inability to produce natural speech, making speech-generating devices (SGDs) important. Historically, synthetic voices generated by SGDs were neither unique, nor age- or dialect-appropriate, which depersonalized SGD use. Voices generated by SGDs can now be customized via voice banking and should ideally sound uniquely like the individual's natural speech, be intelligible, and elicit positive reactions from communication partners. This large-scale 2 x 2 mixed between- and within-participants design examined perceptions of 831 adult listeners regarding custom synthetic voices created for two individuals diagnosed with ALS via two synthesis systems in common clinical use (waveform concatenation and statistical parametric synthesis). The study explored relationships among synthesis system, dysarthria severity, synthetic speech intelligibility, naturalness, and preferences, and also provided a preliminary examination of attitudes regarding the custom synthetic voices. Synthetic voices generated via statistical parametric synthesis trained on deep neural networks were more intelligible, natural, and preferred than voices produced via waveform concatenation, and were associated with more positive attitudes. The custom synthetic voice created from moderately dysarthric speech was more intelligible than the voice created from mildly dysarthric speech. Clinical implications and factors that may have contributed to the relative intelligibilities are discussed.
Assuntos
Esclerose Lateral Amiotrófica , Auxiliares de Comunicação para Pessoas com Deficiência , Transtornos da Comunicação , Voz , Adulto , Humanos , Transtornos da Comunicação/complicações , Disartria , Inteligibilidade da FalaRESUMO
Paroxysmal dysarthria ataxia syndrome presents with recurrent, brief, stereotyped events of dysarthria, limb clumsiness, unsteady gait and vertigo or dizziness that can occur in association with lesions in the midbrain. We describe a case of a woman presenting with paroxysmal dysarthria and ataxia secondary to a midbrain lesion, treated successfully with carbamazepine.