Search

Introduction: The aim of this study was to determine the behaviour of ultrasound biomarkers of fascicle density and muscle strength in patients with amyotrophic lateral sclerosis (ALS). Methods: We conducted an observational, cross-sectional pilot study of 14 patients with ALS (28.6% women) and 14 controls. Bilateral cross-sectional ultrasound scans were performed in the abductor pollicis brevis (APB) and tibialis anterior (TA) muscles, with recording of muscle thickness (MT) at rest and in contraction, and the difference in thickness. In the median, sciatic, and common peroneal nerves, we analysed the cross-sectional area (CSA), number of fascicles (NF) and fascicle density (FD). Analyses were nested by laterality. Results: Intra- and interrater agreement regarding NF was very good, with a minimum detectable error of

Background: amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a progressive course. The current prevalence is between 3 and 6 cases/100,000. Malnutrition is closely related to patient prognosis in ALS. The implications of this conditions have been that we should recommend patient care in a multidisciplinary unit. Case report: the case presented shows the evolution of a patient with ALS. The patient was referred to different clinical departments after neurological evaluation and her nutritional, functional and respiratory status were assessed. There was no nutritional deterioration at diagnosis; however, intake was below energy-protein requirements. The clinical evolution of the patient showed a decrease in muscle mass with preservation of weight and fat mass. "Aggressive" measures to control nutritional status such as gastrostomy were rejected in the initial stages of the disease, but had to be carried out after development of dysphagia and associated malnutrition. This situation of progressive morphofunctional deterioration and the development of disease-related complications made essential the participation of different health services and professionals in its control. Dicussion: the management of ALS in a multidisciplinary manner allows to improve the course of the disease and the quality of life of both the patients and their families. Patient follow-up is based on the adjustment and management of complications. The basis of the relationship with these patients includes maintaining an adequate communication with them and their families, and ensuring joint decision-making about their condition. [ABSTRACT FROM AUTHOR]

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease whose aetiology is unknown. It is characterised by upper and lower motor neuron degeneration. Approximately 90% of cases of ALS are sporadic, whereas the other 10% are familial. Regardless of whether the case is familial o sporadic, patients will develop progressive weakness, muscle atrophy with spasticity, and muscle contractures. Life expectancy of these patients is generally 2 to 5 years after diagnosis. Development: In vivo models have helped to clarify the aetiology and pathogenesis of ALS, as well as the mechanisms of the disease. However, as these mechanisms are not yet fully understood, experimental models are essential to the continued study of the pathogenesis of ALS, as well as in the search for possible therapeutic targets. Although 90% of cases are sporadic, most of the models used to study ALS pathogenesis are based on genetic mutations associated with the familial form of the disease; the pathogenesis of sporadic ALS remains unknown. Therefore, it would be critical to establish models based on the sporadic form. Conclusions: This article reviews the main genetic and sporadic experimental models used in the study of this disease, focusing on those that have been developed using rodents. Resumen: Introducción: La esclerosis lateral amiotrófica (ELA) es una patología neurodegenerativa, progresiva y de etiología desconocida caracterizada por la degeneración de motoneuronas superiores e inferiores. Aproximadamente el 90% de los casos de ELA son esporádicos mientras que el 10% restante se consideran familiares. Independientemente de si son familiares o esporádicas, los pacientes desarrollan una debilidad progresiva, atrofia muscular con espasticidad y contracturas. Por lo general, la esperanza de vida en los pacientes de ELA es de 2 a 5 años. Desarrollo: Los modelos in vivo han ayudado a explicar la etiología y la patogénesis, así como los mecanismos de la esclerosis lateral amiotrófica. Sin embargo, estos mecanismos no están del todo esclarecidos aún, por lo que los modelos experimentales son fundamentales para continuar con el estudio de los mismos, así como para la búsqueda de posibles dianas terapéuticas. A pesar de que el 90% de los casos son esporádicos, la mayoría de los modelos utilizados hasta la actualidad para estudiar la patogénesis están basados en las mutaciones genéticas asociadas a la patología familiar, lo que provoca que la patogénesis de la ELA esporádica no sea aún conocida., Por tanto, sería fundamental el estudio de la enfermedad en modelos basados en la patología esporádica. Conclusión: En el presente artículo se han revisado los principales modelos experimentales tanto genéticos como esporádicos utilizados en el estudio de esta enfermedad, enfocándonos en los que se han desarrollado utilizando el roedor como plataforma experimental.

Introducción. La esclerosis lateral amiotrófica (ELA) es una enfermedad degenerativa del sistema nervioso en la cual las neuronas motoras se desgastan y mueren, lo que se manifiesta con la aparición de debilidad muscular progresiva, espasmos e incapacidad para mover los brazos, las piernas y, finalmente, todo el cuerpo. Los estándares de cuidado para pacientes con ELA recomiendan atención multidisciplinaria que incluya intervenciones de rehabilitación. El ejercicio físico ha sido utilizado para su tratamiento; sin embargo, existen pocas recomendaciones específicas respecto a su prescripción. Desarrollo. Se realizó una revisión de la literatura disponible respecto a la rehabilitación basada en ejercicio físico en pacientes con ELA. Se describen los efectos de los diferentes tipos de ejercicio, los parámetros de prescripción, los aspectos de seguridad de la intervención y las recomendaciones específicas de las guías de práctica clínica y los documentos de consenso. La mejor evidencia disponible indica que el ejercicio físico terapéutico en sus diferentes modalidades produce efectos positivos o neutros en las medidas de desenlace y no se asocia a eventos adversos serios. Conclusión. La rehabilitación basada en ejercicio físico puede ser una intervención factible, tolerable, segura y efectiva para disminuir el deterioro de la funcionalidad y la calidad de vida en pacientes con ELA.

Copyright of Revista de Investigación e Innovación en Ciencias de la Salud (RIICS) is the property of Fundacion Universitaria Maria Cano and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introducción. La esclerosis lateral amiotrófica (ELA) es la forma más común de enfermedad degenerativa de motoneurona en la edad adulta y es considerada una enfermedad terminal. Por lo mismo, el accionar del fonoaudiólogo debe considerar el respeto a los principios bioéticos básicos para garantizar una asistencia adecuada. Objetivo. Conocer aquellas consideraciones bioéticas relacionadas al manejo y estudio de personas con ELA para luego brindar una aproximación hacia el quehacer fonoaudiológico. Método. Se efectuó una búsqueda bibliográfica en las bases de datos PubMed, Scopus y SciELO. Se filtraron artículos publicados desde 2000 hasta junio de 2023 y fueron seleccionados aquellos que abordaban algún componente bioético en población con ELA. Resultados. Aspectos relacionados al uso del consentimiento informado y a la toma de decisiones compartidas destacaron como elementos esenciales para apoyar la autonomía de las personas. Conclusión. Una correcta comunicación y una toma de decisiones compartida son claves para respetar la autonomía de las personas. A su vez, la estandarización de procedimientos mediante la investigación clínica permitirá aportar al cumplimiento de los principios bioéticos de beneficencia y no maleficencia, indispensables para la práctica profesional.

Background: The Amyotrophic Lateral Sclerosis-Parkinsonism-Dementia Complex (ALS-PDC) was first described in the islands of Guam. This pathology presented its peak incidence in the 1950s. Due to the rarity of the association, we report a clinical case with this complex. The objective was to describe the nosological and pathogenic implications of these neurodegenerative disorder, since they are not frequent to find in our population. Clinical case: We present a case of Latinoamerican origin who initially manifested systemic symptoms of more than 6 years of evolution, with subsequent cognitive alterations. Later, patient began with gait disturbances and motor symptoms suggestive of parkinsonism with atypical data and data of motor neurone disease (MND). More studies were carried out and confirmed findings compatible with upper and lower motor neuron involvement. A mutation in the POLG gene was observed, related to mitochondrial depletion syndrome. Conclusion: Despite the knowledge of this association, it is an entity whose clinical diagnosis could be very difficult to achieve. In addition, molecular mechanisms have not been fully identified, the most common genes related to Parkinsonism and ALS have been excluded, and even attempts to locate the locus were made, without achieving accurate results. Unfortunately, being a neurodegenerative disease, the prognosis is fatal, with no disease-modifying treatment. [ABSTRACT FROM AUTHOR]

Copyright of Revista Foco (Interdisciplinary Studies Journal) is the property of Revista Foco and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

La Esclerosis Lateral Amiotrófica (ELA) es una enfermedad neurológica neurodegenerativa, que compromete la funcionalidad de la motoneurona superior e inferior, teniendo una evolución progresiva que puede transcurrir en un lapso de 5 años dejando una disfuncionalidad motora importante y que tiene, en todos los casos, un desenlace fatal. El diagnóstico prematuro permitirá llevar a cabo medidas de soporte, que otorgarán una mejor calidad de vida, durante la evolución de la enfermedad. Nos planteamos una revisión narrativa en la búsqueda de las ventajas y desventajas de los métodos diagnósticos de la ELA. Se utilizaron dos motores de búsqueda PubMed y BVS. Los artículos analizados fueron principalmente revisiones sistemáticas y metaanálisis, encontrándose que algunos biomarcadores en líquido cefalorraquídeo para diagnóstico de ELA, es el LCR TDP-43, por su ventaja de observarse elevado y caracterizar a la enfermedad, así como, algunas pruebas bioquímicas como los niveles elevados de creatina quinasa (CK), FBG, ferritina, TSC y disminución de TIBC juntos se destacan por su gran sensibilidad para el diagnóstico de ELA, así también los biomarcadores plasmáticos de miARN, let-7f-5p y miR-338-3p, pueden ser herramientas de diagnóstico temprano de ELA.

Copyright of Iustitia is the property of Universidad Santo Tomas, Bucaramanga and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Amyotrophic lateral sclerosis (ALS) is a chronic neurodegenerative disorder characterized by upper and lower motor neurons death, the diagnosis remains in clinical basis. Clinical criteria El Escorial for its diagnosis were published in 1994, the revised criteria in 2000 and modified by the Awaji criteria in 2008 to integrate electrophysiological data with the clinical examination. These criteria are complex to apply and have difficulties to determine progression through ALS categories. Recent advances in genetics, biomarkers such as light chain neurofilament (NfL), cytokines, neuroimaging, and neurophysiological probes of upper motor neuron dysfunction are not included in the diagnostic criteria. In 2019, new and simple ALS diagnostic criteria (Gold Coast criteria) have been introduced along with genetic testing and serum and cerebrospinal fluid NfL levels. Early diagnosis for initiating disease-modifying drugs and care in a multidisciplinary ALS clinic will improve quality of life and survival in ALS patients. In this article, I consider that it is an urgency to facilitate an early recognition of possible ALS among primary care physicians as well as to foster of urgency among neurologist to accelerate the diagnostic process of ALS to protect viable motor neurons and slow down the process of neurodegeneration. [ABSTRACT FROM AUTHOR]

Copyright of Revista Científica de Enfermería (2071-5080) is the property of Colegio de Enfermeros del Peru and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

La esclerosis lateral amiotrófica (ELA) es una enfermedad degenerativa y progresiva que afecta a las motoneuronas del sistema nervioso central. Se caracteriza por un comienzo insidioso, habitualmente en individuos de mediana edad de sexo masculino. Los síntomas iniciales son: debilidad y atrofia muscular distal, fasciculaciones, hiperreflexia, espasticidad, signo de Babinski, y alteraciones progresivas de la deglución y la fonación; su pronóstico es reservado. El diagnóstico diferencial más importante es con la mielopatía espondilótica cervical; los estudios neurofisiológicos juegan un papel importante en esta diferenciación y las imágenes por resonancia magnética confirman este diagnóstico. Para demostrar la importancia de los estudios neurofisiológicos en la realización del diagnóstico diferencial entre la esclerosis lateral amiotrófica y la mielopatía espondilótica cervical, se presenta un caso clínico con diagnóstico presuntivo de esclerosis lateral amiotrófica, sin embargo los resultados de los estudios neurofisiológicos lo pusieron en duda, sugiriendo compresión de la médula espinal cervical, sin poder precisar la etiología exacta. Las imágenes de resonancia magnética confirmaron la existencia de una mielopatía espondilítica cervical severa. Se concluye que los estudios neurofisiológicos juegan un papel importante en la realización del diagnóstico diferencial entre las dos entidades y que los estudios de imagen facilitan la identificación de las lesiones espondilóticas.

Introduction: Amyotrophic lateral sclerosis (ALS) is a degenerative disease of unknown origin that affects the motor neurons. It has a rapid, fatal course., Method: For this study, an initial questionnaire of eleven items was developed by experts in the field, who evaluated the suitability and relevance of the items., Results: The questionnaire was then applied to a pilot group of 22 patients diagnosed with ALS. Confirmatory factor analysis, based on estimating maximum likelihood, confirmed the three domains detected in the exploratory factor analysis. The reliability of the scale was tested using Cronbach's α (0.801) and the Kaiser-Meyer-Olkin test (0.770) confirmed the construct validity., Conclusions: The DEREDELA questionnaire is valid, in terms of its content, for monitoring the neurological dysphagia and respiratory deterioration suffered by patients diagnosed with ALS., (Copyright © 2024. Published by Elsevier España, S.L.U.)

Introduction: Ultrasound changes in the cross-sectional area of the median nerve (CSAmn) could be of interest as biomarkers in patients with amyotrophic lateral sclerosis (ALS)., Methods: Eighty-four ALS patients (51 men [60.7%]; mean 62.0 [SD 11.46] years old) and forty-six controls (27 men [58.7%]; mean 59.9 [SD 8.08] years old) of two different cohorts were recruited between September 2013 and February 2018. The CSAmn was measured bilaterally in each cohort, by two different examiners with two different ultrasound machines (one in each cohort). Its association with clinical variables (disease duration, muscle strength, disability, progression rate and tracheostomy-free survival) was assessed., Results: The CSAmn was smaller in patients than in controls, and the study cohort did not influence its values. A mild correlation between the strength of the wrist flexor and the CSAmn was found. In the multivariable analysis, the probability of this association being true was 90%. In the cox regression, both a faster progression rate and a larger CSAmn independently predicted poor survival (HR=4.29, [Cr.I95%: 2.71-6.80], p<0.001; and HR=1.14, [Cr.I95%: 1.03-1.25], p=0.01), after adjusting by age, body mass index, bulbar onset, and diagnostic delay., Conclusions: The CSAmn is an easy to assess biomarker that seems reliable and reproducible. Our data also suggest that it could act as a progression and prognostic biomarker in ALS patients. Longitudinal studies with repeated measures are warranted to confirm its usefulness in the clinical practice., (Copyright © 2022 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: Amyotrophic lateral sclerosis (ALS) is an insidious, clinically heterogeneous neurodegenerative disease associated with a diagnostic delay of approximately 12 months. No study conducted to date has analysed the diagnostic pathway in Spain. Methods: We gathered data on variables related to the diagnostic pathway and delay for patients diagnosed with ALS between October 2013 and July 2017. Results: The study included 143 patients with ALS (57% men; 68% spinal onset). Patients were diagnosed in public centres in 86% of cases and in private centres in 14%. The mean diagnostic delay was 13.1 months (median 11.7). Patients were examined by neurologists a mean time of 7.9 months after symptom onset, with diagnosis being made 5.2 months later. Half of all patients underwent unnecessary diagnostic tests and multiple electrophysiological studies before diagnosis was established. Diagnostic delay was longer in cases of spinal onset (P = .008) due to onset of the disease in the lower limbs. No differences were found between the public and private healthcare systems (P = .897). Conclusions: The diagnostic delay in ALS in Spain is similar to that of neighbouring countries and seems to depend on disease-related factors, not on the healthcare system. Patients with lower-limb onset ALS constitute the greatest diagnostic challenge. Misdiagnosis is frequent, and partly attributable to an incorrect approach or erroneous interpretation of electrophysiological studies. Specific training programmes for neurologists and general neurophysiologists and early referral to reference centres may help to reduce diagnostic delay. Resumen: Introducción: La esclerosis lateral amiotrófica (ELA) es una enfermedad insidiosa y clínicamente heterogénea, lo que resulta en un retraso diagnóstico de unos 12 meses. En España el trayecto diagnóstico no ha sido analizado. Métodos: Se recogieron variables relativas al trayecto y retraso diagnóstico de pacientes diagnosticados de ELA entre octubre del 2013 y julio del 2017. Resultados: Se incluyó a 143 pacientes con ELA (57% varones, 68% de inicio espinal). El 86% de ellos fueron estudiados en centros públicos y un 14% en privados. El retraso diagnóstico medio fue de 13.1 meses (mediana 11.7). El paciente tardó de media 7.9 meses en llegar al neurólogo y este, 5,2 meses más en diagnosticarlo. En la mitad de los pacientes se realizaron pruebas innecesarias y más de un estudio electrofisiológico para llegar al diagnóstico. El retraso diagnóstico fue mayor en los casos espinales (p = 0,008), atribuible a los pacientes cuyos síntomas se iniciaron en miembros inferiores, pero sin diferencias entre el sistema público y privado (p = 0,897). Conclusiones: El retraso diagnóstico de la ELA en nuestro medio es similar al de países de nuestro entorno y parece determinado por factores propios de la enfermedad e independiente del sistema sanitario. Las formas de inicio en miembros inferiores constituyen el mayor reto. Los errores diagnósticos del neurólogo son frecuentes y en parte atribuibles a una mala orientación o interpretación del estudio electrofisiológico. La formación específica del neurólogo y neurofisiólogo general y la derivación precoz a centros de referencia podrían ayudar a reducir la demora.

Introduction: Introduction: amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease. Its symptoms include dysphagia that may make it necessary to place a percutaneous endoscopic gastrostomy (PEG) for feeding. The administration of drugs by PEG can obstruct it, decrease the effectiveness of treatment, and increase the risk of toxicity by altering the original pharmaceutical form. Objective: to describe and analyze the degree of adequacy of the prescription of drugs administered by PEG in patients with ALS and with enteral nutrition (EN). Material and methods: the prescription of pharmacological treatment for patients with ALS who were admitted for placement of a PEG was reviewed. The degree of adequacy of the prescribed drugs was analyzed according to criteria of loss of efficacy, toxicity, risk for handler, and compatibility with EN by consulting the available scientific evidence. Results: the medical prescriptions of the treatments of 34 patients were reviewed, with a total of 307 medications (median of 9 drugs per patient, range 2-17). Via PEG 267 oral medications (median 8 per patient, range 2-15) were prescribed; 81.65 % were solid forms, and the pharmaceutical form was modified in 43 %, due to the risk of catheter occlusion, toxicity or loss of efficacy, affecting 97 % of the patients. Conclusions: patients with ALS and PEG are at risk of presenting safety problems and loss of treatment efficacy related to alteration of the original pharmaceutical form and the interaction with EN. [ABSTRACT FROM AUTHOR]

Copyright of Medicina (Buenos Aires) is the property of Medicina (Buenos Aires) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Revista Científica de Enfermería (2071-5080) is the property of Colegio de Enfermeros del Peru and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Medicina (Buenos Aires) is the property of Medicina (Buenos Aires) and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: The aim of this study was to determine the behaviour of ultrasound biomarkers of fascicle density and muscle strength in patients with amyotrophic lateral sclerosis (ALS)., Methods: We conducted an observational, cross-sectional pilot study of 14 patients with ALS (28.6% women) and 14 controls. Bilateral cross-sectional ultrasound scans were performed in the abductor pollicis brevis (APB) and tibialis anterior (TA) muscles, with recording of muscle thickness (MT) at rest and in contraction, and the difference in thickness. In the median, sciatic, and common peroneal nerves, we analysed the cross-sectional area (CSA), number of fascicles (NF) and fascicle density (FD). Analyses were nested by laterality., Results: Intra- and interrater agreement regarding NF was very good, with a minimum detectable error of < 0.7%. In patients with ALS, MT was lower in the APB both at rest (P = .003; g-Hedges = 1.03) and in contraction (P = .017; g-Hedges = 0.78) and in TA at rest (P = .002; g-Hedges = 0.15) and in contraction (P = .001; g-Hedges = 0.46), with lower thickening capacity. In the nerves, patients displayed lower CSA, with lower NF and higher FD. Significant correlations were found between MT of the ABP and Medical Research Council (MRC) scores for muscle strength (r = 0.34; r 2  = 12%; P = .011) and with revised ALS Functional Rating Scale scores (r = 0.44; r 2  = 19%; P < .001). The difference in TA thickening correlated with MRC scores (r = 0.30; r 2  = 15%; P = .003) and with revised ALS Functional Rating Scale scores (r = 0.26; r 2  = 7%; P = .049). NF in the sciatic nerve showed a significant correlation with MRC scores (r = 0.35; r 2  = 12%; P = .008)., Conclusion: MT measurements derived from dynamic testing together with NF and FD may be useful biomarkers for monitoring patients with ALS and establishing a prognosis., (Copyright © 2021 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Amyotrophic lateral sclerosis (ALS) is a degenerative neurological disease that affects the pyramidal pathway, along its first and second motor neurons. In the United States of America, ALS is better known as Lou Gehrig's disease, alluding to the Yankees baseball player who died in 1941 from this condition. Etymologically, Sclerosis means hardening and refers to the state of the spinal cord in the advanced stages of the disease. Lateral means "to the side" and reveals the location of the spinal cord damage. Finally, the term amyotrophic means "without muscle nutrition" and refers to the loss of signals that the nerves normally send to the muscles. The etiology of this disorder is unknown, it is considered sporadic in 90-95% of cases and with a familial tendency in 5%. Survival at diagnosis is around 20% at 3-5 years. [ABSTRACT FROM AUTHOR]

Copyright of PODIUM- Revista de Ciencia y Tecnologia en la Cultura Física is the property of Universidad de Pinar del Rio and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Amyotrophic lateral sclerosis (ALS) is a progressive disease characterized by degeneration of upper and lower motor neurons. Time from symptom onset to confirmed diagnosis has been reported from 8 to 15 months in ALS. Objectives: To describe the frequency of the split hand phenomenon and propose it as an early biomarker for ALS diagnosis. Methods: A retrospective, analytical, descriptive, and single-center observational study was performed. The split hand ratio was determined by dividing distal abductor pollicis brevis/abductor digit minimi compound muscle action potentials; a result < 0.6 was considered present. Results: Fifty-four patients with ALS diagnosis were included in the study. The split hand ratio was identified in 61.5% of patients with definite ALS, in 68.7% with probable ALS, 80% with possible ALS, and in 50% with suspected ALS. The split hand phenomenon was identified in 60% of patients within 12 months of symptom onset. Conclusion: We provide evidence for an additional neurophysiological tool that helps early diagnosis of ALS. [ABSTRACT FROM AUTHOR]

Introduction: In recent months, the emergence of SARS-CoV-2 as a global pandemic has caused a devastating impact on health worldwide. Health systems have been overwhelmed, and the follow-up of those patients who require continuous monitoring and evaluation, such as Amyotrophic Lateral Sclerosis (ALS), have been negatively affected. Material and method: A search of the current literature has been carried out to determine the impact of the Covid-19 pandemic on the quality of life of patients diagnosed with Amyotrophic Lateral Sclerosis and their family. Results:The diagnosis, treatment, follow-up and home care of these patients have been modified, adapting them as much as possible to the technology and available resources to try to minimize the loss of quality of life of patients diagnosed with Sclerosis Amyotrophic lateral and ensuring comprehensive care. Conclusion: Despite numerous efforts and advances in research, both in the case of ALS and COVID-19, there is still a long way to go. [ABSTRACT FROM AUTHOR]

Copyright of PODIUM- Revista de Ciencia y Tecnologia en la Cultura Física is the property of Universidad de Pinar del Rio and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease whose aetiology is unknown. It is characterised by upper and lower motor neuron degeneration. Approximately 90% of cases of ALS are sporadic, whereas the other 10% are familial. Regardless of whether the case is familial o sporadic, patients will develop progressive weakness, muscle atrophy with spasticity, and muscle contractures. Life expectancy of these patients is generally 2 to 5 years after diagnosis., Development: In vivo models have helped to clarify the aetiology and pathogenesis of ALS, as well as the mechanisms of the disease. However, as these mechanisms are not yet fully understood, experimental models are essential to the continued study of the pathogenesis of ALS, as well as in the search for possible therapeutic targets. Although 90% of cases are sporadic, most of the models used to study ALS pathogenesis are based on genetic mutations associated with the familial form of the disease; the pathogenesis of sporadic ALS remains unknown. Therefore, it would be critical to establish models based on the sporadic form., Conclusions: This article reviews the main genetic and sporadic experimental models used in the study of this disease, focusing on those that have been developed using rodents., (Copyright © 2023 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Introduction: Amyotrophic lateral sclerosis is a central nervous system disease with a progressive degeneration of superior and inferior motor neurons, causing several complications, specially respiratory problems. Respiratory physiotherapy has a great importance in this disease, since it can improve considerably the pulmonary function and gases changes, increasing the air volumes and decreasing the respiratory work, facilitating then the elimination of bronchial secretions. Thus, the aim of this study is to review the scientific literature in the last 5 years about the physiotherapy in ALS patients. Development: A systematic review was carried out, searching in PubMed and Web of Science with the terms "amyotrophic lateral sclerosis" y "respiratory therapy". 217 results were obtained, and after the inclusion and exclusion criteria 19 studies were selected. All works analyzed carried out different interventions in respiratory physiotherapy such as VNI, phrenic stimulation, Cough-Assist or exercise therapy. Conclusions: Respiratory physiotherapy seems to be effective for patients with ALS, except from phrenic stimulation. [ABSTRACT FROM AUTHOR]

Copyright of Revista Habanera de Ciencias Médicas is the property of Universidad de Ciencias Medicas de La Habana and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder characterized by rapid deterioration and selective death of motor neurons (MNs) in central nervous system. There is no effective therapy for ALS patients. Riluzole, only slightly delays disease progression. Edaravone was FDA approved as an intravenous therapy, however, this drug is expensive and has been applied in few cases in our country. There is compelling evidence patients who attend an ALS multidisciplinary clinic experience improved survival. An ALS multidisciplinary clinic was launched in our institution and adapted to our healthcare system. It is described the processes involved in the evaluation of patients in an all day long session where ALS patients are evaluated by several professionals from different specialties specially trained in the needs of subjects living with this disorder. We consider that this model of healthcare for patients with ALS may be useful in another healthcare institutions in our country. [ABSTRACT FROM AUTHOR]

Copyright of Revista de Pesquisa: Cuidado é Fundamental is the property of Revista de Pesquisa: Cuidado e Fundamental Online and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

La esclerosis lateral amiotrófica (ELA) es una enfermedad neurodegenerativa en la que fallan las neuronas llamadas “motoneuronas”, lo que provoca una paralización progresiva de los músculos, que afecta a la movilidad e impide la adecuada deglución, habla y respiración, entre otros síntomas. Además, no tiene cura; solo se pueden paliar sus consecuencias para intentar mejorar la calidad de vida de las personas con que padecen la enfermedad, desde distintos ámbitos sociosanitarios. El presente artículo aborda la percepción de las estrategias de afrontamiento de las y los profesionales que atienden a las personas afectadas, su formación para dicho fin y su satisfacción profesional. Así, a partir de veintidós entrevistas semiestructuradas realizadas a profesionales sociosanitarios de la provincia de Valencia (España), se ha realizado un análisis de contenido cualitativo de la información obtenida. El estudio ha identificado distintas estrategias de afrontamiento que utilizan los profesionales (aceptación, humor y separación, entre otras) y cómo estas varían según la función que los profesionales desempeñan en el tratamiento de la enfermedad. Asimismo, se ha evidenciado que los entrevistados tienen importantes carencias en formación para el afrontamiento, la comunicación con los enfermos y el proceso de duelo.

Amyotrophic Lateral Sclerosis (ALS) is a fatal, progressive neurodegenerative disease affecting upper and lower motor neurons of the central nervous system that is associated to glial reactivity. The pathogenesis of this disease is not entirely clear. Different mechanisms have been postulated, including alterations in RNA processing, protein metabolism, axonal transport and mitochondrial function, increased oxidative stress and excitotoxicity. Astrocytes exhibit processes surrounding the synapse, where glutamate transporters are located to uptake the excess of neurotransmitter during synaptic activity. Alterations in this mechanism have been found in ALS and have highlighted the role of glia in the progression of ALS. Glutamate acts on two receptor families: NMDA and non-NMDA. There is evidence that links glutamate transporters dysfunction to the pathogenesis of the disease. In addition, it has been proven that alteration in the function and availability of the glutamate transporter EAAT2 / GLT1contributes to the increase of extracellular glutamate concentration. In this work, we aim to review the literature on the role of astrocytes and the glutamate transporter EAAT2 / GLT1 in the pathogenesis of ALS, to identify unsolved questions that may guide further research to improve the treatment of these patients.

Amyotrophic lateral sclerosis (ALS) is a progressive and fatal neurodegenerative disease that inevitably affects the respiratory system, and part of its treatment focuses on improving symptoms and minimizing its respiratory complications which are the main cause of death; thus, the purpose of the following review is to describe the pulmonary involvement of ALS, to propose diagnostic/monitoring guidelines, and to expose its therapeutic options. The alveolar hypoventilation syndrome and the poor management of bronchial secretion are the main respiratory difficulties secondary to ALS and they are due to the weakness of the muscles that generate respiration; in this way, the periodic assessment of respiratory function is imperative for monitoring and detecting these complications in early stages. The care of patients with ALS should be multidisciplinary and ideally should be performed in specialized centers; non-invasive mechanical ventilation and assisted cough (manual or mechanical) are the two most cost/effective techniques to treat alveolar hypoventilation and poor secretion management to increase survival, improve symptoms, and increase quality of life. [ABSTRACT FROM AUTHOR]

Stephen Hawking, autor de las teorías más fundamentales sobre el comportamiento de los agujeros negros, entreotras consideraciones sobre el tiempo y el espacio; padece de esclerosis lateral amiotrófica, la cual le fue diagnosticadadurante su último año en Oxford, tal y como se describe en la película La teoría del todo (2014) dirigida por James Marsh y producida por Anthony McCarten. La esclerosis lateral amiotrófica (ELA) o enfermedad de Lou Gehrig es una alteración neurodegenerativa progresiva que ocasiona debilitamiento gradual de la musculatura voluntaria.Se caracteriza por una pérdida progresiva de la moto?neurona de la corteza motora, tronco del encéfalo y médulaespinal.

La vía clínica de la Esclerosis Lateral Amiotrófica (ELA) se desarrolló con el objetivo de ofrecer una atención integral y multidisciplinar de los pacientes con esta enfermedad, con el fin de favorecer y agilizar el diagnóstico y el tratamiento, protocolizando las actuaciones y coordinando las distintas especialidades médicas implicadas en el manejo de esta enfermedad. El modelo en el que se basa es el de gestión de caso, gestión que se lleva a cabo desde la Unidad de Medicina Paliativa. Desde esta Unidad, una enfermera es la responsable de la gestión coordinada de cada consulta y mantiene una comunicación fluida con el paciente y la familia, dando respuesta a todas las necesidades que van surgiendo a lo largo del proceso. Esto se recoge en un documento de consenso: la Vía Clínica de la ELA en Organización Sanitaria Integrada de Alava (OSI-Araba) Con una periodicidad mensual, se realiza la consulta multidisciplinar de la Unidad de ELA. En ella participan los Servicios de Neurología, Neumología, Endocrinología y Nutrición, Rehabilitación y Cuidados Paliativos. Ubicada en Consultas Externas del Hospital Universitario de Araba, se realiza en un espacio, físico y temporal único, para favorecer la comodidad de pacientes y familiares. También se realizan reuniones multidisciplinares e interdisciplinares en la que médicos, enfermeros, fisoterapeutas y psicólogos consensúan la atención de cada paciente, de forma individualizda, sino también se realizar mejoras contínua en la atención mediante la actualización y revisión de la vía clínica. La Unidad de Medicina Paliativa coordina y da soporte a lo largo de todo el proceso (y no sólo en las etapas finales). Organiza y gestiona el seguimiento multidisciplinar y multiservicio y proporciona acceso y atención directa a los pacientes y sus familias. Además, y de una forma prioritaria, se mantiene informado al equipo de Atención Primaria para evitar brechas de continuidad asistencial. Para ello existe un equipo de soporte que acompaña a los equipos en las etapas finales de la enfermedad, coordinado entonces su atención desde el domicilio.

Copyright of Revista Habanera de Ciencias Médicas is the property of Universidad de Ciencias Medicas de La Habana and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Introduction: Life expectancy after diagnosis Amyotrophic lateral sclerosis (ALS) is 2 to 5 years, and approximately 20% of patients live more than 5 years. Only one drug, Riluzol, has been approved for the treatment of ALS, which extends the patient's lifespan by a few months, therefore, there is a need to develop more effective therapies for this disorder. Stem cell therapy is a promising potential treatment option for ALS, for this reason the objective of this review is to investigate the results of treatment with stem cells in patients with ALS and thus open new paths to its effectiveness. Material and methods: During the months of March and April 2017, a search was made of the Medline, Web of Science and Scopus databases. Results: 13 valid studies have been selected for the review and we found various surgical procedures were used to administer stem cells. Among the study variables, the most important measures are bulbar function, motor function, respiratory function, muscle strength, respiratory volume and flow and spasticity. Conclusions: Beneficial results were obtained, since there were significant improvements in the measured variables and there was no apparent progression of the disease related to the surgical procedure. It can be concluded that treatment with stem cells in patients with ALS is safe and viable, however, studies should continue to be carried out that provide greater reliability in terms of the sample and effects obtained. [ABSTRACT FROM AUTHOR]

Recreación de la vida de Stephen Hawking (Eddie Redmayne) desde 1963, cuando se encontraba en Cambridge para realizar su tesis doctoral, hasta que fue nombrado caballero por Isabel II y se vislumbra el fin de su primer matrimonio. En 1963 conoce a la que sería su pri? mera mujer, contrapunto femenino de la historia, y se le manifiesta la enfermedad. Este proceso, que comenzó tempranamente, no acabó con su existencia en poco tiem? po, como suele ser habitual, sino que ha sobrevivido.Premios: Óscar al Mejor Actor (Eddie Redmayne) y nomi? nada a la Mejor Película, Mejor Actriz (Felicity Jones), Mejor Guión Adaptado y Mejor Banda Sonora (2015).

Objective: To determine if there is a relationship between environmental exposure to pesticides and the prevalence of Amyotrophic Lateral Sclerosis (ALS) in Andalusia., Method: We carried out a case-control study using the logistic regression method to verify the relationship between the prevalence of ALS in the area exposed to pesticides versus the unexposed area, through the Odds Ratio statistical test., Results: The study population consisted of 519 individuals diagnosed with ALS between January 2016 and December 2018 according to the CMBD (Minimum Basic Data Set) as cases. In the control group, we have 8,384,083 individuals obtained from data from the National Institute of Statistics (INE). The Odds Ratio (OR) was used as a measure of association between cases and controls, obtaining an OR between 0.76 and 1.08 for the confidence interval of the CI (95%)., Conclusions: Despite the existence of various studies that suggest a possible association between environmental exposure to pesticides and the risk of Amyotrophic Lateral Sclerosis, our analysis of the Andalusian population did not find significant evidence of this association., (Copyright © 2021 Sociedad Española de Neurología. Published by Elsevier España, S.L.U. All rights reserved.)

Background: Amyotrophic lateral sclerosis (ALS), neurodegenerative disease that affects the upper and lower motor neurons. With an average diagnostic confirmation time of 15,6 months and a 3-year survival from diagnosis of 50%. There is poor knowledge of initial symptoms, causing diagnostic delay. Objectives: To determine the diagnostic confirmation time in patients with ALS of HCHM between 2006-2018, Identify initial symptoms, determine overall survival time and by sex. Methods: Descriptive analytical longitudinal study of diagnosis time; primary symptoms: i) motor disorders; ii) language and / or swallowing disorders; and iii) both; and survival of patients with ALS from HCHM between 2006-2018. Results: Between 2016-2018, 19 patients with ALS were diagnosed, 47% women (9) and 53% men (10); average age at diagnosis of 61.44 and 59.30, respectively. The diagnostic confirmation time in women was 17.56 (ES ± 0.71) weeks and in men 34.80 (ES ± 0.68) weeks. In women, the frequency of primary diagnoses: 78% motor disorders; 11% language and / or swallowing disorders; 11% both, in men: 30% motor disorders; 20% language and / or swallowing disorders; 50% both. The mean overall survival was 6.5 years. Conclusions: There is a significant difference between initial symptoms and according to sex, with motor predominance in women and mixed in men. There are no significant differences in diagnostic times or survival in both groups, it is emphasized that 50% of women survived at 7.2 years and 50% of men at 6.3 years. An initial clinical profile is not differentiated. [ABSTRACT FROM AUTHOR]