Biomedical signals and machine learning in amyotrophic lateral sclerosis: a systematic review.

INTRODUCTION: The use of machine learning (ML) techniques in healthcare encompasses an emerging concept that envisages vast contributions to the tackling of rare diseases. In this scenario, amyotrophic lateral sclerosis (ALS) involves complexities that are yet not demystified. In ALS, the biomedical signals present themselves as potential biomarkers that, when used in tandem with smart algorithms, can be useful to applications within the context of the disease. METHODS: This Systematic Literature Review (SLR) consists of searching for and investigating primary studies that use ML techniques and biomedical signals related to ALS. Following the definition and execution of the SLR protocol, 18 articles met the inclusion, exclusion, and quality assessment criteria, and answered the SLR research questions. DISCUSSIONS: Based on the results, we identified three classes of ML applications combined with biomedical signals in the context of ALS: diagnosis (72.22%), communication (22.22%), and survival prediction (5.56%). CONCLUSIONS: Distinct algorithmic models and biomedical signals have been reported and present promising approaches, regardless of their classes. In summary, this SLR provides an overview of the primary studies analyzed as well as directions for the construction and evolution of technology-based research within the scope of ALS.

Respiratory muscle training in children and adults with neuromuscular disease.

BACKGROUND: Neuromuscular diseases (NMDs) are a heterogeneous group of diseases affecting the anterior horn cell of spinal cord, neuromuscular junction, peripheral nerves and muscles. NMDs cause physical disability usually due to progressive loss of strength in limb muscles, and some NMDs also cause respiratory muscle weakness. Respiratory muscle training (RMT) might be expected to improve respiratory muscle weakness; however, the effects of RMT are still uncertain. This systematic review will synthesize the available trial evidence on the effectiveness and safety of RMT in people with NMD, to inform clinical practice. OBJECTIVES: To assess the effects of respiratory muscle training (RMT) for neuromuscular disease (NMD) in adults and children, in comparison to sham training, no training, standard treatment, breathing exercises, or other intensities or types of RMT. SEARCH METHODS: On 19 November 2018, we searched the Cochrane Neuromuscular Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, and Embase. On 23 December 2018, we searched the US National Institutes for Health Clinical Trials Registry (ClinicalTrials.gov), the World Health Organization International Clinical Trials Registry Platform, and reference lists of the included studies. SELECTION CRITERIA: We included randomized controlled trials (RCTs) and quasi-RCTs, including cross-over trials, of RMT in adults and children with a diagnosis of NMD of any degree of severity, who were living in the community, and who did not need mechanical ventilation. We compared trials of RMT (inspiratory muscle training (IMT) or expiratory muscle training (EMT), or both), with sham training, no training, standard treatment, different intensities of RMT, different types of RMT, or breathing exercises. DATA COLLECTION AND ANALYSIS: We followed standard Cochrane methodological procedures. MAIN RESULTS: We included 11 studies involving 250 randomized participants with NMDs: three trials (N = 88) in people with amyotrophic lateral sclerosis (ALS; motor neuron disease), six trials (N = 112) in Duchenne muscular dystrophy (DMD), one trial (N = 23) in people with Becker muscular dystrophy (BMD) or limb-girdle muscular dystrophy, and one trial (N = 27) in people with myasthenia gravis.Nine of the trials were at high risk of bias in at least one domain and many reported insufficient information for accurate assessment of the risk of bias. Populations, interventions, control interventions, and outcome measures were often different, which largely ruled out meta-analysis. All included studies assessed lung capacity, our primary outcome, but four did not provide data for analysis (1 in people with ALS and three cross-over studies in DMD). None provided long-term data (over a year) and only one trial, in ALS, provided information on adverse events. Unscheduled hospitalisations for chest infection or acute exacerbation of chronic respiratory failure were not reported and physical function and quality of life were reported in one (ALS) trial.Amyotrophic lateral sclerosis (ALS)Three trials compared RMT versus sham training in ALS. Short-term (8 weeks) effects of RMT on lung capacity in ALS showed no clear difference in the change of the per cent predicted forced vital capacity (FVC%) between EMT and sham EMT groups (mean difference (MD) 0.70, 95% confidence interval (CI) -8.48 to 9.88; N = 46; low-certainty evidence). The mean difference (MD) in FVC% after four months' treatment was 10.86% in favour of IMT (95% CI -4.25 to 25.97; 1 trial, N = 24; low-certainty evidence), which is larger than the minimal clinically important difference (MCID, as estimated in people with idiopathic pulmonary fibrosis). There was no clear difference between IMT and sham IMT groups, measured on the Amyotrophic Lateral Sclerosis Functional Rating Scale (ALFRS; range of possible scores 0 = best to 40 = worst) (MD 0.85, 95% CI -2.16 to 3.85; 1 trial, N = 24; low-certainty evidence) or quality of life, measured on the EuroQol-5D (0 = worst to 100 = best) (MD 0.77, 95% CI -17.09 to 18.62; 1 trial, N = 24; low-certainty evidence) over the medium term (4 months). One trial report stated that the IMT protocol had no adverse effect (very low-certainty evidence).Duchenne muscular dystrophy (DMD)Two DMD trials compared RMT versus sham training in young males with DMD. In one study, the mean post-intervention (6-week) total lung capacity (TLC) favoured RMT (MD 0.45 L, 95% CI -0.24 to 1.14; 1 trial, N = 16; low-certainty evidence). In the other trial there was no clear difference in post-intervention (18 days) FVC between RMT and sham RMT (MD 0.16 L, 95% CI -0.31 to 0.63; 1 trial, N = 20; low-certainty evidence). One RCT and three cross-over trials compared a form of RMT with no training in males with DMD; the cross-over trials did not provide suitable data. Post-intervention (6-month) values showed no clear difference between the RMT and no training groups in per cent predicted vital capacity (VC%) (MD 3.50, 95% CI -14.35 to 21.35; 1 trial, N = 30; low-certainty evidence).Becker or limb-girdle muscular dystrophyOne RCT (N = 21) compared 12 weeks of IMT with breathing exercises in people with Becker or limb-girdle muscular dystrophy. The evidence was of very low certainty and conclusions could not be drawn.Myasthenia gravisIn myasthenia gravis, there may be no clear difference between RMT and breathing exercises on measures of lung capacity, in the short term (TLC MD -0.20 L, 95% CI -1.07 to 0.67; 1 trial, N = 27; low-certainty evidence). Effects of RMT on quality of life are uncertain (1 trial; N = 27).Some trials reported effects of RMT on inspiratory and/or expiratory muscle strength; this evidence was also of low or very low certainty. AUTHORS' CONCLUSIONS: RMT may improve lung capacity and respiratory muscle strength in some NMDs. In ALS there may not be any clinically meaningful effect of RMT on physical functioning or quality of life and it is uncertain whether it causes adverse effects. Due to clinical heterogeneity between the trials and the small number of participants included in the analysis, together with the risk of bias, these results must be interpreted very cautiously.

Egos has a reduced capacity to predicts GBS prognosis in Northeast Brazil.

OBJECTIVE: The Erasmus Guillain Barre Outcome Score (EGOS) is a prognostic model that predicts the chance of being able to walk independently at 6 months after Guillain Barré syndrome (GBS). This study was conducted aiming to determine the validity of EGOS in a Brazilian population. MATERIAL AND METHODS: Data collected from GBS patients in Rio Grande do Norte, Brazil, were used to determine the validity of EGOS. GBS disability score was assessed in the second week of disease and at 6 months. RESULTS: A total of 206 subjects were studied. The Brazilian patients were younger, with a more severe clinical presentation, with higher percentage of cranial nerve involvement and upper respiratory infection. There was no difference relative to sex or presence of anti-gangliosides antibodies. The demyelinating variant was more common (73.9%). However, only 24% of the Brazilians with EGOS 5.5-7 were not able to walk after 6 months, compared to 52% to European Group. Nine patients (3.8%) presented nodopathies, of these four had an EGOS >5, but only one of the latter group was unable to walk after 6 months of GBS. CONCLUSIONS: Erasmus Guillain Barre Outcome Score was not a good predictor for the ability to walk after 6 months of GBS in Rio Grande do Norte, Brazil. Differences could be that the Brazilian GBS were younger, or alternatively, it could be due to a different infection profile or in the incidence of nodopathies.

Effects of Air Stacking Maneuver on Cough Peak Flow and Chest Wall Compartmental Volumes of Subjects With Amyotrophic Lateral Sclerosis.

OBJECTIVES: To assess the acute effects of air stacking on cough peak flow (CPF) and chest wall compartmental volumes of persons with amyotrophic lateral sclerosis (ALS) versus healthy subjects positioned at 45° body inclination. DESIGN: Cross-sectional study with a matched-pair design. SETTING: University hospital. PARTICIPANTS: Persons (N=24) with ALS (n=12) and age-matched healthy subjects (n=12). MAIN OUTCOMES MEASURES: CPF, chest wall compartmental inspiratory capacity, chest wall vital capacity, chest wall tidal volume and operational volumes, breathing pattern, and percentage of contribution of the compartments to the inspired volume were measured by optoelectronic plethysmography. RESULTS: Compared with healthy subjects, significantly lower CPF (P=.007), chest wall compartmental inspiratory capacity (P<.001), chest wall vital capacity (P<.001), and chest wall tidal volume (P<.001) were found in subjects with ALS. Immediately after air stacking, CPF (P<.001) and chest wall compartmental inspiratory capacity (P<.001) significantly increased in both groups, with values returning to basal only in healthy subjects. After air stacking, the abdominal compartment (P=.004) was determined to be responsible for the inspired volume in subjects with ALS. Significantly higher chest wall vital capacity (P=.05) was observed in subjects with ALS 5 minutes after air stacking, with the rib cage compartment (P=.049) being responsible for volume change. No differences were found in chest wall vital capacity and compartmental volumes of healthy subjects. Chest wall tidal volume (P<.001) significantly increased during the protocol in the healthy subjects, mainly because of end-inspiratory (P<.001) and abdominal volumes (P=.008). No significant differences were observed in percentage of contribution of the compartments to the inspired volume and end-expiratory volume of both groups. No significant differences were found in chest wall tidal volume, operational volume, and breathing pattern in persons with ALS. CONCLUSIONS: Air stacking is effective in increasing CPF, chest wall compartmental inspiratory capacity, and chest wall vital capacity of persons with ALS with no hyperinflation. Differences in compartmental volume contributions are probably because of lung and chest wall physiological changes.

The sural-sparing pattern in clinical variants and electrophysiological subtypes of Guillain-Barré syndrome.

BACKGROUND: Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. OBJECTIVE: This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. METHODS: This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. RESULTS: The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. CONCLUSION: The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency.

ANTECEDENTES: A síndrome de Guillain-Barré (GBS) é a causa mais comum de paralisia flácida aguda em todo o mundo e pode ser classificada em subtipos eletrofisiológicos e variantes clínicas. OBJETIVO: Este estudo teve como objetivo comparar a frequência do padrão de preservação do sural (SSP) em subtipos e variantes de GBS. MéTODOS: É um estudo de coorte retrospectivo que analisou dados clínicos e eletrofisiológicos de 171 pacientes com GBS internados em hospitais públicos e privados de Natal, Rio Grande do Norte, Brasil, entre 1994 e 2018. Todos os casos foram acompanhados pelo mesmo neurologista em centro de referência em neurologia. Os pacientes foram classificados de acordo com os subtipos eletrofisiológicos e variantes clínicas e a frequência do SSP foi comparada em ambas as categorias. O teste exato de Fisher e a correção de Bonferroni foram utilizados para análise estatística. RESULTADOS: O SSP esteve presente em 53% (57 de 107) dos pacientes com polirradiculoneuropatia desmielinizante inflamatória aguda (PDIA), em 8% (4 de 48) dos pacientes com subtipos axonais e em 31% (5 de 16) dos casos não definidos. A frequência do SSP no AIDP foi significativamente maior do que nos subtipos axonais (p < 0,0001); o valor manteve-se elevado após exames eletrofisiológicos seriados. Apenas o subtipo paraparético não apresentou SSP. CONCLUSãO: O SSP pode estar presente na PDIA e nos subtipos axonais, incluindo a neuropatia axonal motora aguda, mas está significativamente mais presente na PDIA. Além disso, as variantes clínicas refletem um processo patológico específico e estão correlacionadas ao seu subtipo eletrofisiológico típico, afetando a frequência do SSP.

Assessment of the Clinical and Functional Health Status of Patients with Amyotrophic Lateral Sclerosis during the COVID-19 Pandemic in Brazil Using Telemedicine.

This study aimed to monitor the clinical and functional progression of patients with amyotrophic lateral sclerosis (ALS) and adjust ventilatory support during the COVID-19 pandemic in Brazil using telemedicine. This longitudinal case series included five evaluations from January 2019 to June 2021. The first and second assessments were performed in person and consisted of pulmonary function, respiratory muscle strength, functionality (ALS Functional Rating Scale-Revised [ALSFRS-R]) and disease staging (King's College criteria). The use of non-invasive ventilation (NIV), ALSFRS-R, and disease staging were assessed in the third, fourth, and fifth assessments during the COVID-19 pandemic, using telemedicine. The rate of functional decline was calculated by the difference in the total score of ALSFRS-R between evaluations. A cutoff of 0.77 in the ALSFRS-R was used to characterize the speed of functional decline. Eleven patients (mean age of 51 years, eight males) were assessed. The total score of the ALSFRS-R (p < 0.01) and its motor domain (p < 0.01) reduced significantly during the pandemic. NIV prescription increased from 54.4% to 83.3%. Telemedicine helped with the clinical and functional follow-up of patients with ALS.

Persistent inflammation and nutritional status in Duchenne muscular dystrophy.

BACKGROUND & AIMS: Duchenne muscular dystrophy (DMD) involves muscle fragility, sarcolemma instability, and chronic inflammation. This study aims to identify the inflammatory profile of DMD patients and evaluate associations between clinical and nutritional variables. METHODS: We performed a cross-sectional study nested in a cohort to obtain sociodemographics, illness time, use of medications, and supplement data through interviews and the patient's medical records. Then, we assessed the relationships between illness time, cytokine levels, and nutritional status. RESULTS: Forty-four male participants, aged 4.3-24.2 years, were evaluated. Concerning nutritional status, 18 participants were eutrophic. The fat mass increased and the lean mass decreased from the beginning of the first signs of DMD. Cytokines levels in DMD patients, even under corticosteroids therapy, are higher than values described in the literature on healthy subjects. The regression models demonstrated that illness time and BMI/A z-scores are associated with higher values of interleukin-6. CONCLUSIONS: A persistent inflammatory profile was observed in the patients evaluated. The data suggest that maintaining adequate nutritional status and body composition is important for determining the inflammation presented by individuals with DMD.

Telehealth for amyotrophic lateral sclerosis in a multidisciplinary service in a Brazilian reference center.

BACKGROUND: Telehealth has been used in the treatment of different diseases, and it has been shown to provide benefits for patients with amyotrophic lateral sclerosis (ALS). Due to the social distancing measures put into effect during the coronavirus disease 2019 (COVID-19) pandemic, there was an urgent need for telehealth to ensure the provision of healthcare. OBJECTIVE: To evaluate the feasibility of telehealth for the provision of multidisciplinary ALS care, and to assess its acceptability among patients and caregivers. METHODS: We conducted a retrospective cohort study in which multidisciplinary evaluations were performed using the Teleconsulta platform. The patients included had ALS and at least one in-person clinical evaluation. The patients and the caregivers answered satisfaction questionnaires. RESULTS: The sample was composed of 46 patients, 32 male and 14 female subjects. The average distance from their residences to the reference services was of 115 km. Respiratory adjustment was the most addressed topic. CONCLUSION: The strategy is viable and well accepted in terms of satisfaction. It was even more positive for patients in advanced stages of the disease or for those living far from the referral center.

ANTECEDENTES: A telessaúde tem sido utilizada no tratamento de diferentes doenças, e demonstrou-se que ela traz benefícios para pacientes com esclerose lateral amiotrófica (ELA). Devido às medidas de distanciamento social postas em prática durante a pandemia de doença do coronavírus 2019 (coronavirus disease 2019, COVID-19, em inglês), houve uma necessidade urgente de se usar a telessaúde para garantir a provisão dos cuidados de saúde. OBJETIVO: Avaliar a viabilidade da telessaúde para a prestação de cuidados multidisciplinares na ELA, e verificar a sua aceitabilidade entre os pacientes e os cuidadores. MéTODOS: Realizou-se um estudo de coorte retrospectivo, com avaliações multidisciplinares realizadas por meio da plataforma Teleconsulta. Os pacientes incluídos apresentavam ELA, e já haviam passado por pelo menos uma avaliação clínica presencial. Os pacientes e os cuidadores responderam a questionários de satisfação. RESULTADOS: A amostra continha 46 pacientes, 32 do sexo masculino e 14 do sexo feminino. A distância média de suas residências ao serviço de referência era de 115 km. O ajuste respiratório foi o tema mais abordado. CONCLUSãO: A estratégia é viável e bem-aceita em termos de satisfação. Foi ainda mais positiva para os pacientes com doença avançada ou residentes em uma cidade distante do centro de referência.

Neurological and vestibular findings in three cases of Multiple Sclerosis. / Achados neurológicos e vestibulares em três casos de Esclerose Múltipla.

Multiple sclerosis (MS) is a chronic and inflammatory autoimmune disease that affects the central nervous system (CNS). Dysfunction of body balance is also a common symptom and may be related to neurological injuries resulting from this disease. The aim of this study was to characterize the neurological and vestibular findings of three clinical cases diagnosed with MS. Data on the neurological evaluation and the magnetic resonance imaging of the skull were collected from the medical records. The patients responded to an initial interview and underwent clinical assessment of body balance and Video Head Impulse Test (vHIT). Vestibular symptoms and alterations were observed in at least one of the clinical tests of body balance and cerebellar function. In vHIT, changes were obtained in oculomotor tests, such as the presence of semi-spontaneous nystagmus and in parameters of the saccade test, and reduced gain in one or more vertical channels. Lesions were found on MRI of the skull in central areas that process vestibular information, such as the cerebellum and brainstem. The association of these findings suggests the presence of central vestibular dysfunction, compatible with the lesions detected in imaging exams.

A Esclerose Múltipla (EM) é uma doença autoimune crônica e inflamatória que afeta o sistema nervoso central (SNC). A disfunção do equilíbrio corporal também é um sintoma comum e pode estar relacionada às lesões neurológicas decorrentes desta doença. O objetivo deste estudo foi caracterizar os achados neurológicos e vestibulares de três casos clínicos com diagnóstico de EM. Dados sobre a avaliação neurológica e do exame de ressonância magnética de crânio foram coletados dos prontuários. Os pacientes responderam a uma entrevista inicial e foram submetidos à avaliação clínica do equilíbrio corporal e ao Video Head Impulse Test (vHIT). Observou-se presença de sintomas vestibulares e alterações em pelo menos uma das provas clínicas do equilíbrio corporal e função cerebelar. No vHIT, obteve-se alterações em testes oculomotores, como presença de nistagmo semi-espontâneo e em parâmetros do teste sacádico, e ganho reduzido em um ou mais canais verticais. Foram verificadas lesões, na ressonância magnética de crânio, em áreas centrais que processam as informações vestibulares, como cerebelo e tronco encefálico. A associação destes achados sugere a presença de disfunção vestibular central, compatível com as lesões detectadas nos exames de imagem.

Noninvasive mechanical ventilation assistance in amyotrophic lateral sclerosis: a systematic review.

BACKGROUND: Respiratory failure is the most common cause of death in patients with amyotrophic lateral sclerosis (ALS), and morbidity is related to poor quality of life (QOL). Non-invasive ventilation (NIV) may be associated with prolonged survival and QOL in patients with ALS. OBJECTIVES: To assess whether NIV is effective and safe for patients with ALS in terms of survival and QOL, alerting the health system. DESIGN AND SETTING: Systematic review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses reporting standards using population, intervention, comparison, and outcome strategies. METHODS: The Cochrane Library, CENTRAL, MEDLINE, LILACS, EMBASE, and CRD databases were searched based on the eligibility criteria for all types of studies on NIV use in patients with ALS published up to January 2022. Data were extracted from the included studies, and the findings were presented using a narrative synthesis. RESULTS: Of the 120 papers identified, only 14 were related to systematic reviews. After thorough reading, only one meta-analysis was considered eligible. In the second stage, 248 studies were included; however, only one systematic review was included. The results demonstrated that NIV provided relief from the symptoms of chronic hypoventilation, increased survival, and improved QOL compared to standard care. These results varied according to clinical phenotype. CONCLUSIONS: NIV in patients with ALS improves the outcome and can delay the indication for tracheostomy, reducing expenditure on hospitalization and occupancy of intensive care unit beds. SYSTEMATIC REVIEW REGISTRATION: PROSPERO database: CRD42021279910 - https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=279910.

Digital health solution for monitoring and surveillance of Amyotrophic Lateral Sclerosis in Brazil.

Amyotrophic Lateral Sclerosis (ALS) is a complex and rare neurodegenerative disease given its heterogeneity. Despite being known for many years, few countries have accurate information about the characteristics of people diagnosed with ALS, such as data regarding diagnosis and clinical features of the disease. In Brazil, the lack of information about ALS limits data for the research progress and public policy development that benefits people affected by this health condition. In this context, this article aims to show a digital health solution development and application for research, intervention, and strengthening of the response to ALS in the Brazilian Health System. The proposed solution is composed of two platforms: the Brazilian National ALS Registry, responsible for the data collection in a structured way from ALS patients all over Brazil; and the Brazilian National ALS Observatory, responsible for processing the data collected in the National Registry and for providing a monitoring room with indicators on people diagnosed with ALS in Brazil. The development of this solution was supported by the Brazilian Ministry of Health (MoH) and was carried out by a multidisciplinary team with expertise in ALS. This solution represents a tool with great potential for strengthening public policies and stands out for being the only public database on the disease, besides containing innovations that allow data collection by health professionals and/or patients. By using both platforms, it is believed that it will be possible to understand the demographic and epidemiological data of ALS in Brazil, since the data will be able to be analyzed by care teams and also by public health managers, both in the individual and collective monitoring of people living with ALS in Brazil.

Digital Alternative Communication for Individuals with Amyotrophic Lateral Sclerosis: What We Have.

Amyotrophic Lateral Sclerosis is a disease that compromises the motor system and the functional abilities of the person in an irreversible way, causing the progressive loss of the ability to communicate. Tools based on Augmentative and Alternative Communication are essential for promoting autonomy and improving communication, life quality, and survival. This Systematic Literature Review aimed to provide evidence on eye-image-based Human-Computer Interaction approaches for the Augmentative and Alternative Communication of people with Amyotrophic Lateral Sclerosis. The Systematic Literature Review was conducted and guided following a protocol consisting of search questions, inclusion and exclusion criteria, and quality assessment, to select primary studies published between 2010 and 2021 in six repositories: Science Direct, Web of Science, Springer, IEEE Xplore, ACM Digital Library, and PubMed. After the screening, 25 primary studies were evaluated. These studies showcased four low-cost, non-invasive Human-Computer Interaction strategies employed for Augmentative and Alternative Communication in people with Amyotrophic Lateral Sclerosis. The strategies included Eye-Gaze, which featured in 36% of the studies; Eye-Blink and Eye-Tracking, each accounting for 28% of the approaches; and the Hybrid strategy, employed in 8% of the studies. For these approaches, several computational techniques were identified. For a better understanding, a workflow containing the development phases and the respective methods used by each strategy was generated. The results indicate the possibility and feasibility of developing Human-Computer Interaction resources based on eye images for Augmentative and Alternative Communication in a control group. The absence of experimental testing in people with Amyotrophic Lateral Sclerosis reiterates the challenges related to the scalability, efficiency, and usability of these technologies for people with the disease. Although challenges still exist, the findings represent important advances in the fields of health sciences and technology, promoting a promising future with possibilities for better life quality.

Coping strategies among amyotrophic lateral sclerosis (ALS) patients: an integrative review.

OBJECTIVE: To identify coping strategies used by amyotrophic lateral sclerosis (ALS) patients. METHODS: Integrative literature review using the Virtual Health Library, MEDLINE, and ScienceDirect databases. RESULTS: Eighteen studies were included. "Seeking social support" was the main coping strategy, while "Confrontive coping" and "Distancing" were the least mentioned. CONCLUSION: The coping strategies used by ALS patients do not seem to focus on emotions or stress-triggering problems. Age and gender did not modify the chosen strategy.

Effects of an optimised approach to home-based respiratory care in individuals with amyotrophic lateral sclerosis: a study protocol for a randomised controlled trial.

INTRODUCTION: This study aims to investigate the effects of an optimal home-based respiratory care protocol in individuals with amyotrophic lateral sclerosis (ALS). METHODS AND ANALYSIS: This is a randomised, blinded controlled trial involving patients diagnosed with ALS, both sexes, age between 18 and 80 years. Patients will be randomly allocated into the conventional respiratory care (CRC) group and the optimised respiratory care home-based (ORC) group. Primary outcomes will be peak cough flow, the number of exacerbations and ALS Functional Rating Scale Revised. Secondary outcomes will include chest wall volumes, maximal respiratory pressures, sniff nasal inspiratory pressure, nasal expiratory pressure and forced vital capacity (FVC), forced expiratory volume in the 1st second (FEV1) and FEV1/FVC. The CRC group will receive educational information about respiratory care at the clinic. The ORC group will receive conventional care and home-based care. The clinical status of all individuals will be monitored weekly through telephone calls. A 6-month intervention is planned, the outcomes will be assessed every 3 months and 3 and 6 months follow-up after final evaluation. The primary and secondary results will be described as average or median for continuous variables and absolute and relative frequencies for qualitative variables. Treatment effects or differences between the outcomes (baseline, 3 months and 6 months) of the study groups will be analysed using an analysis of variance. The level of significance will be set as p≤0.05. ETHICS AND DISSEMINATION: The research ethics committee approved the study. It is expected to evaluate respiratory function in patients with ALS in the short, medium and long terms with home-based care protocol applied. The disease's rapid progression is a limitation for performing a long-term clinical study. TRIAL REGISTRATION NUMBER: RBR-3z23ts; Pre-results.

Cytomegalovirus infection in Guillain-Barré syndrome: a retrospective study in Brazil.

BACKGROUND: Guillain-Barré syndrome (GBS) is currently the most common cause of acute flaccid paralysis worldwide. Risk factors for GBS include previous viral or bacterial infections or vaccination. Recently, an outbreak of Zika virus led to an outbreak of GBS in Latin America, mostly in Brazil, concomitant to continuous circulation of dengue virus serotypes. However, there is no study about cytomegalovirus (CMV) infection as a risk for GBS in Brazil. OBJECTIVES: In this study, we report a series of cases of GBS with the aim of determining the prevalence of CMV and the characteristics associated with the infection. METHODS: A cohort of 111 GBS cases diagnosed between 2011 and 2017 in Natal, northeastern Brazil, was studied. Presence of CMV IgM antibodies was determined by means of electrochemiluminescence. The analysis was performed considering CMV infection status and the clinical outcome. RESULTS: We found seroprevalence of 15.3% (n = 17) for CMV. CMV patients were younger (26 vs. 40; p = 0.016), with no apparent gastrointestinal (p = 0.762) or upper respiratory infections (p = 0.779) or sensory loss (p = 0.03). They presented more often with a classic GBS sensorimotor variant (p = 0.02) and with a demyelinating pattern in electrophysiological studies (p < 0.001). CONCLUSION: In Brazil, the clinical-epidemiological profile of GBS associated with CMV infection is similar to that described in other countries. Better understanding of the relationship between infectious processes and GBS is a key component of the research agenda and assistance strategy for global health initiatives relating to peripheral neuropathic conditions.

Tongue measurements and pharyngeal residue in amyotrophic lateral sclerosis.

Aim: We aimed to analyze the relationship between tongue measurements and vallecular residue in patients with amyotrophic lateral sclerosis (ALS). Materials & methods: Twenty-one patients with ALS were assessed for posterior maximum tongue isometric pressure (PMTIP) and posterior tongue isometric endurance (PTIE) by the Iowa Oral Performance Instrument; vallecular residue after 10 ml of moderately thickened consistency by Fiberoptic Endoscopic Evaluation of Swallowing; and tongue thickness (TT) by ultrasonography. Results: PMTIP, PTIE and TT were decreased compared with the reference values for healthy individuals and were not different between patients with and without vallecular residue. Conclusion: In ALS, PMTIP, PTIE and TT are not good predictors of vallecular residue in the tested volume and food consistency.

Lay abstract This study aimed to analyze the relationship of measurable characteristics of the tongue in patients with amyotrophic lateral sclerosis (ALS) and the accumulation of residue after swallowing in an area located just after the tongue. This accumulation of residue provides a sensation of bolus stuck in the throat and increases the risk of weight loss, choking and pulmonary complications. Twenty-one ALS patients were assessed for tongue pressure, tongue endurance, tongue thickness and residue after swallowing of the 10 ml of moderately thickened consistency. This study concluded that all tongue measurements were low in ALS patients with and without residue. Therefore, these tongue measurements are not good predictors of vallecular residue in the tested volume and food consistency.

Thoracoabdominal asynchrony and paradoxical motion in Duchenne muscular dystrophy and healthy subjects during cough: A case control study.

OBJECTIVE: To assess thoracoabdominal asynchrony (TAA) and inspiratory paradoxical motion at different positionings in subjects with Duchenne muscular dystrophy (DMD) versus healthy subjects during quiet spontaneous breathing (QB) and cough. METHODS: This is a case control study with a matched-pair design. We assessed 14 DMD subjects and 12 controls using optoelectronic plethysmography (OEP) during QB and spontaneous cough in 3 positions: supine, supine with headrest raised at 45°, and sitting with back support at 80°. The TAA was assessed using phase angle (θ) between upper (RCp) and lower rib cage (RCa) and abdomen (AB), as well as the percentage of inspiratory time the RCp (IPRCp ), RCa (IPRCa ), and AB (IPAB ) moved in opposite directions. RESULTS: During cough, DMD group showed higher RCp and RCa θ (p < .05), RCp and AB θ (p < .05) in supine and 45° positions, and higher RCp and Rca θ (p = .006) only in supine position compared with controls. Regarding the intragroup analysis, during cough, DMD group presented higher RCp and AB θ (p = .02) and RCa and AB θ (p = .002) in supine and higher RCa and AB θ (p = .002) in 45° position when compared to 80°. Receiver operating characteristic curve analyzes were able to discriminate TAA between controls and DMD in RCa supine position (area under the curve: 0.81, sensibility: 78.6% and specificity: 91.7%, p = .001). CONCLUSION: Subjects with DMD yields TAA with insufficient deflation of chest wall compartments and rib cage distortion during cough, by noninvasive assessment.

Effects of Positioning on Cough Peak Flow and Muscular Electromyographic Activation in Duchenne Muscular Dystrophy.

BACKGROUND: Advanced stages of Duchenne muscular dystrophy (DMD) result in muscle weakness and the inability to generate an effective cough. Several factors influence the effectiveness of cough in patients with DMD. The aim of this study was to assess whether differences in positioning affect cough peak flow (CPF) and muscular electromyographic activation in subjects with DMD compared with paired healthy subjects. METHODS: Optoelectronic plethysmography and surface electromyography were used to assess chest wall volumes, chest wall inspiratory capacity, CPF, breathing pattern, and electromyographic activity of sternocleidomastoid, scalene, rectus abdominis, and external oblique muscles during inspiratory and expiratory cough phases in the supine position, supine position with headrest raised at 45°, and sitting with back support at 80° in 12 subjects with DMD and 12 healthy subjects. RESULTS: Subjects with DMD had lower CPF (P < .01) in comparison to control subjects in all positions; the DMD group also exhibited lower CPF (P = .045) in the supine position versus 80°. Moreover, the relative volume contributions of the rib cage and abdominal compartments to tidal volume modified significantly with posture. The electromyographic activity during inspiratory and expiratory cough phases was lower in subjects with DMD compared to healthy subjects for all evaluated muscles (P < .05), but no significant differences were observed with posture change. CONCLUSIONS: In subjects with DMD, posture influenced CPF and the relative contribution of the rib cage and abdominal compartments to tidal volume. However, muscular electromyographic activation was not influenced by posture in subjects with DMD and healthy subjects.

Validity of bioelectrical impedance to estimate fat-free mass in boys with Duchenne muscular dystrophy.

The evaluation of fat-free mass (FFM) in patients with Duchenne muscular dystrophy (DMD) is useful to investigate disease progression and therapeutic efficacy. This study aimed to validate the Bioelectrical impedance (BIA) method compared with the dual-energy X-ray absorptiometry (DXA) for estimating the %FFM in boys with DMD. This is a cross-sectional study performed with children and adolescents diagnosed with DMD. Resistance and reactance were measured with a BIA analyzer, from which eight predictive equations estimated the %FFM. The %FFM was also determined by DXA and its used as a reference method. Pearson correlation test, coefficient of determination, the root-mean-square error, the interclass correlation coefficient, and linear regression analysis were performed between %FFM values obtained by BIA and DXA. The agreement between these values was verified with the Bland-Altman plot analysis. Forty-six boys aged from 5 to 20 years were enrolled in the study. All the equations showed a correlation between the %FFM estimated by BIA and determined by DXA (p < 0.05). The Bland-Altman method indicated that two equations have a significant bias (p < 0.05) and six equations showed no significant bias of %FFM (p > 0.05). However, one of them has high variation and wide limits of agreement. Five of eight %FFM predictive equations tested in DMD were accurate when compared with the DXA. It can be concluded that BIA is a validity method to evaluate patients with DMD.

Thoracoabdominal asynchrony and paradoxical motion in middle stage amyotrophic lateral sclerosis.

AIM: To assess thoracoabdominal asynchrony (TAA) and the presence of paradoxical motion in middle stage amyotrophic lateral sclerosis (ALS) and its relationships with chest wall tidal volume (VT,CW), breathing pattern and cough peak flow (CPF). METHODS: Phase angle (θ) between upper (RCp) and lower ribcage (RCa) and abdomen (AB), as well as percentage of inspiratory time for the lower ribcage (IPRCa) and abdomen (IPAB) moving in opposite directions were quantified using optoelectronic plethysmography in 12 ALS patients during quiet breathing and coughing. Paradoxical motion of the compartments was based on threshold values of θ and IP, obtained in twelve age and sex matched healthy persons. RESULTS: During quiet breathing, significantly higher RCa and AB θ (p < .05), IPRCa (p = 0.001) and IPAB (p < 0.05) were observed in ALS patients as compared to controls. In ALS patients, correlations between RCa and AB θ with forced vital capacity (FVC) (r=-0.773, p < 0.01), vital capacity (r=-0.663, p < 0.05) and inspiratory capacity (IC) (r=-0.754, p < 0.01), as well as between RCp and RCa θ with FVC (r=-0.608, p < 0.05) and CPF (r=-0.601, p < 0.05) were found. During coughing, correlations between RCp and AB θ with CPF (r=-0.590, p < 0.05), IC (r=-0.748, p < 0.01) and VT,CW (r=-0.608, p < 0.05), as well as between RCa and AB θ with CPF (r=-0.670, p < 0.05), IC (r=-0.713, p < 0.05) and peak expiratory flow (r=-0.727, p < 0.05) were also observed in ALS patients. ALS patients with paradoxical motion presented lower vital capacity and FVC%pred (p < 0.05) compared to those without paradoxical motion. CONCLUSIONS: Middle stage ALS patients exhibit TAA and paradoxical motion during quiet spontaneous breathing and coughing. In addition, diaphragmatic weakness (i.e. decrease in excursion of the RCa and AB compartments) was observed earlier in the lower ribcage rather than the abdominal compartment in this population.