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WHAT IS THIS SUMMARY ABOUT?: Generalized myasthenia gravis (often shortened to gMG) is a rare health condition that causes muscular weakness. This summary gives an overview of three published articles that report the results of research studies of a medicine called ravulizumab, a treatment approved for adults with gMG. These studies are: The CHAMPION MG study. The CHAMPION MG extension study. A study of how the body processes and responds to ravulizumab (known as pharmacokinetics and pharmacodynamics). These studies looked at how effective and safe ravulizumab is for people with gMG. WHAT WERE THE RESULTS?: Overall, participants with gMG who received ravulizumab showed a significant and rapid improvement in their muscle strength and ability to do daily activities. These improvements were sustained for up to 60 weeks of treatment. Ravulizumab was well-tolerated overall, and no-one in the study had a meningococcal infection (a type of bacterial infection preventable with vaccination). Results from the pharmacokinetic and pharmacodynamic study support the use of ravulizumab every 8 weeks to maintain improvements in gMG. WHAT DO THE RESULTS OF THE STUDY MEAN?: Ravulizumab can be considered as a treatment option for adults with gMG who are appropriately protected against meningococcal infection before starting treatment. The drug, administered every 8 weeks, improves muscle strength and daily performance. These positive effects have been observed to persist over a long period of time.

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RATIONALE: Intensive care unit-acquired weakness (ICUAW) is common in critically ill patients, characterized by muscle weakness and physical function loss. Determining risk factors for ICUAW poses challenges due to variations in assessment methods and limited generalizability of results from specific populations, the existing literature on these risk factors lacks a clear and comprehensive synthesis. OBJECTIVE: This overview aimed to synthesize risk factors for ICUAW, categorizing its modifiable and nonmodifiable factors. METHODS: An overview of systematic reviews was conducted. Six relevant databases were searched for systematic reviews. Two pairs of reviewers selected reviews following predefined criteria, where bias was evaluated. Results were qualitatively summarized and an overlap analysis was performed for meta-analyses. RESULTS: Eighteen systematic reviews were included, comprising 24 risk factors for ICUAW. Meta-analyses were performed for 15 factors, while remaining reviews provided qualitative syntheses. Twelve reviews had low risk of bias, 4 reviews were unclear, and 2 reviews exhibited high risk of bias. The extent of overlap ranged from 0 to 23% for the corrected covered area index. Nonmodifiable factors, including advanced age, female gender, and multiple organ failure, were consistently associated with ICUAW. Modifiable factors, including neuromuscular blocking agents, hyperglycemia, and corticosteroids, yielded conflicting results. Aminoglycosides, renal replacement therapy, and norepinephrine were associated with ICUAW but with high heterogeneity. CONCLUSIONS: Multiple risk factors associated with ICUAW were identified, warranting consideration in prevention and treatment strategies. Some risk factors have produced conflicting results, and several remain underexplored, emphasizing the ongoing need for personalized studies encompassing all potential contributors to ICUAW development.

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BACKGROUND: Handgrip strength (HGS) testing is a highly recommended method for screening for sarcopenia in older adults. However, there is no consensus on the optimal protocol and number of trials for screening sarcopenia in older adults with cognitive impairment. OBJECTIVE: To investigate the use of the first trial (FT), the mean of three trials (MT), and the highest value (HT) from three trials of the HGS test to screen for sarcopenia in older adults with cognitive impairment. Additionally, to analyze the consistency, agreement, and measurement error in the diagnosis of muscle weakness. METHODS: 176 older adults with cognitive impairment were evaluated. The HGS test was repeated three times. Analyses were performed using the Friedman repeated measures test with Wilcoxon post-hoc, intraclass correlation coefficient (ICC), Standard Error of Measurement (SEM), Minimal Detectable Change (MDC95), and Kappa index tests. RESULTS: There was no significant difference between the first trial (FT) and the mean of three trials (MT) (d = 0.17 [95 % CI: -0.08, 0.42]), but both differed significantly from the highest value (HT) (p < 0.001). The ICC indicated a reliability of 0.97 (95 % CI: 0.95, 0.98) across all participants, while the kappa index demonstrated over 80 % agreement. The SEM for the first measure of HGS ranged from 0.59 to 2.12 kgf. The MDC95 ranged from 1.64 to 5.87 kgf. CONCLUSION: For HGS testing, there was excellent consistency between the FM and MT. All three testing methods demonstrated excellent agreement in diagnosing muscle weakness. The measurement errors confirm that FT can be reliably used to monitor changes during rehabilitation.

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BACKGROUND: Despite many efforts to provide children with legal existence over the last decades, 1 in 4 children under the age of 5 (166 million) do not officially exist, with limited possibility to enjoy their human rights. In Latin America and the Caribbean, Haiti has one of the highest rates of undocumented births. This study aimed to analyze the prevalence and the determinant factors of undocumented childhood in Haiti. METHODS: For analysis of undocumented childhood and related socioeconomic determinants, data from the 2016/17 Haiti demographic and health survey were used. The prevalence and the associated factors were analyzed using descriptive statistics and the binary logistic regression model. RESULTS: The prevalence of undocumented childhood in Haiti was 23% (95% CI: 21.9-24.0) among children under-five. Among the drivers of undocumented births, mothers with no formal education (aOR = 3.88; 95% CI 2.21-6.81), children aged less than 1 year (aOR = 20.47; 95% CI 16.83-24.89), children adopted or in foster care (aOR = 2.66; 95% CI 1.67-4.24), children from the poorest regions like "Artibonite" (aOR = 2.19; 95% CI 1.63-2.94) or "Centre" (aOR = 1.51; 95% CI 1.09-2.10) or "Nord-Ouest" (aOR = 1.61; 95% CI 1.11-2.34), children from poorest households (aOR = 6.25; 95% CI 4.37-8.93), and children whose mothers were dead (aOR = 2.45; 95% CI 1.33-4.49) had higher odds to be undocumented. CONCLUSION: According to our findings, there is an institutional necessity to bring birth documentation to underprivileged households, particularly those in the poorest regions where socioeconomic development programs are also needed. Interventions should focus on uneducated mothers who are reknown for giving birth outside of medical facilities. Therefore, an awareness campaign should be implemented to influence the children late-registering behavior.

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In this editorial, we comment on the article by Wang and Long, published in a recent issue of the World Journal of Clinical Cases. The article addresses the challenge of predicting intensive care unit-acquired weakness (ICUAW), a neuromuscular disorder affecting critically ill patients, by employing a novel processing strategy based on repeated machine learning. The editorial presents a dataset comprising clinical, demographic, and laboratory variables from intensive care unit (ICU) patients and employs a multilayer perceptron neural network model to predict ICUAW. The authors also performed a feature importance analysis to identify the most relevant risk factors for ICUAW. This editorial contributes to the growing body of literature on predictive modeling in critical care, offering insights into the potential of machine learning approaches to improve patient outcomes and guide clinical decision-making in the ICU setting.

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BACKGROUND: A nonvolitional diagnostic method based on FES-Cycling technology has recently been demonstrated for mechanically ventilated patients. This method presents good sensitivity and specificity for detecting muscle dysfunction and survival prognosis, even in unconscious patients. As the clinical relevance of this method has already been reported, we aimed to evaluate its safety and feasibility. METHODS: An observational prospective study was carried out with 20 critically ill, mechanically ventilated patients. The FES-cycling equipment was set in a specific diagnostic mode. For safety determination, hemodynamic parameters and peripheral oxygen saturation were measured before and immediately after the diagnostic protocol, as well as venous oxygen saturation and blood lactate. The creatine phosphokinase level (CPK) was measured before and 24, 48, and 72 h after the test. The time taken to carry out the entire diagnostic protocol and the number of patients with visible muscle contraction (capacity of perceptive muscular recruitment) were recorded to assess feasibility. RESULTS: Heart rate [91 ± 23 vs. 94 ± 23 bpm (p = 0.0837)], systolic [122 ± 19 vs. 124 ± 19 mm Hg (p = 0.4261)] and diastolic blood pressure [68 ± 13 vs. 70 ± 15 mm Hg (p = 0.3462)], and peripheral [98 (96-99) vs. 98 (95-99) % (p = 0.6353)] and venous oxygen saturation [71 ± 14 vs. 69 ± 14% (p = 0.1317)] did not change after the diagnostic protocol. Moreover, blood lactate [1.48 ± 0.65 vs. 1.53 ± 0.71 mmol/L (p = 0.2320)] did not change. CPK did not change up to 72 h after the test [99 (59-422) vs. 125 (66-674) (p = 0.2799) vs. 161 (66-352) (p > 0.999) vs. 100 (33-409) (p = 0.5901)]. The time taken to perform the diagnostic assessment was 11.3 ± 1.1 min. In addition, 75% of the patients presented very visible muscle contractions, and 25% of them presented barely visible muscle contractions. CONCLUSIONS: The FES cycling-based muscular dysfunction diagnostic method is safe and feasible. Hemodynamic parameters, peripheral oxygen saturation, venous oxygen saturation, and blood lactate did not change after the diagnostic protocol. The muscle damage marker (CPK) did not increase up to 72 h after the diagnostic protocol.

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BACKGROUND: Intensive care acquired muscle weakness is a common feature in critically ill patients. Beyond the therapeutic uses, FES-cycling could represent a promising nonvolitional evaluation method for detecting acquired muscle weakness. OBJECTIVES: To assess whether FES-cycling is able to identify muscle dysfunctions, and to evaluate the survival rate in patients with detected muscle dysfunction. METHODS: A prospective observational study was carried out, with 29 critically ill patients and 20 healthy subjects. Maximum torque and power achieved were recorded, in addition to the stimulation cost, and patients were followed up for six months. RESULTS: Torque (2.64 [1.53 to 4.81] vs 6.03 [4.56 to 6.73] Nm) and power (3.31 [2.33 to 6.37] vs 6.35 [5.22 to 10.70] watts) were lower and stimulation cost (22 915 [5069 to 37 750] vs 3411 [2080 to 4024] µC/W) was higher in patients compared to healthy people (p < 0.05). Surviving patients showed a nonsignificant difference in power and torque in relation to nonsurvivors (p > 0.05), but they had a lower stimulation cost (4462 [3598 to 11 788] vs 23 538 [10 164 to 39 836] µC/W) (p < 0.05). In total, 34% of all patients survived during the six months of follow-up. Furthermore, 62% of patients with a stimulation cost below 15 371 µC/W and 7% of patients with a stimulation cost above 15 371 µC/W survived. CONCLUSIONS: FES-cycling has good sensitivity and specificity for detecting muscle disorders. Critical patients have low torque and power and a high stimulation cost. Stimulation cost is related to survival. A low stimulation cost was related to a 3 times greater chance of survival.

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Las alteraciones cervicales son un problema multifactorial que afecta a la sociedad moderna. Posturas viciosas, traumatismos y defectos congénitos relacionados con la columna cervical pueden desarrollar inestabilidad, pinzamiento radicular, cervicoartrosis y cervicalgias. Objetivo. Relacionar el uso de dispositivos móviles con las alteraciones cervicales en estudiantes universitarios. Materiales y métodos. Estudio descriptivo, observacional, que se realizó entre los meses de mayo y julio del 2023, cuya muestra fue de 172 estudiantes universitarios que se obtuvo aplicando la fórmula para el cálculo muestral de poblaciones conocidas, mediante un muestreo no probabilístico. Se utilizó el test goniométrico para medir el rango articular, el test postural para identificar las alteraciones posturales, la técnica de palpación para identificar dolor inespecífico, prueba de resistencia para los músculos flexores (NFMET) y extensores (NEET), por último, se realizó la prueba de Spurling para identificar casos de radiculopatías. Resultados. Aunque las relaciones estadísticas no fueron consistentes, se observó que quienes utilizaron más tiempo los teléfonos celulares (87,0%) mostraron más limitaciones cervicales que los usuarios menos frecuentes (73,5%). La movilidad articular fue limitada en el 84,3% de la población, especialmente en varones (93,5%); la resistencia muscular normal en extensión fue más prevalente en el caso de los hombres (84,9%), mientras que la resistencia alterada en flexión fue más prevalente en mujeres (94,9%). Conclusiones. Según los resultados obtenidos en esta investigación, no se encontró suficiente evidencia para determinar una relación estadísticamente significativa (P˃0,05) entre las alteraciones cervicales y el uso de teléfonos celulares, aunque se observó una mayor limitación en el caso de quienes más tiempo utilizaban el dispositivo móvil.

Cervical disorders are a multifactorial problem affecting modern society. Vicious postures, trauma and congenital defects related to the cervical spine can develop instability, radicular impingement, cervicoarthrosis and cervicalgia. Objective. To relate the use of mobile devices with cervical disorders in university students. Materials and methods. Descriptive, observational study carried out between May and July 2023, with a sample of 172 university students obtained by applying the formula for the sample calculation of known populations, by means of non-probabilistic sampling. The goniometric test was used to measure joint range, the postural test to identify postural alterations, the palpation technique to identify non-specific pain, resistance test for flexor (NFMET) and extensor (NEET) muscles, and finally, the Spurling test was performed to identify cases of radiculopathy. Results. Although the statistical relationships were not consistent, it was observed that those who used cell phones longer (87.0%) showed more cervical limitations than less frequent users (73.5%). Joint mobility was limited in 84.3% of the population, especially in men (93.5%); normal muscular endurance in extension was more prevalent in men (84.9%), while impaired endurance in flexion was more prevalent in women (94.9%). Conclusions. According to the results obtained in this investigation, there was not enough evidence to determine a statistically significant relationship (P˃0.05) between cervical alterations and cell phone use, although a greater limitation was observed in the case of those who used the mobile device the longest.

Os distúrbios cervicais são um problema multifatorial que afeta a sociedade moderna. Posturas viciosas, traumas e defeitos congênitos relacionados à coluna cervical podem levar a instabilidade, impacto radicular, cervicoartrose e cervicalgia. Objetivo. Relacionar o uso de dispositivos móveis com distúrbios cervicais em estudantes universitários. Materiais e métodos. Estudo descritivo, observacional, realizado entre maio e julho de 2023, com uma amostra de 172 estudantes universitários obtida pela aplicação da fórmula para o cálculo de amostras de populações conhecidas, por meio de amostragem não probabilística. Foram utilizados o teste goniométrico para medir a amplitude articular, o teste postural para identificar alterações posturais, a técnica de palpação para identificar dores inespecíficas, o teste de resistência para músculos flexores (NFMET) e extensores (NEET) e o teste de Spurling para identificar casos de radiculopatia. Resultados. Embora as relações estatísticas não tenham sido consistentes, observou-se que aqueles que usavam telefones celulares por mais tempo (87,0%) apresentavam mais limitações cervicais do que os usuários menos frequentes (73,5%). A mobilidade articular foi limitada em 84,3% da população, especialmente no sexo masculino (93,5%); a resistência muscular normal em extensão foi mais prevalente no sexo masculino (84,9%), enquanto a resistência prejudicada em flexão foi mais prevalente no sexo feminino (94,9%). Conclusões. De acordo com os resultados obtidos nesta pesquisa, não houve evidências suficientes para determinar uma relação estatisticamente significativa (P˃0,05) entre os distúrbios cervicais e o uso de telefones celulares, embora tenha sido observada uma limitação maior no caso daqueles que usaram o dispositivo móvel por períodos mais longos.

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OBJECTIVE: The objective of this study was to evaluate the impact of intensive care unit (ICU)-acquired weakness (ICUAW) on the functional independence of patients hospitalized for coronavirus disease 2019 (COVID-19) over 6 months after ICU discharge. METHODS: This was a prospective cohort study that included patients who were admitted to the ICU because of COVID-19 and who were monitored for 6 months after discharge from the ICU via telephone. Patients were evaluated at 3 times (30 days, 3 months, and 6 months after discharge from the ICU) for functional independence for personal care and mobility activities (Barthel Scale), independence for self-care (Katz Index), impact of COVID-19 on functional status (post-COVID-19 Functional Status Scale [PCFS]), and mobility level (ICU Mobility Scale). The existence of some degree of dependence was considered when the Barthel Scale score was <100 points, the Katz Index was ≥1, and the PCFS score was ≥1. A PCFS score of ≥3 indicated moderate or severe dependence. Patients with a Medical Research Council score of <48 at discharge from the ICU were diagnosed with ICUAW. RESULTS: Sixty-eight patients were included, with a mean age of 51 (SD = 13) years. The ICUAW rate at ICU discharge was 35%. In the evaluation with the PCFS, the values for the presence of any functional limitation at 30 days, 3 months, and 6 months after ICU discharge were 89.7%, 57.4%, and 38.2%, respectively. The rate of persistence of functional limitations after 6 months was higher in patients with ICUAW than in those without ICUAW (66.7% vs 22.8%; P = .000); the same was true for moderate or severe limitations (20.8% vs 4.5%; P = .035). Likewise, functional independence for personal care, mobility, and self-care activities was poorer in patients with ICUAW. CONCLUSIONS: In patients surviving an ICU stay due to COVID-19, decreased functional independence persists even 6 months after discharge, and patients with ICUAW have worse outcomes. IMPACT: Patients who survive ICU stays due to COVID-19 continue to have greater functional dependence even 6 months after ICU discharge.

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BACKGROUND: Critical illness may affect muscle strength and mobility. OBJECTIVES: To compare muscle strength, mobility, and in intensive care unit (ICU)-acquired weakness (ICUAW) prevalence among individuals with COVID-19 and other critical illnesses; to identify factors associated with muscle strength, mobility, and length of stay in COVID-19; and to determine the Perme Intensive Care Unit Mobility Score (PICUMS) cut-off point for ICUAW. METHODS: We included individuals aged ≥18 in ICU who require mechanical ventilation. We excluded those diagnosed with neuromusculoskeletal diseases or who did not understand the study procedures. The Medical Research Council - sum score (MRC-SS) and the PICUMS were applied before ICU and hospital discharge. Analysis of covariance, Quade´s test, and Fisher's exact test compared groups. Partial correlations were analized between the MRC-SS and PICUMS with clinical variables. Regression models identified the predictors of hospital length of stay. The ROC curve verified the PICUMS related to ICUAW. Significance was set as P<.05. RESULTS: 25 individuals were included in the COVID-19 group and 23 in the non-COVID-19 group. No between-groups difference was observed in MRC-SS and PICUMS at discharge from ICU or hospital. The MRC-SS and PICUMS at ICU discharge predicted the length of hospital stay in the COVID-19 group. The PICUMS cut-off related to ICUAW was 18. CONCLUSIONS: Muscle strength, mobility, and ICUAW are similar between COVID-19 and non-COVID-19. However, muscle strength and mobility at ICU discharge are associated with the length of stay during COVID-19. A PICUMS<18 at ICU discharge may indicate impaired physical functioning due to ICUAW.

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Resumen Introducción : La efectividad de las terapias de reha bilitación física sobre los pacientes que requirieron ven tilación mecánica prolongada y egresaron de Unidad de Cuidados Intensivos (UCI) con debilidad neuromuscular post COVID-19 se conoce principalmente en el perio do agudo. El objetivo de este estudio fue caracterizar la recuperación funcional en personas con debilidad neuromuscular post UCI por COVID-19 admitidas a rehabilitación. Métodos : Estudio retrospectivo que incluyó a 42 pa cientes con debilidad neuromuscular post COVID-19, de dos centros de rehabilitación de tercer nivel, desde abril de 2020 hasta abril de 2022. Resultados : Encontramos diferencias estadísticamen te significativas entre las valoraciones funcionales de ingreso y alta. La Medida de Independencia Funcional (FIM) mejoró de 49 [41-57] a 107 [94-119] (p < 0.001). La escala de Berg de 4 [1-6] a 47 [36-54] (p < 0.001), el test de 6 minutos de 0 [0-0] a 254 [167-400] (p < 0.001), y el test de 10 metros de 0 [0-0] a 0.83 [0.4-1.2] (p < 0.001). No hubo diferencias estadísticamente significativas entre la puntuación total al ingreso y al alta de las evaluaciones funcionales con la edad y la complejidad respiratoria. Discusión : El tratamiento para la recuperación fun cional en un centro de tercer nivel y larga duración, sería beneficioso para personas con grave debilidad neuromuscular post UCI a causa del COVID-19, a pesar que el 43% no alcanzó el nivel de movilidad previo. La edad y la complejidad respiratoria son variables que no impactaron en la recuperación final.

Abstract Introduction : The effectiveness of physical rehabi litation therapies on patients who required prolonged mechanical ventilation and were discharged from the Intensive Care Unit (ICU) with post-COVID-19 neuro muscular weakness is known in the acute period. The objective of this study was to characterize the functional recovery in people hospitalized with post-ICU neuro muscular weakness due to COVID-19 admitted to rehab. Methods : Retrospective study which included 42 patients with post-COVID-19 neuromuscular weakness, who were admitted to two tertiary care rehabilitation centers, from April 2020 to April 2022. Results : We found statistically significant differen ces between the functional evaluations of admission and discharge. The Functional Independence Measure improved from 49 [41-57] a 107 [94-119] (p < 0.001). The Berg scale from 4 [1-6] a 47 [36-54] (p < 0.001), the 6-mi nute test from 0 [0-0] a 254 [167-400] (p < 0.001), and 421 the 10-meter test from 0 [0-0] a 0.83 [0.4-1.2] (p < 0.001). There were no statistically significant differences bet ween the admission and discharge total score of the functional assessments with age and respiratory com plexity. Discussion : Treatment for functional recovery in a tertiary and long-term center is beneficial for people with severe post-ICU neuromuscular weakness due to COVID-19, even though 43% did not reach the previous level of mobility. Age and respiratory complexity are variables that did not impact the final recovery.

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BACKGROUND: Antisynthetase syndrome is an inflammatory myopathy that is characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies. Only 30% of those who suffer from the disease can be identified. We present three Hispanic cases of antisynthetase syndrome with unusual clinical pictures were extended myositis panel results enable disease diagnosis and treatment. CASE PRESENTATION: A 57-year-old Hispanic/Latino female with an erythematous scaly plaque, unresolved fever and non-immune haemolytic anaemia in whom inpatient work-up for fever of unknown origin was positive for anti-PL12 positive myositis extended panel. A 72-year-old Hispanic/Latino male with amyopathic weakness syndrome and mechanic hands in whom impatient work-up was relevant for proximal muscle uptake and anti-PM75 and AntiPL-12 myositis extended panel. And a 67-year-old Hispanic/Latino male with progressive interstitial lung disease and unresolved fever ended in myositis extended panel positive for antiPL-7. After systemic immunosuppressor treatment, patients had favourable clinical and paraclinical responses during outpatient follow-up. CONCLUSIONS: The high variability of the antisynthetase syndrome in these cases demonstrates the importance of identification through an expanded panel and highlights the probability that this is a variable disease and that we need to include emerging molecular tests to promote the timely treatment of patients.

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INTRODUCTION: The effectiveness of physical rehabilitation therapies on patients who required prolonged mechanical ventilation and were discharged from the Intensive Care Unit (ICU) with post-COVID-19 neuromuscular weakness is known in the acute period. The objective of this study was to characterize the functional recovery in people hospitalized with post-ICU neuromuscular weakness due to COVID-19 admitted to rehab. METHODS: Retrospective study which included 42 patients with post-COVID-19 neuromuscular weakness, who were admitted to two tertiary care rehabilitation centers, from April 2020 to April 2022. RESULTS: We found statistically significant differences between the functional evaluations of admission and discharge. The Functional Independence Measure improved from 49 [41-57] a 107 [94-119] (p < 0.001). The Berg scale from 4 [1-6] a 47 [36-54] (p < 0.001), the 6-minute test from 0 [0-0] a 254 [167-400] (p < 0.001), and the 10-meter test from 0 [0-0] a 0.83 [0.4-1.2] (p < 0.001). There were no statistically significant differences between the admission and discharge total score of the functional assessments with age and respiratory complexity. DISCUSSION: Treatment for functional recovery in a tertiary and long-term center is beneficial for people with severe post-ICU neuromuscular weakness due to COVID-19, even though 43% did not reach the previous level of mobility. Age and respiratory complexity are variables that did not impact the final recovery.

Introducción: La efectividad de las terapias de rehabilitación física sobre los pacientes que requirieron ventilación mecánica prolongada y egresaron de Unidad de Cuidados Intensivos (UCI) con debilidad neuromuscular post COVID-19 se conoce principalmente en el periodo agudo. El objetivo de este estudio fue caracterizar la recuperación funcional en personas con debilidad neuromuscular post UCI por COVID-19 admitidas a rehabilitación. Métodos: Estudio retrospectivo que incluyó a 42 pacientes con debilidad neuromuscular post COVID-19, de dos centros de rehabilitación de tercer nivel, desde abril de 2020 hasta abril de 2022. Resultados: Encontramos diferencias estadísticamente significativas entre las valoraciones funcionales de ingreso y alta. La Medida de Independencia Funcional (FIM) mejoró de 49 [41-57] a 107 [94-119] (p < 0.001). La escala de Berg de 4 [1-6] a 47 [36-54] (p < 0.001), el test de 6 minutos de 0 [0-0] a 254 [167-400] (p < 0.001), y el test de 10 metros de 0 [0-0] a 0.83 [0.4-1.2] (p < 0.001). No hubo diferencias estadísticamente significativas entre la puntuación total al ingreso y al alta de las evaluaciones funcionales con la edad y la complejidad respiratoria. Discusión: El tratamiento para la recuperación funcional en un centro de tercer nivel y larga duración, sería beneficioso para personas con grave debilidad neuromuscular post UCI a causa del COVID-19, a pesar que el 43% no alcanzó el nivel de movilidad previo. La edad y la complejidad respiratoria son variables que no impactaron en la recuperación final.

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INTRODUCTION/AIMS: Considering the heterogeneity of the clinical manifestations of Duchenne muscular dystrophy (DMD), it is important to describe their various clinical profiles. Thus, in this study we aimed to develop percentile curves for DMD using a battery of measures to define the patterns of functional abilities, timed tests, muscle strength, and range of motion (ROM). METHODS: This retrospective data analysis was based on the records of patients with DMD using the Motor Function Measure (MFM) scale, isometric muscle strength (IS), dorsiflexion ROM, 10-meter walk test (10 MWT), and 6-minute walk test (6 MWT). Percentile curves (25th, 50th, and 75th percentiles) with MFM, IS, ROM, 10 MWT, and 6 MWT on the y axis and patient age on the x axis were constructed using the generalized additive model for location, scale, and shape, with Box-Cox power exponential distribution. RESULTS: There were records of 329 assessments of patients between 4 and 18 years of age. The MFM percentiles showed a gradual reduction in all dimensions. Muscle strength and ROM percentiles showed that the knee extensors were the most affected from 4 years of age, and dorsiflexion ROM negative values were noted from the age of 8 years. The 10 MWT showed a gradual increase in performance time with age. For the 6 MWT, the distance curve remained stable until 8 years, with a subsequent progressive decline. DISCUSSION: In this study we generated percentile curves that can help health professionals and caregivers follow the trajectory of disease progression in DMD patients.

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Introducción: La degeneración combinada subaguda (DCS) es un trastorno caracterizado por la degeneración difusa de la sustancia blanca a nivel del SNC, que afecta específicamente los cordones posteriores y laterales de la médula espinal, con pérdida de la mielinización periférica y central. De manera frecuente, las manifestaciones clínicas son parestesias y debilidad generalizada causada por deficiencia de vitamina B12. Presentación del caso: Paciente masculino de 79 años, con cuadro clínico de 3 meses de evolución de limitación funcional para la marcha acompañado de desorientación. Al examen físico evidenció desorientación, cuadriparesia e hiporreflexia, con niveles séricos bajos de vitamina B12, RM cervical con focos hiperintensos en el segmento C3/C6 y endoscopia de vías digestivas altas con atrofia de la mucosa gástrica. Presentamos un caso clínico de DCS. Discusión: Este es un caso de DCS que se manifiesta por medio de una alteración neuropsiquiátrica, con una presentación inicial inespecífica que comprende deterioro de la marcha, movimientos anormales con afectación cognitiva y psiquiátrica dada por alucinaciones visuales y desorientación. Su sospecha es importante en pacientes con factores de riesgo por medio del conocimiento de la patología, para una adecuada sospecha diagnóstica y una instauración oportuna de reposición vitamínica, la cual presenta una excelente respuesta. Conclusión: La DCS es un trastorno en el que se evidencia anemia con deficiencia de vitamina B12, des-mielinización del tejido nervioso y en muchos casos signos sugestivos de atrofia gástrica, y para ello es crucial la detección temprana de esta enfermedad por medio de la determinación de niveles séricos de vitamina B12, asociado a síntomas neurológicos, para así lograr su adecuado diagnóstico y tratamiento.

Introduction: Subacute combined degeneration (DCS) is a disorder characterized by diffuse degeneration of white matter at the CNS level, specifically affecting the posterior and lateral cords of the spinal cord, also with loss of peripheral and central myelination, frequently the clinical manifestations are paresthesias and generalized weakness caused by vitamin B12 deficiency. Case presentation: A 79-year-old male patient with a 3-month history of functional limitation for walking accompanied by disorientation. On physical examination, he revealed disorientation, quadriparesis, and hyporeflexia, with low serum levels of vitamin B12, cervical MRI with hyperintense foci in segment C3/C6, and upper digestive tract endoscopy with atrophy of the gastric mucosa. We present a clinical case of DCS. Discussion: This is a case of DCS that manifests itself through neuropsychiatric alteration with a nonspecific initial presentation with gait impairment, abnormal movements with cognitive and psychiatric affectation given by visual hallucinations and disorientation. Its suspicion is important in patients with risk factors. risk through knowledge of the pathology for an adequate diagnostic suspicion and a timely establishment of vitamin replacement for which it presents an excellent response. Conclusion: DCS is a disorder where anemia with vitamin B12 deficiency, demyelination of the nervous tissue and in many cases signs suggestive of gastric atrophy are evident, for which early detection of this disease is crucial through the determination of serum levels of vitamin B12 associated with neurological symptoms, in order to achieve its proper diagnosis and treatment.

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Abstract Objective: To investigate the relationship between Handgrip Strength (HGS), dysphagia classification, nutritional aspects, and Pharyngeal Transit Time (PTT) in subjects with Chronic Obstructive Pulmonary Disease (COPD). Methods: Study based on the analysis of secondary data from a database. The sample comprised 15 COPD patients of both sexes and a mean age of 65.7 years. We collected information on HGS, videofluoroscopic swallowing study, Volume-Viscosity Swallow Test (V-VST), and Body Mass Index (BMI). We applied correlation, effect size, and logistic regression tests at the 5% significance level. Results: Most individuals had severe COPD (66.7%), mean dominant HGS of 28.2, and non-dominant HGS of 25.3. Five subjects were malnourished, five were well-nourished, and five were obese. Most of them had normal swallowing (40%), normal V-VST results (60%), and PTT of 0.89 s (liquid) and 0.81 s (pudding-thick). There was no significant correlation between the swallowing classification and the other variables. We obtained a significant correlation (p = 0.015), though weak (r = -0.611), between non-dominant HGS and PTT. Regarding the binary logistic regression, HGS variables and HGS asymmetry were not enough to be considered a risk to clinically abnormal swallowing (V-VST). Conclusion: Subjects with COPD in this study had a longer PTT than reported in the literature for normal subjects and a weak correlation between PTT and non-dominant HGS. The variables related to muscle condition were not considered predictors for abnormal swallowing. Level of evidence: 3.

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Abstract Objective: To analyze the underlying components of reduced maximal static inspiratory (MIP) and expiratory (MEP) pressures in subjects with Duchenne muscular dystrophy. Methods: Forty-three subjects were assessed based on routine pulmonary function tests. MIP and MEP were measured the subjects performed maximal expirations and inspirations using a snorkel mouthpiece. Lung volumes were measured us ing the helium dilution technique. Results: The mean age was 13 years (range, 7-20 years). Median total lung capacity (TLC) and residual volume (RV) were 78.0 (49.0-94.0) and 27.0 (19.7-30.1) of the predicted values re spectively. The RV/TLC relationship was 35.3% (28.1-47.7). Thirty-five subjects had a TLC below the lower limit of normal, while 31 had an RV/TLC ratio above the upper limit of normal. The median (IQR) MIP and MEP values were -53.0 (-65.5 to -41.8) and 58.0 (41.5-74.8) cmH2O respectively. MIP and MEP in percent of the predicted values (predicted TLC and RV) were 42.6 (33.3-50.8) and 33.7 (23.9-44.5). MIP in percent of the RV reached for Group A (7-11 years old) was higher (p 0.025) while MEP in percent of the TLC reached for Group B (12-16 years) and C (17-20 years) were higher too (0.031). Conclusions: In subjects with Duchenne muscular dystrophy, the intrinsic weakness of respiratory muscles and mechanical disadvantage lead to inadequate maximal static pressure generation. Maximal static pressures should be interpreted cautiously as they overestimate respiratory muscle weakness when compared to predicted values obtained at TLC and RV. Our results provide additional data supporting absolute values use rather than predicted values.

Resumen Objetivo: Analizar los componentes subyacentes de las presiones inspiratorias (MIP) y espiratorias (MEP) es táticas máximas reducidas en sujetos con distrofia de Duchenne (DMD). Métodos: Se evaluaron 43 pacientes mediante pruebas de función pulmonar rutinarias. MIP y MEP fueron medidas a inspiración y espiración máximas. Los volúmenes pulmonares se midieron mediante dilución de helio. Resultados: Edad media 13 años (rango 7-20 años). La capacidad pulmonar total (TLC) y el volumen residual (RV) fueron 78.0% (49.0-94.0) y 27.0% (19.7- 30.1) de los valores predichos. El RV/TLC fue de 35.3% (28.1-47.7). Treinta y cinco sujetos tenían una TLC por debajo del límite inferior de normalidad, 31 tenían una RV/TLC por encima del límite superior de la normalidad. MIP y MEP fueron -53.0 (-65.5 a -41.8) y 58.0 (41.5-74.8) cmH2O, mientras que en % de los predichos (TLC y RV predichos) fueron 42.6 (33.3-50.8) y 33.7 (23.9-44.5). MIP en % del RV alcanzado (Grupo A 7-11 años) fue mayor (p 0.025), y MEP en % de la TLC alcanzada Grupo B (12-16 años) y C (17-20 años), también fue mayor (0.031). Conclusiones: En sujetos con DMD, debilidad intrínseca de los músculos respiratorios y desventaja mecánica conducen a generación de presión estática máxima inadecuada. Las mismas deben interpretarse con cautela, ya que sobrestiman la debilidad de los músculos respiratorios si se las compara con las tablas de valores predichos obtenidos a TLC y RV. Nuestros resultados proporcionan datos adicionales que respaldan la utilización de valores absolutos en lugar de los predichos.

RESUMEN

OBJECTIVE: To analyze the underlying components of reduced maximal static inspiratory (MIP) and expiratory (MEP) pressures in subjects with Duchenne muscular dystrophy. METHODS: Forty-three subjects were assessed based on routine pulmonary function tests. MIP and MEP were measured the subjects performed maximal expirations and inspirations using a snorkel mouthpiece. Lung volumes were measured using the helium dilution technique. RESULTS: The mean age was 13 years (range, 7-20 years). Median total lung capacity (TLC) and residual volume (RV) were 78.0 (49.0-94.0) and 27.0 (19.7-30.1) of the predicted values respectively. The RV/TLC relationship was 35.3% (28.1-47.7). Thirty-five subjects had a TLC below the lower limit of normal, while 31 had an RV/TLC ratio above the upper limit of normal. The median (IQR) MIP and MEP values were -53.0 (-65.5 to -41.8) and 58.0 (41.5-74.8) cmH2O respectively. MIP and MEP in percent of the predicted values (predicted TLC and RV) were 42.6 (33.3-50.8) and 33.7 (23.9-44.5). MIP in percent of the RV reached for Group A (7-11 years old) was higher (p 0.025) while MEP in percent of the TLC reached for Group B (12-16 years) and C (17-20 years) were higher too (0.031). CONCLUSIONS: In subjects with Duchenne muscular dystrophy, the intrinsic weakness of respiratory muscles and mechanical disadvantage lead to inadequate maximal static pressure generation. Maximal static pressures should be interpreted cautiously as they overestimate respiratory muscle weakness when compared to predicted values obtained at TLC and RV. Our results provide additional data supporting absolute values use rather than predicted values.

OBJETIVO: Analizar los componentes subyacentes de las presiones inspiratorias (MIP) y espiratorias (MEP) estáticas máximas reducidas en sujetos con distrofia de Duchenne (DMD). Métodos: Se evaluaron 43 pacientes mediante pruebas de función pulmonar rutinarias. MIP y MEP fueron medidas a inspiración y espiración máximas. Los volúmenes pulmonares se midieron mediante dilución de helio. RESULTADOS: Edad media 13 años (rango 7-20 años). La capacidad pulmonar total (TLC) y el volumen residual (RV) fueron 78.0% (49.0-94.0) y 27.0% (19.7- 30.1) de los valores predichos. El RV/TLC fue de 35.3% (28.1-47.7). Treinta y cinco sujetos tenían una TLC por debajo del límite inferior de normalidad, 31 tenían una RV/TLC por encima del límite superior de la normalidad. MIP y MEP fueron -53.0 (-65.5 a -41.8) y 58.0 (41.5-74.8) cmH2O, mientras que en % de los predichos (TLC y RV predichos) fueron 42.6 (33.3-50.8) y 33.7 (23.9-44.5). MIP en % del RV alcanzado (Grupo A 7-11 años) fue mayor (p 0.025), y MEP en % de la TLC alcanzada Grupo B (12-16 años) y C (17-20 años), también fue mayor (0.031). CONCLUSIONES: En sujetos con DMD, debilidad intrínseca de los músculos respiratorios y desventaja mecánica conducen a generación de presión estática máxima inadecuada. Las mismas deben interpretarse con cautela, ya que sobrestiman la debilidad de los músculos respiratorios si se las compara con las tablas de valores predichos obtenidos a TLC y RV. Nuestros resultados proporcionan datos adicionales que respaldan la utilización de valores absolutos en lugar de los predichos.

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