RESUMEN
BACKGROUND: Myasthenia gravis is largely a treatable disease, but it can result in significant morbidity and even mortality, which can usually be avoided, or at least mitigated, with timely diagnosis and appropriate treatment of the disease. Objective: this review aims to summarize the main practical aspects of the diagnostic approach, treatment and care of myasthenic patients. METHODS: The authors performed a non-systematic critical review summarizing the main practical aspects of myasthenia gravis. RESULTS: Most patients with myasthenia have autoantibodies targeted at acetylcholine receptors or, less commonly, muscle-specific kinase - MuSK. Electrophysiology plays an important role in the diagnosis of neuromuscular junction dysfunction. The central clinical manifestation of myasthenia gravis is fatigable muscle weakness, which can affect eye, bulbar, respiratory, and limb muscles. With rare exceptions, patients have a good response to symptomatic treatment, but corticosteroids and/or immunosuppressants are usually also necessary to obtain good control of the manifestations of the disease. CONCLUSION: Knowledge of the peculiar aspects of their clinical and electrophysiological presentations is important for the diagnosis. Likewise, specific treatment and response time to each drug are crucial for proper care.
Asunto(s)
Miastenia Gravis , Proteínas Tirosina Quinasas Receptoras , Autoanticuerpos , Humanos , Debilidad Muscular , Miastenia Gravis/diagnóstico , Miastenia Gravis/terapia , Unión Neuromuscular , Receptores ColinérgicosRESUMEN
BACKGROUND: Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction that can be exacerbated by many viral infections, including COVID-19. The management of MG exacerbations is challenging in this scenario. We report 8 cases of MG exacerbation or myasthenic crisis associated with COVID-19 and discuss prognosis and treatment based on a literature review. RESULTS: Most patients were female (7/8), with an average age of 47.1 years. Treatment was immunoglobulin (IVIG) in 3 patients, plasma exchange (PLEX) in 2 patients, and adjustment of baseline drugs in 3. In-hospital mortality was 25% and 37.5% in 2-month follow-up. DISCUSSION: This is the largest case series of MG exacerbation or myasthenic crisis due to COVID-19 to this date. Mortality was considerably higher than in myasthenic crisis of other etiologies. Previous treatment for MG or acute exacerbation treatment did not seem to interfere with prognosis, although sample size was too small to draw definitive conclusions. Further studies are needed to understand the safety and effectiveness of interventions in this setting, particularly of PLEX, IVIG, rituximab, and tocilizumab.
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COVID-19 , Miastenia Gravis , COVID-19/complicaciones , Femenino , Humanos , Persona de Mediana Edad , Miastenia Gravis/complicaciones , Miastenia Gravis/terapia , Intercambio Plasmático , SARS-CoV-2RESUMEN
BACKGROUND: Recent medical advances have enabled the control of neurological symptoms and increased survival of patients with myasthenia gravis (MG). However, MG has many veiled consequences that may be underestimated by neurologists. Our aim was to clarify the social, professional, and neuropsychiatric issues of MG patients. METHODS: We carried out a cross-sectional cohort study with MG patients from a university-affiliated referral hospital. We registered clinical and sociodemographic data, and patients were classified according the MGFA classification. Clinical severity was assessed with Myasthenia Gravis Composite (MGC) scale. Trained and blind investigators analyzed social and professional outcomes. Neuropsychiatric symptoms were evaluated with the Hospital Anxiety and Depression Scale (HADS) and the social support with the Multidimensional Scale of Perceived Social Support (MSPSS). RESULTS: We enrolled 49 patients with MG. The mean age was 45.3 ± 18.1 years and 39 (79.6%) were women. There were 19 (38.8%) patients who become unemployed, 23 (46.9%) who had to retire prematurely, 31 (63.3%) that reported a significant reduction in work performance, and 23 (46.9%) who had a reduction in monthly income after the diagnosis of MG. Only 16 (32.6%) received any financial support and 24 (48.9%) patients had the perception of receiving a satisfactory social support. The practice of physical activity is a habit in only 10 patients (20.4%). Neuropsychiatric symptoms were present in 26 (53.1%) patients. CONCLUSION: Patients with MG are vulnerable to social, professional, and mental disadvantages. Therapeutic success in MG goes beyond symptom relief and inevitably depends on a personalized approach to the patient.
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Miastenia Gravis , Calidad de Vida , Adulto , Estudios de Cohortes , Estudios Transversales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/epidemiología , Miastenia Gravis/terapia , Apoyo SocialRESUMEN
Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the efficacy and results of different treatments. Although there are differences between the juvenile myasthenia gravis and that of the adult, the data provided by some researches in adults in the treatment of juvenile myasthenia gravis have been used. The different therapeutic options will be evaluated, with the different evidences that sustain it and a treatment algorithm will be elaborated keeping always in mind that each patient offers us different challenges.
La miastenia gravis juvenil es una enfermedad autoimmune poco frecuente, por lo que ha sido difícil recopilar datos de estudios controlados aleatorizados prospectivos para evaluar la eficacia y los resultados de distintos tratamientos. Si bien hay diferencias entre la miastenia gravis juvenil y la del adulto, se han utilizado los datos aportados por algunas investigaciones en adultos en el tratamiento de la miastenia gravis juvenil. Se evaluarán las distintas opciones terapéuticas, con las distintas evidencias que lo sostienen y se elaborará un algoritmo de tratamiento teniendo siempre presente que cada paciente nos ofrece distintos desafíos.
Asunto(s)
Miastenia Gravis/terapia , Niño , Inhibidores de la Colinesterasa/uso terapéutico , Humanos , Inmunosupresores/clasificación , Inmunosupresores/uso terapéutico , Miastenia Gravis/cirugía , Esteroides/uso terapéutico , TimectomíaRESUMEN
La miastenia gravis juvenil es una enfermedad autoimmune poco frecuente, por lo que ha sido difícil recopilar datos de estudios controlados aleatorizados prospectivos para evaluar la eficacia y los resultados de distintos tratamientos. Si bien hay diferencias entre la miastenia gravis juvenil y la del adulto, se han utilizado los datos aportados por algunas investigaciones en adultos en el tratamiento de la miastenia gravis juvenil. Se evaluarán las distintas opciones terapéuticas, con las distintas evidencias que lo sostienen y se elaborará un algoritmo de tratamiento teniendo siempre presente que cada paciente nos ofrece distintos desafíos.
Juvenile myasthenia gravis is a rare autoimmune disease, which has made it difficult to collect data from prospective randomized controlled trials to evaluate the efficacy and results of different treatments. Although there are differences between the juvenile myasthenia gravis and that of the adult, the data provided by some researches in adults in the treatment of juvenile myasthenia gravis have been used. The different therapeutic options will be evaluated, with the different evidences that sustain it and a treatment algorithm will be elaborated keeping always in mind that each patient offers us different challenges.
Asunto(s)
Humanos , Niño , Miastenia Gravis/terapia , Esteroides/uso terapéutico , Timectomía , Inhibidores de la Colinesterasa/uso terapéutico , Inmunosupresores/clasificación , Inmunosupresores/uso terapéutico , Miastenia Gravis/cirugíaRESUMEN
Myasthenia gravis (MG) is an autoimmune disease that can mimic a variety of symptoms leading to a delay in diagnosis and treatment. We report the case of 48 year-old woman, in whom initial presentation of MG with predominance of bulbar symptoms was mistaken for thyroid disease complications. Subsequently, correct diagnosis and optimal management resulted on significant improvement of her functional state. We discuss the importance of considering MG as one of the potential differential diagnoses among cases of new onset or recurrent unexplained bulbar symptoms.
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Miastenia Gravis/diagnóstico , Tiroiditis Subaguda/diagnóstico , Errores Diagnósticos , Femenino , Humanos , Persona de Mediana Edad , Miastenia Gravis/terapia , Índice de Severidad de la EnfermedadRESUMEN
Introdução: A ptose palpebral é uma afecção comum na prática clínica na qual uma perfeita avaliação torna-se mandatória. Definimos ptose quando a margem palpebral encontra-se abaixo de 2 mm da junção córneo escleral e pode ser classificada em leve, moderada e grave. Existem inúmeras técnicas de reparo e a escolha dependerá da classificação da função do músculo levantador. Métodos: Foram analisados de forma prospectiva, no período de março de 2013 a maio de 2015, quatorze (n = 14) pacientes submetidos ao tratamento cirúrgico de ptose palpebral moderada e grave (n = 21). Inúmeros fatores foram estudados, tais como grau de ptose e função do músculo elevador da pálpebra, tipo de técnica de reparo, complicações imediatas e tardias, etc. Resultados: Quatorze pacientes foram opera-dos, totalizando 21 pálpebras, sendo que, 85% foram de etiologia adquirida e 15% congênita. Com relação ao grau de ptose, 64,3% (n = 9) foram moderadas e 35,7% (n = 5) graves. No que tange à função do músculo levantador, encontramos função boa 28,5% (n = 4), moderada 28,5% (n = 4) e pobre 43% (n = 6). Em relação às com-plicações, 2 casos de hiperemia conjutival e um caso de edema. Obtivemos um alto índice de satisfação com 85,7% (n = 12), com baixas taxas de complicações. Conclusão: A ptose palpebral é uma enfermidade comum na prática clínica e exige por parte do cirurgião um perfeito conhecimento anatômico da delicada estrutura palpebral e também de sua fisiopatologia. Uma perfeita avaliação desse paciente torna-se mandatória para o emprego do tratamento mais adequado.
Introduction: Eyelid ptosis is a common condition in clinical practice for which a complete evaluation is mandatory. Ptosis is defined when the eyelid margin is 2 mm below the corneoscleral junction and can be classified as mild, moderate, and severe. There are numerous repair techniques, and the choice will depend on the classification of the function of the levator muscle. Methods: We evaluated prospectively, from March 2013 to May 2015, 14 patients who underwent surgical treatment of moderate and severe ptosis (n = 21). Several factors were studied, such as degree of ptosis and function of the eyelid levator muscle, type of repair technique, and immediate and late complications. Results: Fourteen patients (21 eyelids) underwent operation. The etiology was acquired in 85% of the cases and congenital in 15%. With respect to the degree of ptosis, 64.3% (n = 9) of the cases were moderate and 35.7% (n = 5) were severe. With respect to the muscle function of the levator, good, moderate, and poor functions were observed in 28.5% (n = 4), 28.5% (n = 4), and 43% (n = 6) of the cases, respectively. With regard to complications, 2 cases of conjunctival hyperemia and one case of edema were observed. We obtained a high satisfaction rate of 85.7% (n = 12), with low complication rates. Conclusion: Eyelid ptosis is a common presentation in clinical practice and requires on the part of the surgeon a detailed anatomical knowledge of the delicate structure of the eyelid and its pathophysiology. A complete evaluation of these patients is mandatory for the employment of the most appropriate treatment.
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Humanos , Masculino , Femenino , Niño , Adolescente , Adulto , Persona de Mediana Edad , Anciano , Historia del Siglo XXI , Procedimientos Quirúrgicos Oftalmológicos , Blefaroptosis , Estudios Prospectivos , Blefarofimosis , Enfermedades de los Párpados , Fascia Lata , Miastenia Gravis , Procedimientos Quirúrgicos Oftalmológicos/métodos , Procedimientos Quirúrgicos Oftalmológicos/rehabilitación , Blefaroptosis/cirugía , Blefaroptosis/rehabilitación , Blefarofimosis/cirugía , Blefarofimosis/rehabilitación , Enfermedades de los Párpados/cirugía , Enfermedades de los Párpados/rehabilitación , Fascia Lata/anatomía & histología , Fascia Lata/anomalías , Fascia Lata/cirugía , Miastenia Gravis/cirugía , Miastenia Gravis/terapiaRESUMEN
The relationship between myasthenia gravis (MG) and epilepsy has been rarely reported. As consequence, there are no specific guidelines for the management of these conditions when they mutually occur. We reported on three patients in whom epilepsy and MG are coexisting, but in different clinical settings. Two patients were treated with antiepileptic drugs which improved their symptoms. One patient has controlled the seizures after a successful anterior temporal lobectomy with no appreciable consequences to her MG. We discuss the difficulties in the management of epilepsy in patients with MG. In addition, we report on the first epileptic surgery in a MG patient, indicating that this surgical procedure as a safe option for the treatment of intractable epilepsy in patients with MG.
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Lobectomía Temporal Anterior , Anticonvulsivantes/uso terapéutico , Epilepsia/complicaciones , Epilepsia/terapia , Miastenia Gravis/complicaciones , Miastenia Gravis/terapia , Adulto , Epilepsia/tratamiento farmacológico , Epilepsia/cirugía , Femenino , Humanos , Miastenia Gravis/tratamiento farmacológico , Miastenia Gravis/cirugíaRESUMEN
TITLE: Miastenia grave y tratamiento con inmunoglobulinas por via intravenosa.
Asunto(s)
Inmunoglobulinas Intravenosas/uso terapéutico , Miastenia Gravis/terapia , HumanosRESUMEN
Introducción: la exacerbación aguda grave de la enfermedad pulmonar obstructiva crónica es la sexta causa de muerte a nivel mundial y su asociación a la miastenia es excepcional. Cuando ambas enfermedades coexisten en un paciente que necesita una cirugía de urgencia, plantea un reto anestésico debido a la dificultad en el mantenimiento de la ventilación y la necesidad de una extubación precoz. En situaciones de este tipo el uso de BiPAP mejora los parámetros ventilatorios e incrementa el margen de seguridad para un barotrauma, así como permite en teoría reducir el periodo de ventilación. Objetivo: mostrar la conducta ventilatoria en un caso clínico que padece enfermedad pulmonar obstructiva crónica y miastenia gravis aceptado transversostomía. Caso clínico: paciente ingresado en la unidad de cuidados intensivos por exacerbación aguda grave de EPOC asociado a crisis colinérgica que presenta una fistula recto vesical y fue intervenido de urgencia para realizar una transversostomía. Se describe la valoración preoperatoria realizada por los anestesiólogos, resultados de estudios imaginológicos y gasométricos. También se expone la medicación preanestésica y el plan anestésico elaborado con especial énfasis en la aplicación de BiPAP intraoperatoria. Conclusiones: este modo ventilatorio puede ser una alternativa eficiente en el intraoperatorio de pacientes de alto riesgo de complicaciones respiratorias postoperatorias(AU)
Introduction: severe acute exacerbation of chronic obstructive pulmonary disease is the sixth leading cause of death worldwide and its association with myasthenia is exceptional. When both conditions coexist in a patient requiring emergency surgery, it poses a challenge due to anesthetic difficulty in maintaining ventilation and the need for early extubation. In such situations, the use of BiPAP improves the ventilatory parameters and increases the safety margin for barotrauma, as well as it theoretically allows reducing the ventilation period. Objective: To show the ventilatory behavior in a clinical case with chronic obstructive pulmonary disease and myasthenia gravis accepted for transversostomy. Clinical case: A patient admitted to the intensive care unit for severe acute exacerbation of COPD associated with cholinergic crisis presented a bladder and rectal fistula and was intervened as an emergency for a transversostomy. Preoperative assessment made by the anesthesiologists is described, together with the blood gas and imaging studies. The preanesthetic medication is presented, together with the action plan developed with special emphasis on the application of intraoperative BiPAP.Conclusions: This ventilatory mode can be an efficient choice in the intraoperative time for patients at high risk for postoperative respiratory complications(AU)
Asunto(s)
Humanos , Masculino , Persona de Mediana Edad , Enfermedad Pulmonar Obstructiva Crónica/mortalidad , Enfermedad Pulmonar Obstructiva Crónica/patología , Fístula Rectal/diagnóstico , Miastenia Gravis/terapia , Respiración ArtificialRESUMEN
Tecnologías evaluadas: -Tecnologías actuales: electromiografía con electrodo de fibra única e ICE test;\r\n-Tecnología nueva: anticuerpos bloqueadores de acetilcolina receptores, prueba de Tensilon, prueba de estímulo repetitivo. Población: Esta prueba se puede aplicar a todas las edades y a todos los sexos, ya que la aparición de la enfermedad puede presentarse en toda la población. Perspectiva: Tercer pagador - Sistema General de Seguridad en Salud (SGSSS) colombiano. Horizonte temporal: El horizonte temporal de este AIP en el caso base corresponde a un año. Adicionalmente se reportan las estimaciones del impacto presupuestal para los años 2 y 3, bajo el supuesto de la inclusión en el POS en el año 1. Costos incluidos: Solo se tuvieron en cuenta los costos de las pruebas: -Electromiografía con electrodo de fibra única: $71.262,1; -Ice test: $26.223,3; Prueba completa de Tensilon: $24.000; -Prueba de estímulo repetitivo: $46.219,1; -Test de anticuerpos contra receptor de acetilcolina por RIA (ACRA): $45.416. Fuente de costos: Para todas las pruebas diagnósticas se utilizó el promedio ponderado estimado desde los registros de uso de servicios de 2014 SISPRO (módulo de prestación de servicios, mediante conexión OBDS), teniendo como corte de búsqueda la fecha del desarrollo de este impacto (20/10/2015). Todos los costos de las pruebas son ponderados por el número de unidades utilizadas que reporta la misma base de datos. Además, todas\r\nlas tecnologías son costeadas desde bases de aseguradores, para confirmación de precios. Resultados: Actualmente, el mercado se encuentra dominado por la electromiografía con electrodo de fibra única, la cual se encuentra dentro del plan de beneficios, pero por opinión de los realizadores, una vez que la prueba de acetilcolina receptores y de Lambert entre al plan de beneficios, se aumentará su participación, lo cual repercutirá en un ahorro al sistema, dado que dichas pruebas son menos costosas.(AU)
Asunto(s)
Humanos , Acetilcolina/análisis , Anticuerpos Bloqueadores/uso terapéutico , Edrofonio/análisis , Electromiografía/métodos , Miastenia Gravis/terapia , Reproducibilidad de los Resultados , Colombia , Costos y Análisis de Costo/métodos , Tecnología Biomédica , ElectrodosRESUMEN
OBJECTIVE: to determine the clinical profile of patients with myasthenia gravis (MG); followed at the Neuromuscular Diseases Clinic of the University Hospital, Federal University of Minas Gerais, Brazil, and to compare it with other Brazilian case series. METHODS: sociodemographic and clinical data were collected from patients, and a systematic literature review performed, focusing on national studies on the clinical profile of MG patients. RESULTS: sixty nine patients were enrolled in the study. Fifty five (91%) subjects were female and the mean age (SD) was 37.6 (± 11.4) years. The mean disease duration was 14.1 years. Regarding treatment, prednisone was the most used strategy (64%), followed by the use of azathioprine (43%). There was no difference between thymectomized (42) and non-thymectomized (27) patients regarding disease severity and medication use. CONCLUSION: clinical and socio-demographic features of this MG sample from a University-based clinic resemble those reported in other Brazilian series and in the international literature.
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Miastenia Gravis/diagnóstico , Adolescente , Adulto , Brasil , Estudios Transversales , Femenino , Hospitales Universitarios , Humanos , Masculino , Persona de Mediana Edad , Miastenia Gravis/terapia , Índice de Severidad de la Enfermedad , Factores Socioeconómicos , Timectomía , Adulto JovenRESUMEN
Summary Objective: to determine the clinical profile of patients with myasthenia gravis (MG); followed at the Neuromuscular Diseases Clinic of the University Hospital, Federal University of Minas Gerais, Brazil, and to compare it with other Brazilian case series. Methods: sociodemographic and clinical data were collected from patients, and a systematic literature review performed, focusing on national studies on the clinical profile of MG patients. Results: sixty nine patients were enrolled in the study. Fifty five (91%) subjects were female and the mean age (SD) was 37.6 (±11.4) years. The mean disease duration was 14.1 years. Regarding treatment, prednisone was the most used strategy (64%), followed by the use of azathioprine (43%). There was no difference between thymectomized (42) and non-thymectomized (27) patients regarding disease severity and medication use. Conclusion: clinical and socio-demographic features of this MG sample from a University-based clinic resemble those reported in other Brazilian series and in the international literature. .
Objetivo: determinar o perfil clínico dos pacientes com miastenia gravis (MG) atendidos na Clínica de Doenças Neuromusculares do Serviço de Neurologia do Hospital das Clínicas da Universidade Federal de Minas Gerais, e comparar com outras séries de casos brasileiras. Métodos: foram coletados dados clínicos e sociodemográficos dos pacientes, e realizada revisão sistemática de literatura, focando em estudos nacionais sobre o perfil clínico de pacientes com MG. Resultados: sessenta e nove pacientes participaram do estudo, sendo 55 (91%) do sexo feminino, e a média de idade (desvio-padrão) de 37,6 (±11,4) anos. O tempo médio de doença foi de 14,1 anos. Em relação ao tratamento, o uso isolado de prednisona foi a estratégia mais adotada (64%), seguida do uso de azatioprina (43,3%). Não houve diferença entre os pacientes timectomizados (47) e não timectomizados (22) no que diz respeito à gravidade da doença e ao tratamento. Conclusão: as características clínicas e sociodemográficas da presente amostra de pacientes com MG de um serviço universitário assemelham-se às características de outras séries brasileiras e da literatura internacional. .
Asunto(s)
Adolescente , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Adulto Joven , Miastenia Gravis/diagnóstico , Brasil , Estudios Transversales , Hospitales Universitarios , Miastenia Gravis/terapia , Índice de Severidad de la Enfermedad , Factores Socioeconómicos , TimectomíaAsunto(s)
Humanos , Masculino , Femenino , Anestesiología , Miastenia Gravis/patología , Miastenia Gravis/terapia , Bloqueo NeuromuscularRESUMEN
Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission leading to generalized or localized muscle weakness due most frequently to the presence of autoantibodies against acetylcholine receptors in the postsynaptic motor end-plate. Myasthenic crisis (MC) is a complication of MG characterized by worsening muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. It also includes postsurgical patients, in whom exacerbation of muscle weakness from MG causes a delay in extubation. MC is a very important, serious, and reversible neurological emergency that affects 20-30% of the myasthenic patients, usually within the first year of illness and maybe the debut form of the disease. Most patients have a predisposing factor that triggers the crisis, generally an infection of the respiratory tract. Immunoglobulins, plasma exchange, and steroids are the cornerstones of immunotherapy. Today with the modern neurocritical care, mortality rate of MC is less than 5%.
Asunto(s)
Miastenia Gravis , Diagnóstico Diferencial , Humanos , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Miastenia Gravis/terapia , Factores de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
Myasthenia gravis (MG) is an autoimmune disorder affecting neuromuscular transmission leading to generalized or localized muscle weakness due most frequently to the presence of autoantibodies against acetylcholine receptors in the postsynaptic motor end-plate. Myasthenic crisis (MC) is a complication of MG characterized by worsening muscle weakness, resulting in respiratory failure that requires intubation and mechanical ventilation. It also includes postsurgical patients, in whom exacerbation of muscle weakness from MG causes a delay in extubation. MC is a very important, serious, and reversible neurological emergency that affects 20–30% of the myasthenic patients, usually within the first year of illness and maybe the debut form of the disease. Most patients have a predisposing factor that triggers the crisis, generally an infection of the respiratory tract. Immunoglobulins, plasma exchange, and steroids are the cornerstones of immunotherapy. Today with the modern neurocritical care, mortality rate of MC is less than 5%.
Miastenia grave (MG) é um distúbio autoimune que afeta principalmente a transmissão neuromuscular, levando a fraqueza muscular generalizada ou localizada. É devida mais frequentemente à presença de auto-anticorpos anti-receptores de acetilcolina na fenda pós-sináptica da placa motora. A crise miastênica (CM) é uma complicação da MG caracterizada por piora da fraqueza muscular, resultando en falência respiratória, o que requer entubação endotraqueal e ventilação mecânica. Isto ocorre também em pacientes pós-cirúrgicos, em que há piora da fraqueza muscular devido à MG, causando um atraso na extubação. MC é uma emergência neurológica importante, séria e reversível que afeta 20–30% dos pacientes miastênicos, usualmente duranteo primeiro ano de enfermidade, podendo a crise miastênica ser a manifestação inicial da MG. A maioria dos pacientes tem fatores predisponentes que desencadeiam a crise, geralmente uma infecção do trato respiratório. Imunoglobulina, plasmaférese e esteróides são a pedra angular da imunoterapia. Hoje, dentro da terapia neurocrítica, a taxa de mortalidade na CM é menor que 5%.
Asunto(s)
Humanos , Miastenia Gravis , Diagnóstico Diferencial , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Miastenia Gravis/terapia , Factores de Riesgo , Índice de Severidad de la EnfermedadRESUMEN
La miastenia grave es una enfermedad caracterizada por debilidad y fatiga de los músculos voluntarios debido a una trasmisión anómala a nivel de la unión neuromuscular. La prevalencia es aproximadamente de 5 casos/100 000 personas. La miastenia grave puede ser bulbar, ocular o generalizada. Existen formas clínicas en la infancia como son la miastenia neonatal transitoria, la miastenia congénita y la miastenia juvenil. Los músculos oculares, faciales y bulbares son los más frecuentes afectados por la enfermedad. Cuando los síntomas se limitan a la musculatura cercana al ojo se denomina miastenia grave ocular. Una vez el oftalmólogo diagnostica o sospecha la miastenia grave, un neurólogo generalmente dirige la comprobación y tratamiento. El papel del oftalmólogo continúa siendo importante, además de chequear la motilidad y disfunción palpebral y proporcionar el alivio sintomático para estos desórdenes, debe estar alerta a la posibilidad de ambliopía(AU)
Myasthenia gravis is an illness characterized by weakness and fatigue of the voluntary muscles due to an anomalous transmission at neuromuscular juncture. The prevalence rate is approximately 5 cases/100 000 people. Myasthenia gravis can be bulbar, ocular or widespread. Clinical forms exist in the childhood such as neonatal transitory myasthenia, congenital myasthenia and juvenile myasthenia. The ocular facial and bulbar muscles are the most frequently affected by this illness. When the symptoms occur in the musculature close to the eye, it is named ocular myasthenia gravis. Once the ophthalmologist suspects of or diagnoses myasthenia gravis, a neurologist generally leads the confirmation tests and the treatment. The role of the ophthalmologist continues being important, since he should check the palpebral motility and dysfunction, provide the relief to symptoms and be alert to the probable amplyopia(AU)