Exploration on the diagnosis and treatment of generalized myasthenia gravis with acupuncture and moxibustion based on the study of ancient medical works / 中国针灸

Through collecting the relevant provisions and medical cases of

Tratamiento de la miastenia gravis juvenil / Treatment of juvenile myasthenia gravis

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.

Tratamento cirúrgico da ptose palpebral moderada e grave: análise de resultados / Surgical treatment of moderate and severe ptosis: analysis of results

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.

Análisis de impacto presupuestal del uso de anticuerpos bloqueadores de acetilcolina receptores, comparado con la prueba de Tensilon, de estímulo repetitivo, electromiografía con electrodo de fibra única e I CE test para pacientes con miastenia gravis / Budget impact analysis of the use of acetylcholine receptor blocking antibodies, compared to the Tensilon test, repetitive stimulus, electromyography with single fiber electrode and IEC test for patients with myasthenia gravis

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)

Clinical profile of patients with myasthenia gravis followed at the University Hospital, Federal University of Minas Gerais / Perfil clínico de pacientes com miastenia gravis em acompanhamento no Hospital das Clínicas, da Universidade Federal de Minas Gerais

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. .

The myasthenic patient in crisis: an update of the management in Neurointensive Care Unit / Pacientes miastenicos em crise: uma melhora de conduta na unidade de terapia intensiva

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%.

Miastenia grave y miastenia grave ocular / Myasthenia gravis and ocular myasthenia gravis

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

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

Timectomía videotoracoscópica en miastenia gravis: técnica quirúrgica y experiencia inicial / Thymectomy in myasthenia gravis thoracic: surgical technique and initial experience

Introducción: Desde la década del 40 se reconoce el beneficio de la timectomía en el tratamiento de la miastenia gravis. La misma se realizó tradicionalmente por esternotomía o cervicotomía. La videotoracoscopia permite la extirpación tímica con menor agresión quirúrgica y recuperación más temprana. Material y Métodos: En los últimos 4 años se realizó un total de 14 timectomía por videotoracoscopia a pacientes portadores de miastenia gravis. Los pacientes eran 11 mujeres y 3 varones, con una edad media de 34,1 años, con un rango entre 16 y 53 años. En todos los casos se realizó mediante videotoracoscopía de 3 trocares, con acceso por hemitorax derecho. Resultados: Se realizó timectomía total exclusivamente toracoscópica en todos los casos, sin conversiones ni reoperaciones. Un solo paciente requirió ARM posterior menor a 4hs de postoperatorio, en 4 pacientes se extubó en UTI durante las 2 hs de postoperatorio y los 9 restantes salieron sin tubo endotraqueal de quirófano. La mortalidad postoperatoria fue nula. Conclusiones: la timectomía videotoracoscópica es útil en el tratamiento de la miastenia gravis y tiene como ventaja una mejor recuperación del paciente, y una mejor visión quirúrgica, que redunda en menor morbimortalidad quirúrgica.

Introduction: From the 40 recognized the benefit of thymectomy in the treatment of myasthenia gravis. The same was done traditionally by sternotomy or neck incision. VATS allows removal thymic less aggressive surgery and early recovery. Material and Methods: In the last 4 years was a total of 14 video-assisted thymectomy in patients with myasthenia gravis. Patients were 11 women and 3 men, mean age of 34.1 years, ranging between 16 and 53 years. In all cases was performed by video-assisted thoracoscopy of 3 trocars, accessible by right hemithorax. Results: We performed thoracoscopic thymectomy only com­plete in all cases without conversion or reoperation. One patient required subsequent ARM less than 4 hrs after surgery in 4 patients were extubated in the ICU during the 2 hrs after surgery and the remaining 9 endotracheal tube left without surgery. Postoperative mortality was nil. Conclusions: VATS thymectomy is useful in the treatment of myasthenia gravis and has the advantage of better patient recovery, and better surgical view, resulting in less surgical morbidity.

Thymoma followed by aplastic anemia: two different responses to immunosuppressive therapy

Aplastic anemia is an uncommon complication of thymoma and is extremely infrequent after the surgical removal of a thymic tumor. Aplastic anemia is a result of marrow failure and is characterized by peripheral pancytopenia and severely depressed marrow cellularity; it may be an autoimmune manifestation of thymoma. As thymoma-associated hematological dyscrasias, which include pure red cell aplasia, aplastic anemia and myasthenia gravis, are supposed to be of immunologic origin, two cases of very severe aplastic anemia following the resection of lymphocytic thymomas treated with immunosuppression are herein presented.

Myasthenia gravis in Ceará, Brazil: clinical and epidemiological aspects / Miastenia gravis no Ceará, Brasil: aspectos clínicos e epidemiológicos

A retrospective chart review was performed on patients diagnosed as having myasthenia gravis in Ceará State, Brazil and who were followed from October 1981 to June 2009. Clinical and epidemiologic aspects were evaluated. In this work, 122 patients were studied, of whom 85 (69.7 percent) were females and 37 (30.3 percent) were males. The disease duration ranged from five months to 50 years (8.9±8.1 years). Age at the first symptoms varied from 0 to 74 years (31.9±14.4 years). The first main symptoms and signs were ptosis, diplopia and limb weakness. Generalized myasthenia was the most common clinical presentation, but 5.1 percent (n=6) persisted as ocular myasthenia. Thymectomy was performed in 42.6 percent (n=52) of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR) antibodies were present in 80 percent (n=20) of specimens tested. The data presented are similar to those of studies performed in other countries.

Foram analisados, retrospectivamente, os prontuários de pacientes miastênicos, diagnosticados e seguidos entre outubro de 1981 e junho de 2009 no Estado do Ceará, Brasil. Foram coletados dados clínicos e epidemiológicos. Na casuística foram estudados 122 pacientes: 85 (69,7 por cento) do sexo feminino e 37 (30,3 por cento) do sexo masculino. O tempo de doença variou de 5 meses a 50 anos (8,9±8,1 anos). A idade de inicio da doença variou de 0 a 74 anos (31,9±14,4 anos). Na amostra estudada, os primeiros sintomas foram principalmente ptose, diplopia e fraqueza dos membros. A maioria dos pacientes apresentou a forma generalizada, enquanto 5,1 por cento (n= 6) persistiram com miastenia ocular. Timectomia foi realizada em 42,6 por cento (n=52) dos pacientes. Timoma estava presente em 10 pacientes. Anticorpo anti-receptor de acetilcolina foi positivo em 80 por cento (n=20) das amostras testadas. Os aspectos clínicos e epidemiológicos da amostra estudada têm semelhança com aqueles estudados em outros países.

Plasmaféresis en el tratamiento de la Miastenia Gravis: presentación de caso / Plasmapheresis in the treatment of the Miastenia Gravis: a case presentation

Se presentó una paciente de19 años de edad, raza blanca, femenina, con antecedentes de padecer de miastenia gravis desde hace aproximadamente 2 años, que acudió al cuerpo de guardia en plena crisis de su enfermedad de base; este cuadro progresó tomando los músculos respiratorios por lo que necesitó de ventilación mecánica artificial, cuadro clínico que llevó a realizar tres sesiones de plasmaféresis como tratamiento de la crisis aguda de la miastenia gravis con toma de la musculatura respiratoria, se efectuaron en días consecutivos con un promedio de cuatro horas y media, la albúmina humana como solución de reemplazo del plasma separado de la sangre de la paciente para eliminar los mediadores que llevaron a este cuadro a la paciente, acto seguido a la última sesión se separó del respirador artificial.

A nineteen years patient, white race, female with antecedents of suffering miastenia gravis since two years approximately, arrived to the emergency department in full crisis of her basic disease, guardhouse, this profile progressed taking respiratory muscles, reason why she needed artificial mechanical ventilation, this clinical profile carried out three sessions of plasmapheresis as acute crisis treatment of miastenia gravis with taking of respiratory muscle, they took place in consecutive days with an average of four hours and a half, human albumin as replaced solution plasma separated from the patient blood to eliminate the mediators that carried out this patient to this clinical profile, immediately after wards to the last session she was apart from the artificial respirator.

Isaac's syndrome associated with myasthenia gravis and thymoma.

A 65-year-old male developed fatigable weakness of ocular and bulbar muscle and positive anti-acetyl cholinesterase antibodies suggesting the diagnosis of myasthenia gravis. His condition responded to anticholinesterase and immunotherapy. However, 18 months later, he developed painful paresthesiae, muscle cramps with hyperhiderosis, and was diagnosed as having Isaac's syndrome (neuromyotonia, continuous muscle fibre activity). Computed tomography of the chest revealed a thymic mass, which was confirmed after surgery and histopathology as thymic cell carcinoma. The co-occurrence of myasthenia gravis and continuous muscle fiber activity should prompt the consideration of the occurrence of these disorders as one of the paraneoplastic manifestations, most often due to a thymic neoplasm. Both these conditions respond to treatment of underlying thymoma. This case is a very rare presentation worth reporting.

The cost-effectiveness of open or thoracoscopic thymectomy compared to medical treatment in managing Myasthenia gravis without thymomas / Costo efectividad de timectomía abierta y toracoscópica frente al tratamiento médico, en el manejo de Miastenia gravis sin timoma

Objective Assessing the cost-effectiveness of open or thoracoscopic thymectomy compared to medical therapy in managing myasthenia gravis not associated with thymoma. Methods A Markov model was designed for evaluating three strategies' cost-effectiveness. Transition probabilities were taken from the pertinent literature; the costs were estimated from official tariff manuals. Incremental cost-effectiveness ratios were estimated and probabilistic and deterministic sensitivity analysis was used for clinical variables, costs and the model's assumptions. Results Thoracoscopic thymectomy was the most effective and least costly strategy and dominated the other two alternatives. The cost per life year gained was Col § 1 129 531 without discount and Col § 805 179 with discount. Univariate sensitivity analysis showed that the main variables affecting the results were the effects' discount rate, the cost of a myasthenic crisis and the probability of complete remission. Thoracoscopy thymectomy was the most cost-effective strategy for different thresholds of willingness to pay in probabilistic analysis. Conclusions Thoracoscopic thymectomy is a cost-effective strategy in the treatment of MG without thymoma.

Objetivo Evaluar la costo efectividad de las timectomía abierta y toracoscópica frente a la terapia mé,dica en el manejo de miastenia gravis sin timoma. Mé,todo Se construyó un modelo de Markov para evaluar la costo efectividad de las 3 estrategias. Las probabilidades de transición se obtuvieron de la literatura. Los costos se estimaron a partir de las tarifas oficiales. Se calculó la costo-efectividad incremental. Se realizaron análisis de sensibilidad probabilísticos y determinísticos para las variables clínicas, los costos supuestos del modelo. Resultados La timectomía toracoscópica es la estrategia más efectiva y menos costosa, y domina a las otras dos alternativas. El costo por año de vida ganado fue de § 1 129 531 y § 805 179 pesos colombianos, con y sin descuento. El análisis de sensibilidad univariado mostró que las principales variables que afectan los resultados son la tasa de descuento, el costo de una crisis miasté,nica y la probabilidad de remisión completa. En el análisis de sensibilidad probabilístico, la timectomía toracoscópica es la estrategia costo-efectiva para los diferentes umbrales de disponibilidad a pagar. Conclusiones La timectomía toracoscópica es una estrategia costo-efectiva en el tratamiento de miastenia gravis sin timoma.

Thymoma Associated with Myasthenia Gravis in Infancy.

Thymomas are associated with various autoimmune disorders in adults, most commonly with myasthenia gravis (MG). In MG antibodies developed against acetylcholine (ACh) receptors, resulting weakness of muscle after repeated use and recovery following a period of rest. However, MG due to thymoma is extremely rare in pediatric age group.1We report an infant with MG due to invasive thymoma.

Miastenia gravis juvenil / Juvenile myasthenia gravis

La miastenia gravis juvenil (MGJ) es un trastorno crónico auto inmune en el cual existen anticuerpos séricos que al unirse a los receptores de acetilcolin nicotínicos de la membrana muscular de la placa motora alteran la transmisión neuromuscular. El resultado es fatiga muscular precoz con progresión a la parálisis durante estados de contracción muscular iterativos (movimientos) o sostenidos (posturas) y más raramente parálisis permanente durante el reposo. Los músculos inervados por los nervios craneales, especialmente los extraoculares y elevadores de los párpados, tienen más tendencia a la debilidad muscular persistente que los inervados por otros pares craneales y las extremidades. Las formas clínicas de presentación son generalizadas, oculares y respiratorias. El diagnóstico se sospecha mediante la anamnesia, la fatiga anormal se comprueba mediante el examen físico y la estimulación eléctrica iterativa del nervio que inerva al músculo afectado pero no paralizado. Se corrobora mediante la administración de inhibidores de la acetilcolin esterasa (IACE) que al aumentar la cantidad de acetilcolin en la hendidura sináptica, corrigen la fatiga o la debilidad muscular transitoriamente. Se hace el diagnóstico de certeza mediante la demostración sérica de anticuerpos contra los receptores de acetilcolin (ACRA). El tratamiento es a largo plazo sintomático con IACE y etiopatogénico con inmunosupresores, plasmaféresis, gamma globulina endovenosa y timectomía. El curso es crónico. La remisión espontánea o después de tratamiento sintomático o etiopatogénico ocurre entre 1-10 años respectivamente. La mortalidad es prácticamente nula aun durantes las crisis miastenias gracias a la educación de padres, pacientes y público en general sobre el tema, al desarrollo del sistema de respuesta rápida de auxilio domiciliario y las unidades de cuidados intensivos y el empleo de la ventilación asistida profiláctica, plasmaféresis y administración endovenosa de gamma globulina.

Juvenile myasthenia gravis is a chronic autoimmune disorder which occurs when serum antibodies combine with nicotinic acetylcholine receptors at the muscle membrane of the motor endplate imparing the neuromuscular transmission. It results in early muscle fatigability with progression to a complete paralysis during repetitive muscle contraction (movements) or steady muscle contraction (postures), and less common persistent paralysis at rest. The cranial nerves, mainly the one innervating the extraoccular and palpebral levator, are the most susceptible to permanent weakness and paralysis at rest. Initial clinical presentations are generalized, ocular and respiratory forms. The diagnosis is suspected through medical history of abnormal fatigability and corroborated by physical examination, repetitive nerve stimulation of an affected but not complete paralyzed muscle, correction of fatigability by the intravenous administration of acetylcholine esterase inhibitors, and by the presence of serum acetylcholine receptors antibodies (ACRA). The long term treatment is symptomatic (acetylcholine inhibitors) and etiopathogenic (immunosupresor drugs, plasmapheresis, intravenous gamma globulin and thymectomy. Spontaneous or post symptomatic and etiopathogenic treatment remissions occur from 1 to 10 years. Fatality is rare but children are at high risk during myasthenia crisis.

The Pattern and Presentation of Myathenia Gravis in Al-Shaab and Omdurman Teaching Hospitals

Introduction:Myasthenia gravis (MG) is the most common acquired autoimmune disorder of neuromuscular transmission. It has different patterns of presentation. Objective: to study the pattern and mode of presentation of Myasthenia Gravis in Al-Shaab and Omdurman Teaching Hospitals; Khartoum Sudan. Methods: The demographic and clinical characteristics of 50 patients of myasthenia gravis (MG) were reviewed in Alshab and Omumdrman Teaching Hospitals for five months period from May to October 2008.Results: Out of 2400 patients attending neurology clinics 50 were found to have Myathenia Gravis [MG] with female; male ratio of 2.5:1. The age at presentation was the second decade of life in 34. Most of the patients [42] were from Khartoum State followed by North Kordofan State [14]. High occurrence of late onset fatigability and weakness was seen in 92of our patients. Ocular muscles involvement was detected in 78. MG was aggravated by hot weather and fever in 12of the patients. Quinine represented the most common drug which aggravated the myasthenia symptoms in 4. Diabetes mellitus and thyroid diseases were the most associated auto immune diseases seen in 12and 4respectively. The diagnosis was made by classic history and neurological examinations in 88and positive neostigmin test in64. The majority of patients were treated with steroids. Thymectomy was done in 10patients. The outcome was excellent as 82improved and only one [2] patient died.Discussion: Going with literature diagnosis of MG was made depending on classical history; full neurological examination and confirmed by tensilon test. Because of different reasons more sophisticated tests were not done in our patients. Only patients with malaria who were treated with quinine showed deterioration of their symptoms in our study. Strikingly; myasthenia crisis which were reported in 27of patients were not seen in our population. Because of lack of usage of steroid sparing agents the vast majority [96] of our patients were treated with steroids. Conclusion:Generalized myasthenia gravis with ocular involvement is common in our patients. However; pure OMG was not seen. Thymectomy was done in a small number of our patients with reference to other studies

Myasthenia gravis: presentation and outcome in 104 patients managed in a single institution

Few studies have attempted to delineate the clinical profile of myasthenia gravis [MG] among people of Arab ancestry. Therefore, we sought to clarify the clinical profile, the outcome of treatment and the role of thymectomy in non-thymomatous MG in Saudi Arabia. We retrospectively studied 104 patients followed over a mean period of 7.2 years [range, 1 to 22 years] at the King Khaled University Hospital, Riyadh, Saudi Arabia. Disease outcomes were compared among thymectomized and non-thymectomized patients according to the post-intervention status criteria of the Myasthenia Gravis Foundation of America [MGFA]. Age of onset was 22.5 +/- 9.3 years [mean +/- SD] in females and 28.2 +/- 15.9 years in males, with peaks in the second and third decades among females and the third and fourth decades among males. At diagnosis, a majority of patients had moderate generalized weakness, equivalent to MGFA class III severity. After medical treatment with or without thymectomy, 9.6% of all patients had achieved complete stable remission, 3.8% had pharmacological remission, 27.9% had minimal manifestations, 23.1% were improved, 20.2% were unchanged and 15.4% were worse. Only thymectomized patients without a thymoma achieved remission, a significant benefit over those who had no thymectomy [P=.02]. MG presents at a younger age among Saudi Arabs compared to other racial groups. Thymectomy conferred significant benefits towards achievement of remission