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.

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

ABSTRACT 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.

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.

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.

Telehealth for amyotrophic lateral sclerosis in a multidisciplinary service in a Brazilian reference center / Telessaúde para esclerose lateral amiotrófica em um serviço multidisciplinar em um centro de referência brasileiro

Abstract 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.

Resumo 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.

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

RESUMO 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.

ABSTRACT 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.

Parâmetros e tipos de avaliação da disartria na esclerose lateral amiotrófica / Parameters and types of dysarthria assessment in amyotrophic lateral sclerosis

RESUMO Objetivo identificar estudos a respeito dos parâmetros e dos tipos de avaliação utilizados para avaliar a disartria na esclerose lateral amiotrófica (ELA). Estratégia de pesquisa estudo de revisão integrativa da literatura realizada nas bases de dados LILACS, SciELO, PubMed, Web of Science, CINAHL, Scopus e Cochrane, por meios dos descritores, em português e em inglês, "Avaliação AND Disartria AND Esclerose Lateral Amiotrófica". Critérios de seleção os critérios de inclusão foram: artigos que abordavam estudos sobre avaliação da disartria na ELA, nas línguas inglesa, espanhola e portuguesa, disponíveis na íntegra, no período de 2015 a 2022. Resultados do total de 38 estudos, apenas 3 usaram um único tipo de avaliação da disartria. A maior parte dos estudos utilizou mais de um tipo de avaliação variando de 2 a 4. Foram 3 os tipos de avaliação mais utilizados, com o intuito de avaliar o grau de inteligibilidade de fala: avaliação perceptivo-auditiva (31 estudos), avaliação acústica (18 estudos) e avaliação do movimento (27 estudos). Conclusão a avaliação da disartria na ELA é realizada por diferentes procedimentos e com vários parâmetros de análise, em especial pela avaliação perceptivo-auditiva e do movimento.

ABSTRACT Purpose to identify studies regarding the parameters and types of assessment used to evaluate dysarthria in amyotrophic lateral sclerosis (ALS). Research strategy an integrative literature review study was conducted on the LILACS, SciELO, PubMed, Web of Science, CINAHL, Scopus, and Cochrane databases using the descriptors "Assessment AND Dysarthria AND Amyotrophic Lateral Sclerosis" in both Portuguese and English. Selection criteria the inclusion criteria consisted of articles that addressed studies on dysarthria assessment in ALS, written in English, Spanish, and Portuguese, which should be available in full, and published from 2015 to 2022. Results: out of the total of 38 studies, only 3 used a single type of dysarthria assessment. Most studies employed more than one type of assessment, ranging from 2 to 4 types. Three assessment types were predominantly used to assess the degree of speech intelligibility: auditoryperceptual assessment (31 studies), acoustic assessment (18 studies), and movement assessment (27 studies). Conclusion dysarthria assessment in ALS is conducted through various procedures and with multiple analysis parameters, notably through auditory-perceptual and movement assessments.

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.

Video Head Impulse Test e doenças do sistema nervoso central: uma revisão integrativa / Video Head Impulse Test and central nervous system diseases: a integrative review

RESUMO Objetivos verificar a aplicabilidade do Video Head Impulse Test (vHIT) em doenças do sistema nervoso central (SNC), bem como os resultados encontrados e as doenças descritas. Estratégia de pesquisa revisão integrativa da literatura, em que foi realizada a busca em nove bases eletrônicas de dados, a partir da palavra-chave "video head impulse test". Critérios de seleção foram incluídos estudos que utilizaram o vHIT no diagnóstico de doenças do SNC e excluídos os estudos publicados antes de 2009 e estudos que realizaram outros procedimentos de investigação clínica, ou que aplicaram o teste no diagnóstico de doenças vestibulares periféricas. Resultados a amostra final foi composta por 18 estudos. Os resultados verificados mostraram que o reflexo vestíbulo-ocular (RVO) tem apresentado alterações na população investigada. Foram observados achados sugestivos de acometimento central, tais como ganho ou média de ganho do RVO nos canais semicirculares verticais, inferior aos laterais, ganho aumentado, correlação negativa do ganho com a gravidade da doença na ataxia espinocerebelar tipo 3, ponto de corte de 0,70 e assimetria de ganho menor de 20% para diferenciar neurite vestibular de derrame no ramo medial da artéria cerebelar posteroinferior, ganho normal com provas oculomotoras alteradas, presença de nistagmo espontâneo vertical, além de alterações no RVO com e sem otimização visual, na perseguição sacádica e no teste de desvio de inclinação. Conclusão verificou-se que o vHIT é aplicável quanto a avaliação do RVO de alta frequência em indivíduos com doenças do SNC, uma vez que trouxe evidências clínicas sobre alterações da função vestibular periférica e central nos diferentes quadros neurológicos.

ABSTRACT Purpose To verify the applicability of the Video Head Impulse Test (vHIT) in central nervous system (CNS) diseases, as well as the results found and the diseases described. Research strategy Integrative literature review, in which nine electronic databases were searched using the keyword "video head impulse test". Selection criteria Studies that used the vHIT in the diagnosis of CNS diseases were included, and studies published before 2009, studies that performed other clinical investigation procedures or that concerned the diagnosis of peripheral vestibular diseases were excluded. Results The final sample consisted of 18 studies. The verified results show that the vestibulo-ocular reflex (VOR) has shown alteration in this population. Suggestive findings of central involvement were observed, such as lower gain or average VOR in the vertical semicircular canals than in the lateral ones, increased gain, the negative correlation of gain with disease severity in Spinocerebellar Ataxia Type 3, cutoff point of 0.70, and gain asymmetry of less than 20% to differentiate vestibular neuritis from a stroke in the medial branch of the posteroinferior cerebellar artery, normal gain with altered oculomotor tests, presence of spontaneous vertical nystagmus, as well as alterations in the VOR with and without visual enhancement, in saccadic pursuit, and the tilt deviation test. Conclusion We found that the vHIT applies to the assessment of high-frequency VOR in individuals with CNS diseases since it provided clinical evidence of changes in peripheral and central vestibular function in different neurological conditions

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.

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.

Fiberoptic endoscopic evaluation of swallowing in patients with amyothrophic lateral sclerosis: an integrative literature review

ABSTRACT Purpose: to provide an integrative review of the current evidence on the fiberoptic endoscopic evaluation of swallowing performing procedures and analysis parameters in patients with amyotrophic lateral sclerosis. Methods: the study question followed the PECO strategy, and the search was performed in Medline, Cochrane, Scopus, Lilacs, Web of Science, and CINAHL databases, using keywords and specific free terms. Two authors screened eligible articles published from 2013 to October 2021 and extracted data on fiberoptic endoscopic evaluation of swallowing performing procedures and analysis parameters in patients with amyotrophic lateral sclerosis. Literature Review: of the 1,570 articles initially identified, 14 were eligible. There was no consensus on the consistency, volume, and type of food, utensils, sequence, and number of repetitions of each task during the exam. The analysis parameters, when described, were distinct. Although with different classification criteria, the observation of pharyngeal residue, laryngeal penetration, and laryngotracheal aspiration was included in all studies. Conclusion: fiberoptic endoscopic evaluation of swallowing performing procedures and analysis parameters are not standardized in studies with amyotrophic lateral sclerosis patients.

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.

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.

Dietary intake and zinc status in amyotrophic lateral sclerosis patients / Ingesta dietética y estado de zinc en pacientes con esclerosis lateral amiotrófica

Background: There is considerable evidence that abnormal zinc homeostasis is related to amyotrophic lateral sclerosis (ALS) pathogenesis, and malnutrition is an independent prognostic factor for worsened survival of ALS patients. Objective: To evaluate the dietary intake and zinc status in patients with ALS, treated in a specialized outpatient facility in Natal, Brazil. Methods: Twenty patients with ALS (case group) and 37 healthy subjects (control group) were included. Clinical and anthropometric assessments were carried out and dietary intake was obtained from two 24-hour recalls. Plasma and urinary zinc concentrations were determined by atomic absorption spectrophotometry. Results: Most of the participants were eutrophic. Mean energy, protein, carbohydrate and fat intake was significantly lower for the case group. There was greater prevalence of inadequate zinc intake in the case group (35%) compared to controls (27%). Mean plasma zinc was significantly lower in the case group than in controls (77.13 ± 22.21 vs 87.84 ± 17.44 μgZn/dl). Urinary zinc did not differ significantly between cases and controls. In the case group, plasma and urinary zinc concentrations were below reference values in 50.0% and 52.6% of patients, respectively. Conclusion: A large portion of patients with ALS exhibited poor dietary intake and changes in body zinc status. The zinc deficiency found in half of the ALS patients may contribute to a worsened prognosis and should be the target of nutritional intervention that aims to correct this deficiency (AU)

Introducción: hay pruebas considerables de que los cambios en la homeostasis del zinc están relacionados con la patogénesis de la esclerosis lateral amiotrófica (ELA) y que la malnutrición es un factor pronóstico capaz de reducir la supervivencia de los pacientes con ELA. Objetivo: evaluar la ingesta dietética y el estado de zinc en pacientes con ELA, tratados en un centro de atención ambulatoria especializado en Natal, Brasil. Métodos: se incluyeron 20 pacientes con ELA (grupo de casos) y 37 sujetos sanos (grupo control). Se realizaron evaluaciones clínicas y antropométricas y se obtuvo la ingesta dietética en dos recordatorios de 24 horas. Las concentraciones plasmáticas y urinarias de zinc se determinaron por espectrofotometría de absorción atómica. Resultados: la mayoría de los participantes fueron eutróficos. El consumo medio de energía, proteínas, carbohidratos y grasas fue significativamente menor en el grupo de casos. Hubo una mayor prevalencia de ingesta inadecuada de zinc en el grupo de casos (35%) en comparación con los controles (27%). El zinc plasmático medio fue significativamente menor en el grupo de casos que en los controles (77,13 ± 22,21 frente a 87,84 ± 17,44 μgZn/dl). El zinc urinario no difirió significativamente entre los casos y los controles. En el grupo de casos, las concentraciones de zinc plasmático y urinario fueron inferiores a los valores de referencia en el 50,0% y 52,6% de los pacientes, respectivamente. Conclusión: gran parte de los pacientes con ELA exhibieron una ingesta dietética pobre y modificación en el estatus de zinc corporal. La deficiencia de zinc encontrada en la mitad de los pacientes con ELA puede contribuir a un empeoramiento del pronóstico y debe ser el objetivo de la intervención nutricional que apunta a corregir esta deficiencia (AU)

Noninvasive assessment of respiratory muscle strength and activity in Myotonic dystrophy.

OBJECTIVE: To evaluate sensitivity/specificity of the maximum relaxation rate (MRR) of inspiratory muscles, amplitude of electromyographic activity of the sternocleidomastoid (SCM), scalene (SCA), parasternal (2ndIS) and rectus abdominis (RA) muscles; lung function and respiratory muscle strength in subjects with Myotonic dystrophy type 1 (DM1) compared with healthy subjects. DESIGN AND METHODS: Quasi-experimental observational study with control group. MRR of inspiratory muscles, lung function and amplitude of the electromyographic activity of SCM, SCA, 2ndIS and RA muscles during maximum inspiratory pressure (PImax), maximum expiratory pressure (PEmax) and sniff nasal inspiratory pressure (SNIP) tests were assessed in eighteen DM1 subjects and eleven healthy. RESULTS: MRR was lower in DM1 group compared to healthy (P = 0.001) and was considered sensitive and specific to identify disease in DM1 and discard it in controls, as well as SNIP% (P = 0.0026), PImax% (P = 0.0077) and PEmax% (P = 0.0002). Contraction time of SCM and SCA was higher in DM1 compared to controls, respectively, during PImax (P = 0.023 and P = 0.017) and SNIP (P = 0.015 and P = .0004). The DM1 group showed lower PImax (P = .0006), PEmax (P = 0.0002), SNIP (P = 0.0014), and higher electromyographic activity of the SCM (P = 0.002) and SCA (P = 0.004) at rest; of 2ndIS (P = 0.003) during PEmax and of SCM (P = 0.02) and SCA (P = 0.03) during SNIP test. CONCLUSIONS: MD1 subjects presented restrictive pattern, reduced respiratory muscle strength, muscular electrical activity and MRR when compared to higher compared to controls. In addition, the lower MRR found in MD1 subjects showed to be reliable to sensitivity and specificity in identifying the delayed relaxation of respiratory muscles.

Identification of peripheral arterial disease in diabetic patients and its association with quality of life, physical activity and body composition / Identificação de doença arterial obstrutiva periférica e sua associação com a qualidade de vida, a atividade física e a composição corporal em pacientes diabéticos

Diabetes mellitus (DM) is a risk factor for peripheral arterial disease (PAD). Neither the prevalence of PAD in type 2 (T2) DM nor its detrimental effects on quality of life (QoL) or physical activity (PA) have been well described in the Brazilian population. OBJECTIVES: To evaluate the prevalence of newly diagnosed PAD and its associations with QoL, PA and body composition in a sample of T2DM patients from a University Hospital. METHODS: Seventy-three (73) T2DM patients without previous diagnoses of major complications related to T2DM were enrolled. PAD was assessed using the ankle-brachial index (ABI); QoL was measured using a translated and validated SF-36 questionnaire; PA was measured using a modified Baecke questionnaire; and body composition was measured by segmental multi-frequency bioelectrical impedance. RESULTS: PAD prevalence was 13.7%, predominantly of mild severity (ABI between 0.8-0.9). The ABI results correlated with age (ρ=-0.26, P=0.03), DM duration (ρ=-0.28, P=0.02) and systolic and diastolic blood pressure (ρ=-0.33, P=0.007 and ρ=-0.28, P=0.02; respectively). Scores for the SF-36 physical component summary (PCS) were below the normal range, but no negative impact from PAD was identified by the PCS scores (normal-ABI 42.9±11.2 vs. PAD-ABI 38.12±11.07) or the Baecke PA results. Body composition analysis detected excessive body fat, especially in women, but there was no difference between groups. CONCLUSIONS: The prevalence of previously undiagnosed PAD in this population of T2DM patients was 13.7%, predominantly mild and asymptomatic forms, and was not yet associated with worsened QoL, PA levels or body composition variables...

O Diabetes Mellitus (DM) é fator de risco para a doença arterial obstrutiva periférica (DAOP). A prevalência de DAOP no DM tipo 2 (T2) e o prejuízo adicional causado por esta na qualidade de vida (QoL) e na atividade física (AF) não são bem descritos na população brasileira. OBJETIVOS: Avaliar a prevalência e a associação da DAOP recém-diagnosticada com a QoL, a AF e a composição corporal em pacientes T2DM provenientes de um hospital universitário. MÉTODOS: Setenta e três pacientes T2DM, sem complicações maiores relacionadas ao T2DM, foram incluídos. A DAOP foi avaliada pelo índice tornozelo-braquial (ITB); a QoL, pelo questionário traduzido e validado SF-36, e a AF, pelo questionário modificado de Baecke. A composição corporal foi avaliada pela impedância bioelétrica segmentar multifrequencial. RESULTADOS: A prevalência de DAOP foi 13,7%, predominantemente de severidade leve (ITB entre 0,8-0,9). O ITB correlacionou-se com a idade (ρ= -0,26; P= 0,03), a duração do DM (ρ=-0,28; P=0,02) e a pressão arterial sistólica e diastólica (ρ= -0,33; P= 0,007 e ρ= -0,28; P= 0,02; respectivamente). O sumário de saúde física (PCS) do questionário SF-36 estava abaixo da variação normal; contudo, nenhum impacto negativo da DAOP foi identificado no PCS (ABI normal = 42,9±11,2 vs. ABI-DAOP = 38,12±11,07) ou no nível de AF. A análise da composição corporal demonstrou gordura corporal excessiva, especialmente em mulheres; contudo, sem diferenças entre grupos. CONCLUSÃO: A prevalência de DAOP sem diagnóstico prévio nesta amostra de pacientes T2DM foi de 13,7%, predominantemente assintomática e leve, e ainda não associada com piores índices de QoL, nível de AF e composição corporal...

Protocolo diferenciado para terapia nutricional na esclerose lateral amiotrófica / Special protocol to nutritional therapy in amyotrophic lateral sclerosis

OBJETIVO: Discutir a terapia nutricional na Esclerose Lateral Amiotrófica (ELA), compartilhando um protocolo elaborado e utilizado em ambulatório especializado. MATERIAL E MÉTODOS: A fundamentação teórica desse artigo foi baseada na literatura científica publicada nas bases de dados PubMed e ISI Web of Science, utilizando os descritores "amyrotrophic lateral sclerosis" and "nutrition". RESULTADOS E DISCUSSÃO: Em síntese, a ELA é uma doença neurodegenerativa rara, com prognóstico desfavorável e tratamento paliativo. A desnutrição é bastante frequente nessa doença e aumenta o risco de morte dos pacientes. Assim, a terapia nutricional é imprescindível e deve estar integrada a uma assistência multidisciplinar. O monitoramento nutricional é recomendado pelo menos a cada três meses, incluindo avaliação nutricional e prescrição de dieta hipercalórica e hiperproteica, com adequada quantidade hídrica e de fibras. Em adição, os micronutrientes, sobretudo os antioxidantes, devem atingir a Ingestão Diária Recomendada. Modificações na consistência da dieta para tratar a disfagia são indicadas e a nutrição enteral deve ser desmistificada entre os pacientes e cuidadores. Esses pacientes são candidatos potenciais para uso da nutrição enteral, a qual deve ser indicada em casos de disfagia significativa ou prejuízo da função respiratória associada com baixa ingestão alimentar, índice de massa corporal menor que 18,5 ou 22,0 kg/m2 (para adultos e idosos, respectivamente) e/ou perda de peso acima de 10%. CONCLUSÃO: Sugere-se a padronização da terapia nutricional na ELA e a instituição de protocolo diferenciado na prática clínica. Com isso, a desnutrição pode ser evitada ou minimizada, contribuindo para melhor qualidade de vida e sobrevida desses pacientes

OBJECTIVE: To discuss the nutritional therapy in Amyotrophic Lateral Sclerosis (ALS), sharing a protocol performed and used by a specialized ambulatory care service. MATERIAL AND METHODS: The theoretical foundation of this paper was based on the literature published in both PubMed and ISI Web of Science, using "amyrotrophic lateral sclerosis" and "nutrition" as keywords. RESULTS AND DISCUSSION: In brief, ALS is a rare neurodegenerative disease with poor prognosis and palliative treatment. Malnutrition is very common in these patients and increases the risk of death among them. Nutritional therapy is essential and must be integrated into a multidisciplinary care. Nutritional monitoring is recommended at least once every three months. Nutritional assessment, high-calorie and highprotein diet with an adequate amount of water and fiber are recommended. In addition, micronutrients, especially antioxidants, must reach the Recommended Dietary Allowances. A diet texture modification for dysphagia is indicated and enteral nutrition should be demystified to patients and care givers during the follow-up care. ALS patients are potential candidates to use enteral nutrition, which should be indicated in cases of significant dysphagia or impaired respiratory function associated with low food intake, body mass index less than 18.5 or 22.0 kg/m² (for adults or elderlies, respectively), and/or body weight loss above 10%. CONCLUSION: We suggest the standardization of nutritional therapy in ALS and the establishment of a distinct protocol in clinical practice. Thus, malnutrition can be avoided or minimized, contributing to a better quality of life and survival of these patients

Um anjo para ELA: arquitetura baseada em visão computacional aplicada ao monitoramento on-line de parâmetros respiratórios de pacientes com Esclerose Lateral Amiotrófica (ELA) em ambiente hospitalar / An angel for ALS: architecture based on computer vision applied for on-line monitoring respiratory parameters of patients with Amyotrophic Lateral Sclerosis (ALS) in hospital environment

INTRODUÇÃO: A Esclerose Lateral Amiotrófica (ELA) é uma doença neurodegenerativa, caracterizada por uma progressiva e fatal perda de neurônios motores do córtex cerebral, tronco encefálico e medula espinhal, mas que mantém preservada a atividade intelectual e cognitiva do paciente. Pacientes acometidos por essa doença irão invariavelmente necessitar do auxílio de ventiladores mecânicos. MÉTODOS: Foi utilizado um conjunto de hardware e software para realizar o monitoramento dos parâmetros respiratórios dos pacientes em leitos hospitalares como forma de auxiliar à equipe de saúde. O monitoramento desses parâmetros deu-se por meio de uma webcam, que capturava os valores exibidos na tela do ventilador mecânico, e do emprego de técnicas de visão computacional e Optical Character Recognition (OCR). Neste sentido, o sistema foi testado sob três condições de luminosidade diferentes para verificar a eficácia do mesmo. RESULTADOS: O sistema apresentou uma média geral de acertos de 94.90%. Além disso, quando a interferência luminosa foi mínima, o sistema obteve uma média geral de acertos de 97,76%. CONCLUSÃO: A adoção de um sistema computacional baseado em visão computacional para auxílio da equipe de saúde no monitoramento hospitalar de pacientes com ELA mostrou-se satisfatória. No entanto, a pesquisa mostrou que a adoção de um sistema com maior imunidade à interferências luminosas externas tende a apresentar melhores resultados.

INTRODUCTION: Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by a progressive and fatal loss of motor neurons in the cerebral cortex, brainstem and spinal cord. In spite of that, the patient's intellectual and cognitive activity remains preserved. Patients affected by this disease will invariably need the help of mechanical ventilators. METHODS: A set of hardware and software was used to perform the monitoring of respiratory parameters of patients in hospital beds as a means of assisting the healthcare team. The monitoring of these parameters was performed by a webcam that captured the values displayed on the screen of the ventilator, and the employment of computer vision techniques and Optical Character Recognition (OCR). In this sense, the system was tested under three different lighting conditions to verify its effectiveness. RESULTS:The system presented an overall average of 94.90% of correct answers. Furthermore, when the luminous interference was minimum, it achieved an overall average of success of 97.76%. CONCLUSION: The adoption of a computational system based on computer vision to aid the healthcare team in hospital monitoring of patients with ALS was satisfactory. However, the research has shown that the adoption of a system with greater immunity to external light interference tends to achieve better results.