Navegando por Autor "Dourado Junior, Mário Emílio Teixeira"

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Accessory nerve injury after deep brain stimulation surgery in a Parkinson’s disease patient
(2022-03) Aquino, Bárbara Cristina Vieira de; Pessoa Neto, Agábio Diógenes; Dourado Junior, Mário Emílio Teixeira; Dantas, Sérgio Adrian Fernandes; Silva, Rodrigo Alencar e; Godeiro Junior, Clécio de Oliveira
DBS is an established form of surgical therapy for Parkinson's disease and is also used for other pathologies, such as dystonia and essential tremor. There are few articles in the literature discussing exclusively about surgical complications. In fact, most papers describe surgical-related complications such seizures, device infection, hemorrhage and permanent deficit. However, less usual complications can also occur, as in the case described below, of an injury to the accessory nerve after the DBS procedure. We describe a case of a 56-year-old woman with PD who underwent to STN DBS, presented hypotrophy and weakness of the right trapezius after the surgery. Electroneuromyography (ENMG) suggested partial right spinal accessory injury. Patient recovery spontaneously 5 months after surgery. While DBS promises dramatic improvement in select PD symptoms, it can have disappointing or even serious adverse results. This paper ratifies the need of screening of accessory nerve injury for early rehabilitation.
Antecedent infections in Guillain-Barré syndrome in endemic areas of arbovirus transmission: a multinational case-control study
(Wiley, 2021) Dourado Junior, Mário Emílio Teixeira; Leonhard, Sonja E.; Tan, heng Yin; Leonhard, Sonja E; Tan, Cheng Yin; Eijk, Annemiek A.; Reisin, Ricardo R.; Franken, Suzanne C.; Huizinga, Ruth; Arends, Samuel; Batstra, Manou R.; Jeronimo, Selma M. Bezerra; Drenthen, Judith; https://orcid.org/0000-0002-9462-2294
Half of the world's population is at risk of arthropod-borne virus (arbovirus) infec-tions. Several arbovirus infections have been associated with Guillain-Barré syn-drome (GBS). We investigated whether arboviruses are driving GBS beyond epidemicphases of transmission and studied the antibody response to glycolipids. The proto-col of the International Guillain-Barré syndrome Outcome Study (IGOS), an observa-tional prospective cohort study, was adapted to a case-control design. Serumsamples were tested for a recent infection with Zika virus (ZIKV), dengue virus(DENV), chikungunya (CHIKV) virus, hepatitis E virus, Epstein-Barr virus (EBV), cyto-megalovirus (CMV),Campylobacter jejuni, andMycoplasma pneumoniae, and for anti-bodies to glycolipids. Forty-nine patients were included from Brazil (63%), Argentina(14%), and Malaysia (22%). Evidence of a recent infection was found in 27/49 (55%)patients:C jejuni(n=15, 31%),M pneumoniae(n=5, 10%), CHIKV (n=2, 4%), EBV(n=1, 2%),C jejuniandM pneumoniae(n=2, 4%), CMV and DENV (n=1, 2%), andC jejuniand DENV (n=1, 2%). In 22 patients, 35 paired controls were collected.Odds ratio for recent infections did not significantly differ between cases and con-trols. No typical anti-ganglioside antibody binding was associated with recent arbovi-rus infection. We conclude that arbovirus infections occur in GBS patients outside ofepidemic viral transmission, although not significantly more than in controls. Broadinfection and anti-ganglioside antibody serology are important to establish the mostlikely pathogenic trigger in GBS patients. Larger studies are necessary to determinethe association between arboviruses and GBS.
Association of copper status with lipid profile and functional status in patients with amyotrophic lateral sclerosis
(Journal Of Nutrition And Metabolism, 2018-07-19) Dourado Junior, Mário Emílio Teixeira; Barros, Acsa Nara A. B.; Pedrosa, Lucia de Fatima C.; Lais, Lucia Leite; https://orcid.org/0000-0002-9462-2294
Oxidative stress is one of the main mechanisms associated with the pathogenesis of amyotrophic lateral sclerosis (ALS). Copper can affect cellular oxidation and lipid metabolism. The aim of this study was to evaluate the association of copper status with lipid profile and functional status in patients with ALS. A cross-sectional study was carried out including 27 patients with ALS (case group) and 26 healthy individuals (control group). Copper status was evaluated by habitual dietary copper intake, plasma copper, and serum ceruloplasmin concentrations. The lipid profile included analysis of serum total cholesterol (TC), LDL-cholesterol (LDL-c), HDL-cholesterol (HDL-c), and triglycerides (TGL). The functional status of patients with ALS was assessed by the ALS Functional Rating Scale-Revised (ALSFRS-R). In the case group, plasma copper was lower compared with the control group (133.9 versus 164.1 μg/dL, ) and was positively correlated with HDL-c (, ). In the control group, plasma copper was positively correlated with serum ceruloplasmin (, ), TC (, ), LDL-c (, ), and HDL-c (, ), and serum ceruloplasmin was positively correlated only with LDL-c (, ). In the case group, dietary copper intake (, ), plasma copper (, ), and TC (, ) were inversely associated with the functional status of patients with ALS. In contrast, serum ceruloplasmin (, ), LDL-c (, ), HDL-c (, ), and TGL (; ) were positively associated with their functional status. In conclusion, this study suggests a disturbance of copper status and its connection with the lipid profile in patients with ALS. Furthermore, copper status and lipid profile may influence the functional status of patients with ALS, standing out as potential biomarkers of disease severity.
Biomedical signals and machine learning in amyotrophic lateral sclerosis: a systematic review
(Biomedical Engineering Online, 2021) Dourado Junior, Mário Emílio Teixeira; Fernandes, Felipe; Barbalho, Ingridy; Barros, Daniele; Valentim, Ricardo; Teixeira, César; Henriques, Jorge; Gil, Paulo; https://orcid.org/0000-0002-9462-2294
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 demystifed. 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 defnition 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 identifed 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.
CAG repeats ≥ 34 in Ataxin-1 gene are associated with amyotrophic lateral sclerosis in a Brazilian cohort
(Elsevier, 2020) Dourado Junior, Mário Emílio Teixeira; Gonçalves, João Pedro Nunes; Andrade, Helen Maia Tavares de; Cintra, Vívian Pedigone; et, al; https://orcid.org/0000-0002-9462-2294
Little is known about the genetic basis of amyotrophic lateral sclerosis (ALS) outside Europe and US. In this study, we investigated whether intermediate CAG expansions at ATXN1 were associated to ALS in the Brazilian population. To accomplish that, representative samples from 411 unrelated patients and 436 neurologically normal controls from 6 centers spread over the territory were genotyped to quantify ATXN1 expansions. We found that ATXN1 intermediate-length expansion (≥34 CAG repeats) are associated with the disease (odds ratio = 2.19, 95% CI = 1.081–4.441, p = .026). Most ATXN1-positive patients had classical phenotype, but some of them presented predominant lower motor neuron involvement. None of them had associated ataxia. Frontotemporal dementia was concomitantly found in 12.5% of patients carrying the intermediate ATXN1 expansion. Further studies are needed to validate these findings and to understand the pathophysiological mechanisms that connect ataxin-1 and ALS.
Clinical and genetic spectrum of a large cohort of patients with δ-sarcoglycan muscular dystrophy
(Brain, 2021) Dourado Junior, Mário Emílio Teixeira; Perez, Jorge Alonso; Quereda, Lidia Gonzalez; et, al; https://orcid.org/0000-0002-9462-2294
Sarcoglycanopathies include four subtypes of autosomal recessive limb-girdle muscular dystrophies (LGMDR3, LGMDR4, LGMDR5 and LGMDR6) that are caused, respectively, by mutations in the SGCA, SGCB, SGCG and SGCD genes. Delta-sarcoglycanopathy (LGMDR6) is the least frequent and is considered an ultra-rare disease. Our aim was to characterize the clinical and genetic spectrum of a large international cohort of LGMDR6 patients and to investigate whether or not genetic or protein expression data could predict a disease’s severity. This is a retrospective study collecting demographic, genetic, clinical and histological data of patients with genetically confirmed LGMDR6 including protein expression data from muscle biopsies. We contacted 128 paediatric and adult neuromuscular units around the world that reviewed genetic data of patients with a clinical diagnosis of a neuromuscular disorder. We identified 30 patients with a confirmed diagnosis of LGMDR6 of which 23 patients were included in this study. Eighty-seven per cent of the patients had consanguineous parents. Ninety-one per cent of the patients were symptomatic at the time of the analysis. Proximal muscle weakness of the upper and lower limbs was the most common presenting symptom. Distal muscle weakness was observed early over the course of the disease in 56.5% of the patients. Cardiac involvement was reported in five patients (21.7%) and four patients (17.4%) required non-invasive ventilation. Sixty per cent of patients were wheelchair-bound since early teens (median age of 12.0 years). Patients with absent expression of the sarcoglycan complex on muscle biopsy had a significant earlier onset of symptoms and an earlier age of loss of ambulation compared to patients with residual protein expression. This study confirmed that delta-sarcoglycanopathy is an ultra-rare neuromuscular condition and described the clinical and molecular characteristics of the largest yet-reported collected cohort of patients. Our results showed that this is a very severe and quickly progressive disease characterized by generalized muscle weakness affecting predominantly proximal and distal muscles of the limbs. Similar to other forms of sarcoglycanopathies, the severity and rate of progressive weakness correlates inversely with the abundance of protein on muscle biopsy.
Clinical and molecular findings in a cohort of ANO5-related myopathy
(Wiley, 2019) Dourado Junior, Mário Emílio Teixeira; Silva, André M. S.; Coimbra-Neto, Antônio R.; et al.; https://orcid.org/0000-0002-9462-2294
Objective:ANO5-related myopathy is an important cause of limb-girdle muscu-lar dystrophy (LGMD) and hyperCKemia. The main descriptions have emergedfrom European cohorts, and the burden of the disease worldwide is unclear. Weprovide a detailed characterization of a large Brazilian cohort fANO5patients.Methods: A national cross-sectional study was conducted to describe clinical,histopathological, radiological, and molecular features of patients carrying reces-sive variants inANO5. Correlation of clinical and genetic characteristics with dif-ferent phenotypes was studied.Results: Thirty-seven patients from 34 nonrelatedfamilies with recessive mutations ofANO5were identified. The most commonphenotype was LGMD, observed in 25 (67.5%) patients, followed by pseu-dometabolic presentation in 7 (18.9%) patients, isolated asymptomatic hyperCK-emia in 4 (10.8%) patients, and distal myopathy in a single patient. Nine patientspresented axial involvement, including one patient with isolated axial weakness.The most affected muscles according to MRI were the semimembranosus and gas-trocnemius, but paraspinal and abdominal muscles, when studied, were involved in most patients. Fourteen variants inANO5were identified, and the c.191dupAwas present in 19 (56%) families. Sex, years of disease, and the presence of loss-of-function variants were not associated with specific phenotypes.Interpretation:We present the largest series of anoctaminopathy outside Europe. The most com-mon European founder mutation c.191dupA was very frequent in our population.Gender, disease duration, and genotype did not determine the phenotype.
Clinical characteristics of Guillain–Barré syndrome in a tropical country: a Brazilian experience
(Acta Neurologica Scandinavica, 2011) Dourado Junior, Mário Emílio Teixeira; Félix, R. H.; Silva, W. K. A. da; Queiroz, J. W.; Jeronimo, Selma Maria Bezerra; https://orcid.org/0000-0002-9462-2294
Objective – To analyze the clinical variants, outcomes, and prognosis of Guillain–Barré syndrome (GBS) in a Brazilian population. Materials and methods – Clinical and laboratory data of 149 cases of GBS diagnosed from 1994 to 2007 were analyzed. Results – Acute inflammatory demyelinating polyneuropathy (AIDP) was the most frequent variant (81.8%) of GBS, followed by acute motor axonal neuropathy (AMAN) (14.7%) and acute motor and sensory axonal neuropathy (AMSAN) (3.3%). The incidence of GBS was 0.3/100,000 for the state of Rio Grande do Norte and cases occurred at a younger age. GBS was preceded by infections, with the axonal variant associated with episodes of diarrheas (P = 0.025). Proximal weakness was more frequent in AIDP, and distal weakness predominant in the axonal variant. Compared to 42.4% of cases with AIDP (P < 0.0001), 84.6% of cases with the axonal variant had nadir in <10 days. Individuals with the axonal variant took longer to recover deambulation (P < 0.0001). The mortality of GBS was 5.3%. Conclusion – A predominance of the AIDP variant was seen, and the incidence of the disease decreased with age. As expected, the distribution of weakness correlated with the clinical variants, and individuals with the axonal variant had a poorer prognosis.
Clinical Features of COVID-19 on patients with neuromyelitis optica spectrum disorders
(Neuroimmunology Neuroinflammation, 2021) Dourado Junior, Mário Emílio Teixeira; Ferreira, Lis Campos; Pereira, Samira Luisa Apostolos; et, al; https://orcid.org/0000-0002-9462-2294
Background and Objectives To describe the clinical features and disease outcomes of coronavirus disease 2019 (COVID-19) in patients with neuromyelitis optica spectrum disorder (NMOSD). Methods The Neuroimmunology Brazilian Study Group has set up the report of severe acute respiratory syndrome (SARS-CoV2) cases in patients with NMOSD (pwNMOSD) using a designed web-based case report form. All neuroimmunology outpatient centers and individual neurologists were invited to register their patients across the country. Data collected between March 19 and July 25, 2020, were uploaded at the REDONE.br platform. Inclusion criteria were as follows: (1) NMOSD diagnosis according to the 2015 International Panel Criteria and (2) confirmed SARS-CoV2 infection (reverse transcription-polymerase chain reaction or serology) or clinical suspicion of COVID-19, diagnosed according to Center for Disease Control / Council of State and Territorial Epidemiologists (CDC/CSTE) case definition. Demographic and NMOSD-related clinical data, comorbidities, disease-modifying therapy (DMT), COVID-19 clinical features, and severity were described. Results Among the 2,061 pwNMOSD followed up by Brazilian neurologists involved on the registry of COVID-19 in pwNMOSD at the REDONE.br platform, 34 patients (29 women) aged 37 years (range 8–77), with disease onset at 31 years (range 4–69) and disease duration of 6 years (range 0.2–20.5), developed COVID-19 (18 confirmed and 16 probable cases). Most patients exhibited mild disease, being treated at home (77%); 4 patients required admission at intensive care units (severe cases); and 1 patient died. Five of 34 (15%) presented neurologic manifestations (relapse or pseudoexacerbation) during or after SARS-CoV2 infection. Discussion Most NMOSD patients with COVID-19 presented mild disease forms. However, pwNMOSD had much higher odds of hospitalization and intensive care unit admission comparing with the general Brazilian population. The frequency of death was not clearly different. NMOSD disability, DMT type, and comorbidities were not associated with COVID-19 outcome. SARS-CoV2 infection was demonstrated as a risk factor for NMOSD relapses. Collaborative studies using shared NMOSD data are needed to suitably define factors related to COVID-19 severity and neurologic manifestations.
Clinical neurophysiology of Zika virus–related disorders of the peripheral nervous system in adults
(Journal Of Clinical Neurophysiology, 2022) Dourado Junior, Mário Emílio Teixeira; Salazar, Carelis González; Tartaglia, Jordana Sartori; França Jr, Marcondes C.; https://orcid.org/0000-0002-9462-2294
: During the 2013 to 2016 outbreak in the Pacific and Americas, Zika virus infection resulted not only in febrile and cutaneous manifestations but also in (severe) neurologic complications. These included both central and peripheral nervous system disorders. The most frequent was Guillain–Barré syndrome that typically developed 1 to 2 weeks after the acute infection. Later, other peripheral nervous system syndromes were recognized in association with the viral infection, broadening the spectrum of Zika virus–related peripheral nervous system syndromes. In the current article, the authors review all available clinical neurophysiology data on Guillain–Barré syndrome and other peripheral nervous system syndromes in an attempt to characterize the major patterns of involvement related to Zika virus. The authors also highlight the clinical usefulness of nerve conduction studies and needle EMG in the investigation of suspected Zika virus–related Guillain–Barré syndrome.
Confiabilidade do teste da caminhada de seis minutos em pacientes com miastenia gravis generalizada
(Fisioterapia e Pesquisa, 2009) Dourado Junior, Mário Emílio Teixeira; Resqueti, Vanessa Regiane; Oliveira, Georges Willeneuwe de Sousa; Andrade, Armèle Dornelas de; Casan, Pere; Fregonezi, Guilherme Augusto de Freitas; https://orcid.org/0000-0002-9462-2294
Este estudo objetivou determinar a confiabilidade do teste da caminhada de seis minutos (TC6M) como um teste de capacidade funcional em pacientes com miastenia gravis generalizada (MG). Foram selecionados 11 pacientes com MG - 5 homens, 6 mulheres - com idade de 55±9 anos, avaliados inicialmente quanto à função fulmonar, que se submeteram a três TC6M em dias diferentes. Durante e/ou após cada teste foram medidas freqüência cardíaca e saturação de oxigênio (por oxímetro portátil), sensação de dispnéia (pela escala de Borg) e distância percorrida. Nos três testes as distâncias percorridas foram 498 m, 517 m e 520 m (respectivamente 99%, 103% e 104% do valor predito). Em média, a freqüência cardíaca, dispnéia e saturação de oxigênio mostraram comportamento constante nos três testes. Foram encontradas alta confiabilidade relativa, com coeficiente de correlação interclasse maior que 0,90 entre os testes (TC6M1-TC6M2, 0,960; TC6M1-TC6M3, 0,945; e TC6M2-TC6M3, 0,970) e confiabilidade absoluta de 4%, 3,5% e 4,8%, com reprodutibilidade de 11%, 9,8% e 13,4%, respectivamente para o primeiro, segundo e terceiro testes. Os limites superiores e inferiores de concordância e o valor médio das médias das diferenças (bias) calculados pelo teste de Bland-Altman mostraram-se clinicamente aceitáveis. Conclui-se que o TC6M se mostrou seguro, confiável e reprodutível, podendo ser aplicado para avaliação e seguimento da tolerância ao exercício em pacientes com MG generalizada.
Coping strategies among amyotrophic lateral sclerosis (ALS) patients: an integrative review
(Springer, 2022) Dourado Junior, Mário Emílio Teixeira; Leandro, Georgiana Soares; Santana, Glauciane Costa; Dantas, Luan Samy Xavier; https://orcid.org/0000-0002-9462-2294
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.
Cytomegalovirus infection in Guillain-Barré syndrome: a retrospective study in Brazil
(Arquivos de Neuro-Psiquiatria, 2021) Dourado Junior, Mário Emílio Teixeira; Sousa, Bruno Fernandes de; Costa, Nathaly M. Coelho da; Jeronimo, Selma Maria Bezerra; https://orcid.org/0000-0002-9462-2294
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.
Diagnosis and management of Guillain–Barré syndrome in ten steps
(Springer, 2019) Dourado Junior, Mário Emílio Teixeira; Leonhard, Sonja E.; Mandarakas, Melissa R.; et al.; https://orcid.org/0000-0002-9462-2294
Guillain–Barré syndrome (GBS) is a rare, but potentially fatal, immune-mediated disease of the peripheral nerves and nerve roots that is usually triggered by infections. The incidence of GBS can therefore increase during outbreaks of infectious diseases, as was seen during the Zika virus epidemics in 2013 in French Polynesia and 2015 in Latin America. Diagnosis and management of GBS can be complicated as its clinical presentation and disease course are heterogeneous, and no international clinical guidelines are currently available. To support clinicians, especially in the context of an outbreak, we have developed a globally applicable guideline for the diagnosis and management of GBS. The guideline is based on current literature and expert consensus, and has a ten-step structure to facilitate its use in clinical practice. We first provide an introduction to the diagnostic criteria, clinical variants and differential diagnoses of GBS. The ten steps then cover early recognition and diagnosis of GBS, admission to the intensive care unit, treatment indication and selection, monitoring and treatment of disease progression, prediction of clinical course and outcome, and management of complications and sequelae.
Dietary intake and zinc status in amyotrophic lateral sclerosis patients
(Nutrición Hospitalaria, 2017) Dourado Junior, Mário Emílio Teixeira; Silva, Heloisa Fernanda Lopes da; Brito, Acsa Nara de Araújo; Freitas, Erika Paula Silva de; Evangelista, Karine Cavalcanti Maurício de Sena; Lais, Lúcia Leite; https://orcid.org/0000-0002-9462-2294
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.
Effects of air stacking maneuver on cough peak flow and chest wall compartmental volumes of subjects with amyotrophic lateral sclerosis
(Elsevier, 2017) Dourado Junior, Mário Emílio Teixeira; Sarmento, Antonio; Resqueti, Vanessa Resqueti; Saturnino, Lailane; Aliverti, Andrea; Fregonezi, Guilherme; Andrade, Armele Dornelas de; https://orcid.org/0000-0002-9462-2294
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.
Effects of positioning on cough peak flow and muscular electromyographic activation in duchenne muscular dystrophy
(Respiratory Care, 2020) Dourado Junior, Mário Emílio Teixeira; Marques, Layana; Santos, Ilsa Priscila; Marcelino, Ana Aline; Fonseca, Jessica Danielle Medeiros da; Aliverti, Andrea; Sarmento, Antonio; Resqueti, Vanessa Regiane; Fregonezi, Guilherme Augusto de Freitas; https://orcid.org/0000-0002-9462-2294
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 5 .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.
Effects of respiratory muscle training on respiratory muscle strength and heart rate variability in myotonic dystrophy patients type 1
(Journal of Respiratory and CardioVascular Physical Therapy, 2012) Dourado Junior, Mário Emílio Teixeira; Araújo, Thayse Lucena; Resqueti, Vanessa Regiane; Lima, Illia Nadinne Dantas Florentino; Fregonezi, Guilherme; https://orcid.org/0000-0002-9462-2294
Introduction: Myotonic dystrophy (MD) is a multisystemic neuromuscular disease responsible for causing progressive respiratory muscle weakness. Respiratory muscle training (MRT) has been shown to be effective in several diseases; however, its effects on respiratory and cardiac functions in MD are still inconsistent. Objective: Assess the effects of MRT on respiratory muscle strength and heart rate variability in patients with type 1 myotonic dystrophy. Methods: The sample was composed of 6 individuals of both sexes. The following was assessed: respiratory muscle strength, before and after training (sessions 1-4) and heart rate variability before and after sessions 2 and 3. The group submitted to MRT used the Threshold IMT device, adapted for inspiratory and expiratory training, three times a week, once at an outpatient facility and twice at home. Results were expressed as median and interquartile ranges for pulmonary function variables, and the Friedman and Wilcoxon tests were applied to compare heart rate variability. Results: respiratory muscle strength significantly improved expiratory and inspiratory muscles (33% and 20%, respectively), considering training sessions 1-4. With respect to variability, there was a 102% increase in sympathetic activity, reflected by low frequency and 194% increase in vagal tonus, represented by high frequency. Conclusions: Preliminary study results demonstrate that a partial home-based respiratory muscle-training program is feasible, in addition to improving strength and heart rate variability in patients with MD.
Egos has a reduced capacity to predicts GBS prognosis in Northeast Brazil
(Wiley, 2018) Dourado Junior, Mário Emílio Teixeira; Fernandes, Ugor T.; Ramos, Eugênio S.; Vital, Ana L. F.; Urbano, João C. C.; Queiroz, José W.; Jeronimo, Selma M. B.; https://orcid.org/0000-0002-9462-2294
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 as-sessed 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 in-volvement and upper respiratory infection. There was no difference relative to sex or presence of anti- gangliosides antibodies. The demyelinating variant was more com-mon (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%) pre-sented 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.
Eletroneuromiografia: estimativa do número de unidades motoras na ELA
(Revista Neurociências, 2006) Dourado Junior, Mário Emílio Teixeira; https://orcid.org/0000-0002-9462-2294
Passados 132 anos da descrição do quadro clínico da Esclerose Lateral Amiotrófica (ELA) por Charcot infelizmente a história natural desta enfermidade não se modificou. É a degeneração dos neurônios motores, cerebral e espinhal, responsável pelos principais sinais e sintomas da doença, que leva a perda progressiva da função e ao óbito, em média, cinco anos após o diagnóstico. Nos últimos anos, entretanto, houve um grande avanço no entendimento desta enfermidade, tornando-se um exemplo de doença neurodegenerativa como modelo para testes terapêuticos.

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