Diabetes tipo 1 y Miastenia Gravis / Type-1 Diabetes and Myasthenia Gravis

Introducción: El desarrollo de la tolerancia inmunológica frente a los autoantígenos se denomina autotolerancia. La Diabetes Mellitus tipo 1A (1ADM) es un trastorno metabólico secundario a la destrucción autoinmune de las células beta pancreáticas e insulitis. La miastenia grave (MG) es una enfermedad autoinmune causada por el bloqueo postsináptico de la placa mioneural por AAcs contra los receptores de acetilcolina (ACRA) o contra moléculas de la membrana postsináptica. La asociación entre DM1A y MG se puede observar en el síndrome poliglandular tipo III, caracterizado por enfermedad autoinmune de la glándula tiroides asociada con otras entidades autoinmunes. Método: Reporte de Casos, cuatro pacientes entre 7-19 años, con asociación de MG y DM1A atendidos en el Hospital Garrahan. Conclusión: La Tiroiditis de Hashimoto y la Enfermedad Celíaca son las enfermedades autoinmunes relacionadas más frecuentemente con DM1A en nuestra población. La bibliografía describe la asociación de MG y Tiroiditis de Hashimoto y su coexistencia con DM1A se describe en el Síndrome Poliglandular III. En este trabajo presentamos 4 casos de DM1A asociado con MG fuera de dicho síndrome (AU)

Introduction: The development of immune tolerance to autoantibodies (AAbs) is referred to as self-tolerance. Type 1A Diabetes Mellitus (1ADM) is a metabolic disorder secondary to autoimmune destruction of pancreatic beta cells and insulitis. Myasthenia gravis (MG) is an autoimmune disease caused by postsynaptic blockade of the myoneural plate by AAbs against acetylcholine receptors (Acra) or against postsynaptic membrane molecules. The association between 1ADM and MG may be observed in polyglandular syndrome type III, characterized by autoimmune disease of the thyroid associated with other autoimmune conditions. Methods: Case report; four patients between 7-19 years old, with an association of MG and 1ADM seen at the Garrahan Hospital. Conclusion: Hashimoto's thyroiditis and celiac disease are autoimmune diseases most frequently related to 1ADM in our population. In the literature, the association of MG and Hashimoto's thyroiditis has been described and its coexistence with 1ADM is reported in polyglandular syndrome III. In this study we present 4 cases of 1ADM associated with MG unrelated to this syndrome. (AU)

Tratamiento de la eclampsia y miastenia gravis: reporte de un caso y revisión de la literatura / Eclampsia treatment and myasthenia gravis: case report and review of literature

OBJETIVO: Reportar el caso de una gestante con miastenia grave (MG) más preeclampsia-eclampsia y crisis miasténica en el puerperio mediato, y realizar una revisión de la literatura sobre el manejo farmacológico. MÉTODO: Se presenta el caso de una mujer de 26 años con MG, primigesta de 36 semanas de gestación, quien cursó con eclampsia y recibió fenitoína por 24 horas. Tuvo parto espontáneo sin complicaciones y crisis miasténica al día 11 del puerperio asociada a infección de vías urinarias y sepsis. Se realiza revisión de la literatura en PubMed, Cochrane, Embase, LILACS y Scopus, empleando los términos "Hypertension, Pregnancy-Induced", "Preeclampsia" y "Eclampsia", combinados con "Myasthenia Gravis", durante el periodo de publicación de 1960 a junio 2020, en inglés y español. RESULTADOS: Se encontraron 12 reportes de caso, dos con eclampsia y MG; el caso aquí reportado es el número 13. Ocho pacientes no recibieron medicamentos profilácticos de eclampsia y tres de ellas convulsionaron. En las que se usó sulfato de magnesio, todas cursaron con crisis miasténica. CONCLUSIONES: La evidencia actual en cuanto a la profilaxis y el tratamiento de la eclampsia y la MG corresponde a reportes de casos. El uso de sulfato de magnesio está contraindicado en pacientes con MG, por lo que se han utilizado fenitoína y levetiracetam.

OBJECTIVE: To report a case of pregnant women with myasthenia gravis (MG), plus preeclampsia-eclampsia and myasthenic crisis in the mediate puerperium; to conduct a literature review regarding its pharmacological management. METHOD: 26-year-old primigravida with 36 weeks of gestation and previous history of MG, who developed eclampsia and was treated with phenytoin for 24 hours, with later spontaneous delivery without any complications nor new seizures; and myasthenic crisis on day 11 of the puerperium associated with urinary tract infection and sepsis. A literature review was conducted in PubMed, Cochrane, Embase, LILACS and Scopus, using the controlled vocabulary "Hypertension, Pregnancy-Induced", "Preeclampsia" and "Eclampsia", combined with "Myasthenia Gravis", between 1960 and June 2020, in English and Spanish. RESULTS: 12 case reports were found, two of these with eclampsia and MG, the case reported here was number 13. In eight cases patients did not receive any prophylactic drugs for eclampsia and three of them had convulsions. In the cases where magnesium sulfate was used, all developed myasthenic crisis. CONCLUSIONS: The current evidence regarding prophylactic management and treatment corresponds to case reports. The use of magnesium sulfate is contraindicated in patients with MG, therefore phenytoin and levetiracetam have been used.

VATS Uniportal, masterclass Guatemala / VATS Uniportal, masterclass Guatemala

En las dos últimas décadas la evolución de la cirugía mínimamente invasiva del tórax ha transmutado de un abordaje de tres puertos, siguiendo dos puertos hasta llegar a puerto único, conocido también como VATS Uniportal, procurando un confort mucho mejor para el paciente y resultados quirúrgicos similares. Objetivos. Presentar la técnica quirúrgica de VATS Uniportal en un hospital nacional, efectuadas por un experto internacional en este campo. Pacientes y Métodos. Se presentan dos casos clínicos quirúrgicos: El de una paciente con Miastenia Gravis a quien se le realizó timectomía por abordaje sub-xifoideo y otra paciente, a quien se le completó una lobectomía inferior derecha por hallazgos de patología posterior a la resección de un nódulo pulmonar solitario, reportado como cáncer primario de pulmón. Conclusiones. La técnica de cirugía mínimamente invasiva, VATS Uniportal, ofrece grandes beneficios para el paciente, tanto estéticos como funcionales y su aprendizaje es posible con la transmisión de conocimientos y experiencias directa con la presencia del experto o indirectas a través de la información publicada. (AU)

In the last two decades, the evolution of minimally invasive chest surgery has transmuted from a three-port approach, following two ports until reaching a single port, also known as VATS Uniportal, seeking much better comfort for the patient and similar surgical results. Objective. Present the VATS Uniportal surgical technique in a national hospital, performed by an international expert in this field. Patients and Methods. Two surgical clinical cases are presented: that of a patient with Myasthenia Gravis who underwent thymectomy through the sub-xiphoid approach and another patient, who underwent a right lower lobectomy due to findings of pathology after the resection of a pulmonary nodule. solitary, reported as primary lung cancer. Conclusions. The minimally invasive surgery technique, VATS Uniportal, offers great benefits for the patient, both aesthetic and functional and its learning is possible with the transmission of knowledge and experiences directly with the presence of the expert or indirectly through published information. (AU)

Miastenia gravis y su asociación con trastornos linfoproliferativos: casos clínicos / Myasthenia gravis and its association with lymphoproliferative disorders: a case series

Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile. During a three-year period we identified four patients who developed different lymphoproliferative disorders: two with B-cell lymphoma, one with chronic lymphocytic leukaemia and one plasmacytoma with an associated amyloidosis. The MG was generalized but mild, all cases classified as type IIa according to the definition proposed by the MG Foundation of America. The neoplasia appeared two to 36 years after the onset of MG. These cases provide additional evidence of the association between MG and lymphoproliferative disorders.

Rendimiento neuropsicológico en pacientes con miastenia gravis / Rendimiento neuropsicológico en pacientes con miastenia gravis

La miastenia gravis es una enfermedad crónica, autoinmune que afecta la transmisión neuromuscular. Se han comunicado hallazgos contradictorios acerca del deterioro cognitivo en dicha enfermedad. El objetivo del presente estudio fue investigar el patrón cognitivo de pacientes con miastenia gravis. Participaron 24 pacientes con miastenia gravis, anticuerpos anti-receptor de acetilcolina (ACRA) positivos, y 24 controles. Pacientes: edad 43.9 ± 14.8, años de escolaridad 10.9 ± 3.3. Controles: edad 44.5 ± 15.4, años de escolaridad 11.5 ± 3.3. Se evaluaron las siguientes áreas: memoria verbal: (largo plazo almacenamiento, recuperación y recuerdo diferido) del test selectivo de memoria; atención: Paced Auditory Serial Addition Task (PASAT, 2 y 3 segundos); funciones ejecutivas (FE): analogías y secuencia números-letras. Se administró además el Inventario de Depresión de Beck II (IDB II). El 33.3% de los pacientes obtuvo un desempeño anómalo en dos o más test cognitivos. El 37.5% mostró deterioro en atención; 33.3% en memoria verbal; 29.2% en FE. Se encontraron diferencias significativas entre pacientes y controles en almacenamiento (p = 0.001); recuperación (p = 0.007); recuerdo diferido (p = 0.000); PASAT 3 (p = 0.009); PASAT 2 (p = 0.009); analogías (p = 0.003). Se encontró evidencia de depresión: leve en el 4.2 %; moderada en el 25% y grave en el 29.2%. El rendimiento neuropsicológico declina en los pacientes con miastenia gravis, encontrándose mayor alteración en la atención que en el resto de las áreas cognitivas.

Myasthenia gravis is a chronic autoimmune disease that affects the neuromuscular transmission. Controversial findings had been reported about cognitive impairment in this disease. The aim of this study was to investigate the cognitive pattern of patients with myasthenia gravis. There were enrolled 24 patients with myasthenia gravis, anti-acetylcholine receptor antibodies (ACRA) positive, and 24 healthy controls. Patients: age 43.9 ± 14.8, years of education 10.9 ± 3.3. Controls: age 44.5 ± 15.4, years of education 11.5 ± 3.3. The following areas were evaluated: verbal memory: (long-term storage, retrieval, delayed recall) of the Selective Remained Test; attention: Paced Auditory Serial Addition Task (PASAT 2 and 3 seconds); executive functions: analogies and numbers-letters sequence. Also, it was administered the Beck Depression Inventory II (BDI II). About 33.3% of patients obtained abnormal performance in two or more cognitive tests. 37.5% showed deterioration in attention; 33.3% in verbal memory; 29.2% in executive functions. Significant differences between patients and healthy controls were found in long-term storage (p = 0.001); retrieval (p = 0.007); delayed recall (p = 0.000); PASAT 3 (p = 0.009); PASAT 2 (p = 0.009) and analogies (p = 0.003). Evidence of depression was found: mild in 4.2% of patients; moderate in 25% and severe in 29.2%. Neuropsychological performance declines in patients with myasthenia gravis: attention was more affected than other cognitive areas.

Embarazada con miastenia grave ocular. Reporte de un caso / Pregnant woman with ocular myasthenia gravis. A case report

La miastenia grave es una enfermedad autoinmune de la placa motora del músculo estriado, caracterizada por fatiga y debilidad muscular fluctuante. La prevalencia es aproximadamente de 5 casos por cada 100 000 personas. La paciente reportada presentó síntomas que limitaban la musculatura cercana al ojo. Esta entidad se denomina miastenia grave ocular, su incidencia en el embarazo es infrecuente. Se reportó un caso de una paciente de 23 años de edad, atendida en la Consulta de Infertilidad del municipio Isla de la Juventud. Se consultó el caso con varias especialidades médicas. El nacimiento se produjo por cesárea de urgencia producto de estado fetal intranquilizante y aplicando anestesia regional. Se obtiene bebé sano, con buena puntuación de apgar y buen peso. Durante el puerperio la madre y el recién nacido evolucionaron satisfactoriamente (AU).

Myasthenia gravis is an autoimmune disease of the motor end-plate of the striated muscle, characterized by fluctuant muscular fatigue and weakness. Its prevalence is around 5 cases per 100 000 persons. The reported patient presented symptoms limiting the muscles near the eye. This entity is named ocular myasthenia gravis. Its incidence in pregnancy is infrequent. It is reported the case of a female patient, aged 23 years, attended in the Infertility Consultation of the municipality Isla de la Juventud. The case was consulted with several medical specialties. The delivery was produced by emergency cesarean section, applying regional anesthesia, due to the worrying fetal status. The newborn had good apgar score and weight. Mother and child showed satisfactory evolution during the postpartum period (AU).

Miastenia gravis: un diagnóstico diferencial importante a la hora de evaluar a un paciente con sintomatología ORL / Myasthenia gravis: an important differential diagnosis when assessing a patient with ent symptomatology

La miastenia gravis (MG) es una enfermedad autoinmune que afecta a la unión neuromuscular, y que se caracteriza por la presencia de autoanticuerpos dirigidos contra los receptores de membrana postsinápticos, bloqueando así la transmisión neuromuscular, y logrando de esta forma un descenso en el número de receptores en la placa neuromotora. Se presenta clínicamente como una debilidad muscular focalizada, de algunos grupos musculares, o generalizada. En el contexto ORL las presentaciones más clásicas afectan a la musculatura bulbar, presentándose como disfonía, disfagia, debilidad de la musculatura facial o la combinación de todas ellas. Presentamos 2 casos clínicos de pacientes con manifestaciones totalmente distintas de la misma enfermedad. En el primer caso se presenta a una paciente con compromiso leve de la musculatura bulbar y en el segundo, un paciente con una miastenia generalizada que es internado grave en la UCI por una crisis miasténica. Realizamos una revisión bibliográfica de las últimas pruebas diagnósticas y tratamientos para la MG, y tratamos de definir los signos y síntomas que nos ayudarán como otorrinolaringólogos a un diagnóstico y tratamiento oportuno.

Myasthenia gravis is an autoimmune disease of the neuromuscular junction, characterized by the presence of autoantibodies directed against the postsynaptic membrane receptors, blocking neuromuscular transmission in skeletal striated muscles, leading to a decrease in the number of receptors at the motor neuroplate. Clinically, myasthenia gravis presents as focalized or generalized muscle weakness. For the ENT the most frequent presentation is the bulbar involvement which presents as dysphonia, dysphagia, weakness of facial musculature or a combination of all. We report 2 cases of patients with completely different manifestations of the same disease. The first case is a patient with mild bulbar musculature compromise and the other, is a patient with severe generalized myasthenia who had to be admitted to the ICU for a myasthenic crisis.

Localized Thymic Amyloidosis Presenting with Myasthenia Gravis: Case Report

A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was located in the thymus. Microscopically, the mass consisted of diffuse amorphous eosinophilic materials. These deposits exhibited apple-green birefringence under polarized light microscopy after Congo red staining. Immunohistochemical analysis revealed that they were positive for both kappa and lambda light chains and negative for amyloid A. A diagnosis of localized primary thymic amyloidosis was finally made. After thymectomy, the symptoms of MG were controlled with reduced corticosteroid requirements. Localized thymic amyloidosis associated with MG has not been reported to date.

A Case of Ocular Myasthenia Gravis Presenting as Double Depressor Palsy

A 65-year-old man who had been experiencing diplopia in front and down gaze for 15 days visited our hospital. Hypertropia was noted in the patient's left eye, and limitation of depression was found in the adduction, primary gaze, and abduction. Brain magnetic resonance imaging showed no remarkable findings. Two weeks after the first visit, the patient complained of ptosis in the left eye. An ice test was performed and the ptosis was resolved after the test. Then, anti-acetylcholine receptor binding antibody levels were checked and found to be slightly elevated. We prescribed methylprednisolone per os 24 mg for 2 weeks, and his symptoms improved after the 2-week treatment. Five weeks after his first visit, the patient showed an ortho result in the alternate prism cover test and normal ocular movements. This may be the first case in which ocular myasthenia gravis presented as double depressor palsy, and in such cases, the possibility of ocular myasthenia gravis should be considered to rule out double depressor palsy.

Thymoma associated with myasthenia gravis and Sjögren syndrome / Timoma asociado con miastenia grave y síndrome de Sjögren

Thymoma is the most common neoplasm of the anterior mediastinum but thymoma with Sjögren syndrome (SS) is rare. Sjögren syndrome is a systemic autoimmune inflammatory disorder. It is characterized by lymphocytemediated destruction of exocrine glands, which leads to absent glandular secretion. Here, we present the case of a 63yearold man with thymoma and concurrent myasthenia gravis and SS, who achieved remission after thymectomy.

El timoma es la neoplasia más frecuente del mediastino anterior, pero un timoma acompañado del síndrome de Sjögren (SS) constituye una ocurrencia rara. El síndrome de Sjögren es un trastorno inflamatorio autoinmune sistémico. Se caracteriza por la destrucción - mediada por linfocitos - de las glándulas exocrinas, lo cual conduce a la ausencia de secreción glandular. Aquí presentamos el caso de un hombre de 63 años de edad con timoma, y miastenia gravis y SS concurrentes, que logró la remisión después de una timectomía.

Disfonia como principal queixa num quadro de miastenia grave: diagnóstico e fonoterapia / Dysphonia as the primary complaint in a case of myasthenia gravis: diagnosis and speech therapy

Miastenia grave é uma doença autoimune que se manifesta por fraqueza e fadiga muscular progressivas. São frequentes os sintomas oculares e bulbares, dentre eles, a disfonia. Este artigo relata um caso de disfonia, cuja avaliação fonoaudiológica contribuiu com o diagnóstico de miastenia grave e seu tratamento. A paciente em questão procurou o atendimento fonoaudiológico com diagnóstico otorrinolaringológico de presbifonia. A avaliação perceptivo-auditiva e acústica da voz identificou alterações em respiração, fonte glótica e filtro/ressonância. Como alguns dados obtidos com a anamnese e observados nas provas vocais não se relacionavam diretamente com a presença de presbifonia, houve a necessidade de discussão do caso com o médico, quando ambos concluíram a necessidade de encaminhar a paciente para avaliação neurológica. O neurologista consultado levantou a hipótese diagnóstica de miastenia grave e solicitou exames. A paciente seguiu em acompanhamento fonoaudiológico e medicamentoso. Na reavaliação vocal, ocorrida cerca de dois meses após o início do tratamento, foi constatada melhora na qualidade vocal, com grande impacto na qualidade de vida. Este trabalho evidencia a importância da avaliação fonoaudiológica detalhada e da participação do fonoaudiólogo na equipe interdisciplinar.

Myasthenia gravis is an autoimmune disease, manifested by progressive muscular weakness and fatigue. There are frequent ocular and bulbar symptoms, among them, dysphonia. This article reports a case of dysphonia that contributed to the diagnosis of myasthenia gravis through a speech evaluation. The patient sought speech therapy with the ENT diagnosis of presbyphonia. The perceptual voice assessment and acoustic analysis pointed out respiration, glottal voice source and resonance affections. Considering that some of the data obtained from anamnesis and vocal assessments were not directly related to presbyphonia, the speech therapist discussed the case with the physician and they both concluded it was necessary to refer the patient to a neurological evaluation. The neurologist then raised the diagnostic hypotheses of myasthenia gravis and requested further examinations. The patient underwent speech therapy and drug treatment. A vocal reassessment, which occurred two months after the initial treatment, showed improvement in voice quality, with great impact on quality of life. This article shows the importance of detailed clinical speech evaluation and participation of a speech therapist in an interdisciplinary team.

Timoma esclerosante: relato de caso / Sclerosing thymoma: case report

We present a rare case of thymoma in a 36-year old woman, who was initially diagnosed with severe myasthenia gravis and subsequently undergone surgical resection. During surgery tumor was found at the anterior mediastinum, tightly attached to the phrenic nerve, pleura and pericardium. Histological assessment showed large areas of sclerosis and fibrous collagenous tissue as well as islands of epithelial and lymphoid cells. Sclerosing thymoma, which is a rare subtype of thymoma (< 1%), was diagnosed, thus confirming the first report in Brazil. The patient showed partial improvement of symptoms associated with myasthenia gravis.

Relatamos um caso raro de timoma em uma mulher de 36 anos de idade, com clínica e diagnóstico de miastenia gravis de difícil controle clínico, submetida à ressecção cirúrgica. No intraoperatório, observou-se tumor no mediastino anterior, firmemente aderido ao nervo frênico, à pleura e ao pericárdio. Ao exame histológico, foram evidenciadas extensas áreas de tecido fibrocolagenoso e esclerose, assim como ilhas de células epiteliais e células linfoides. Diagnosticado timoma esclerosante, subtipo raro de timoma (< 1%), sendo este o primeiro caso relatado no Brasil. A paciente apresentou melhora parcial dos sintomas associados à miastenia gravis.

Enfermedad de graves en asociación con miastenia gravis: presentación de un caso y revisión de la literatura / Myasthenia gravis with concomitant hyperthyroidism: report of one case

Myasthenia gravis and Graves disease are autoimmune diseases that can coexist in the same patient. We report a 18 years old female with a history of myasthenia gravis diagnosed at the age of six, treated with neostigmine and prednisone. She starts with palpitations, dyspnea, proximal muscle weakness and exophthalmos appearing seven months after the onset of symptoms. She was admitted to the hospital due to her decompensated hyperthyroidism. After admission, she develops a myasthenic crisis, that requires mechanical ventilation. Afterwards, the patient is subjected to a thymectomy and thyroidectomy with a good postoperative evolution.

Tumores del timo y cirugía / Tumors of thymus and surgery

Los tumores del timo constituyen menos del 1 por ciento de todas las neoplasias, y es la cirugía el tratamiento de elección. Objetivos: conocer el tipo de tratamiento quirúrgico y la magnitud de la resección, así como la relación con el tamaño del tumor, el sangrado, el tiempo quirúrgico, la morbilidad y la mortalidad. Métodos: se realizó un estudio prospectivo en 22 pacientes con tumor mediastinal, que tuvieron criterios de cirugía durante el ingreso en los servicios de neurología o cirugía general del hospital Hermanos Ameijeiras, desde enero de 2007 hasta febrero de 2009. Los resultados se presentan en por cientos y se empleó el chi cuadrado en la relación de variables. Resultados: 12 fueron del sexo femenino (54,5 por ciento ). El tratamiento más empleado fue la esternotomía total en 9 pacientes (40,9 por ciento ), el tiempo quirúrgico varió de 50 a 260 min con mediana de 127,5, mientras el sangrado por encima de 100 mL estuvo asociado a un tiempo quirúrgico de 61 a 180 min (p= 0,036). Se complicaron 11 pacientes (50 por ciento ) y hubo 1 fallecido (4,5 por ciento ). El tamaño del tumor varió de 3,5 a 20 cm. El paciente con tumor neuroendocrino recidivó a los 10 meses, mientras los pacientes con timomas no muestran hasta la fecha recidiva local ni se ha comprobado actividad metastásica. Conclusión: la cirugía constituye el paso más importante en el tratamiento de los tumores mediastinales, y se logra, en la gran mayoría, la resección completa, a pesar del tamaño y la relación con estructuras vecinas(AU)

Introduction: the tumors of thymus account for less than 1 percent of all neoplasms and the choice treatment is the surgery. Objectives: to know the type of surgical treatment and the magnitude of resection, as well as the relationship with the tumor size, bleeding, surgical time and morbidity and mortality. Methods: a prospective study was conducted in 20 patients presenting with mediastinum tumor with surgery criteria over the admission in the services of neurology or general surgery of the Hermanos Ameijeiras Clinical Surgical Hospital from January, 2007 to February, 2009. Results are showed in percentages and variables relation Chi2 was the test used. Results: twelve patients were of female sex (54.5 percent). The more applied treatment was the total sternotomy in 9 patients (40.9 percent), surgical time varied from 50 to 260 min with a mean of 127,5, whereas the bleeding over of 100 mL was associated with a surgical time from 61 to 180 min (p= 0.036). Eleven patients have complications (50 percent) with a death (4.5 percent). The tumor size varied from 3,5 to 20 cm. The patients with neuroendocrine tumor have a relapse at 10 months, whereas in patients with thymoma until now there was neither relapse nor metastatic activity. Conclusion: surgery is the more important step in the treatment of mediastinum tumors and in most of them it is possible a total resection despite its size and the relationship with neighbouring structures(AU)

Myasthenia gravis: A rare cause of orthopnoea due to bilateral diaphragmatic paralysis.

A case of a 68-year-old patient with bronchial asthma who presented with orthopnoea and respiratory failure in supine position is presented.

Lúpus Eritematoso Sistêmico com fraqueza muscular por Miastenia Gravis / Systemic Lupus Erythematosus with muscle weakness due to Myasthenia Gravis

O Lúpus Eritematoso Sistêmico (LES) e a Miastenia Gravis (MG) são doenças autoimunes cuja associação em um mesmo paciente é raramente descrita. Essas patologias compartilham algumas características como acometimento de mulheres jovens, positividade para anticorpos antinucleares, evolução em períodos de exacerbações e remissões. O presente relato de caso analisa as possíveis hipóteses diagnósticas para um quadro clínico de ptose palpebral e fraqueza muscular proximal em uma paciente portadora de lúpus recente que evoluiu com MG associada.

Systemic lupus erythematosus (SLE) and myasthenia gravis (MG) are autoimmune diseases, whose association in the same patient is rarely reported. Both pathologies share the following characteristics: affect mainly young women; alternate exacerbation and remission periods; and have positive antinuclear antibody (ANA) test. This case report assesses possible diagnostic hypotheses for the clinical findings of eyelid ptosis and proximal muscle weakness in a female patient recently diagnosed with SLE, who evolved with associated MG.

Comparison of Clinical Manifestations between Patients with Ocular Myasthenia Gravis and Generalized Myasthenia Gravis

PURPOSE: To compare the clinical manifestations between patients with ocular myasthenia gravis and those with generalized myasthenia gravis (MG). METHODS: The medical records of 71 patients diagnosed with MG between January 1995 and December 2007 were reviewed. Demographics, sensitivities of diagnostic methods, the presence of systemic autoimmune diseases, ophthalmic complications caused by MG, and treatments were evaluated and compared. RESULTS: Fourteen patients (20%) were diagnosed with ocular MG and 57 patients (80%) with generalized MG. Sensitivities of anti-acetylcholine receptor antibody and repetitive nerve stimulation tests were significantly higher in the generalized MG group (84%, 89%) compared to those in the ocular MG group (50%, 54%) (p = 0.011, p = 0.008). The sensitivity of the neostigmine test was the highest in both groups (98% of generalized MG, 79% of ocular MG), and the difference between the two groups was borderline significant (p = 0.058). The most common symptoms were ptosis and diplopia, and both groups presented with pain, blurred vision, and tearing. Systemic autoimmune disease was more prominent in the generalized MG group (21%) than in the ocular MG group (14%), and steroid therapy was used more frequently in the generalized MG group (82%) than in the ocular MG group (57%). Ophthalmic complications associated with long-term steroid treatment were more profound in the generalized MG (30%) compared to those of the ocular MG (21%). CONCLUSIONS: The generalized MG group was associated with higher sensitivities to diagnostic tests, more systemic steroid use, higher ophthalmic complications caused by systemic autoimmune disease, and long-term steroid treatment compared to those of the ocular MG group.