RESUMO
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.
Assuntos
Miastenia Gravis , Receptores Proteína Tirosina Quinases , Autoanticorpos , Humanos , Debilidade Muscular , Miastenia Gravis/diagnóstico , Miastenia Gravis/terapia , Junção Neuromuscular , Receptores ColinérgicosRESUMO
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.
Assuntos
COVID-19 , Miastenia Gravis , COVID-19/complicações , Feminino , Humanos , Pessoa de Meia-Idade , Miastenia Gravis/complicações , Miastenia Gravis/terapia , Troca Plasmática , SARS-CoV-2RESUMO
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.
Assuntos
Miastenia Gravis , Qualidade de Vida , Adulto , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Miastenia Gravis/epidemiologia , Miastenia Gravis/terapia , Apoio SocialRESUMO
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.
Assuntos
Miastenia Gravis/terapia , Criança , Inibidores da Colinesterase/uso terapêutico , Humanos , Imunossupressores/classificação , Imunossupressores/uso terapêutico , Miastenia Gravis/cirurgia , Esteroides/uso terapêutico , TimectomiaRESUMO
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.
Assuntos
Humanos , Criança , Miastenia Gravis/terapia , Esteroides/uso terapêutico , Timectomia , Inibidores da Colinesterase/uso terapêutico , Imunossupressores/classificação , Imunossupressores/uso terapêutico , Miastenia Gravis/cirurgiaRESUMO
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.
Assuntos
Miastenia Gravis/diagnóstico , Tireoidite Subaguda/diagnóstico , Erros de Diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Miastenia Gravis/terapia , Índice de Gravidade de DoençaRESUMO
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.
Assuntos
Humanos , Masculino , Feminino , Criança , Adolescente , Adulto , Pessoa de Meia-Idade , Idoso , História do Século XXI , Procedimentos Cirúrgicos Oftalmológicos , Blefaroptose , Estudos Prospectivos , Blefarofimose , Doenças Palpebrais , Fascia Lata , Miastenia Gravis , Procedimentos Cirúrgicos Oftalmológicos/métodos , Procedimentos Cirúrgicos Oftalmológicos/reabilitação , Blefaroptose/cirurgia , Blefaroptose/reabilitação , Blefarofimose/cirurgia , Blefarofimose/reabilitação , Doenças Palpebrais/cirurgia , Doenças Palpebrais/reabilitação , Fascia Lata/anatomia & histologia , Fascia Lata/anormalidades , Fascia Lata/cirurgia , Miastenia Gravis/cirurgia , Miastenia Gravis/terapiaRESUMO
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.
Assuntos
Lobectomia Temporal Anterior , Anticonvulsivantes/uso terapêutico , Epilepsia/complicações , Epilepsia/terapia , Miastenia Gravis/complicações , Miastenia Gravis/terapia , Adulto , Epilepsia/tratamento farmacológico , Epilepsia/cirurgia , Feminino , Humanos , Miastenia Gravis/tratamento farmacológico , Miastenia Gravis/cirurgiaRESUMO
TITLE: Miastenia grave y tratamiento con inmunoglobulinas por via intravenosa.