Did the speech of patients with Myasthenia Gravis decline over 4 years? / Há declínio na fala de pacientes com Miastenia Gravis ao longo de 4 anos?

ABSTRACT Purpose To compare the speech and voice patterns of myasthenia gravis (MG) patients over four years, and correlate the results with clinical aspects of the disease. Methods Data was collected for 4 years. The clinical assessment tools included the Quantitative Myasthenia Gravis (QMG) score, the Myasthenia Gravis Foundation of America (MGFA) clinical classification, and the Myasthenia Gravis Quality of Life 15-item Scale (MG-QoL). To assess speech, the recorded speaking tasks were analyzed acoustically and given auditory-perceptual ratings. Sex (equal distribution) and age (p=0.949) were used as matching criteria in the final sample, which consisted of 10 individuals in the MG group (MGG) and 10 individuals in the control group (CG). Results After 4 years, the MG participants presented stable health status, increased mild and moderate dysarthria (from 40% to 90% of the subjects), and a significant deterioration in the respiration, phonation, and articulation subsystems. The acoustic analysis showed a decline in articulatory patterns (speech rate p=0.047, articulation rate p=0.007, mean syllable duration p=0.007) and vocal quality (increased jitter p=0.022). In the follow-up comparison, there was a significant difference between the phonation variables (shimmer and harmonic-to-noise ratio) of the MGG and CG. Conclusion The MG patients presented a decline in speech over four years and an increase in mild and moderate dysarthria. Despite presenting stable health status, their respiratory, phonatory, and articulatory subsystems worsened. There was no correlation between speech patterns and clinical characteristics of the disease (severity and motor scale).

RESUMO Objetivo Comparar o padrão de fala e voz de indivíduos com Miastenia Gravis (MG) em um intervalo de quatro anos e correlacionar com aspectos clínicos da doença. Método A coleta de dados foi realizada ao longo de 4 anos. A avaliação clínica foi composta pelo Quantitative Myasthenia Gravis Score (QMGS), pela Myasthenia Gravis Foundation of America Classification (MGFA) e pela escala de qualidade de vida para Miastenia Gravis (MG-QOL). A avaliação da fala foi composta por gravação de tarefas, análise perceptivo-auditiva e análise acústica. A amostra final foi composta por 10 indivíduos em MG e 10 indivíduos no grupo controle (GC), pareados por sexo (distribuição igualitária) e idade (p=0,949). Resultados Após 4 anos, os indivíduos com MG apresentaram estabilidade clínica, aumento do diagnóstico de disartria leve e moderada (de 40% para 90% dos sujeitos) e diminuição significativa no desempenho dos subsistemas da fala: respiração, fonação e articulação. Na análise acústica, houve declínio do padrão articulatório (taxa de fala p=0,047, taxa de articulação p=0,007, duração média das sílabas p=0,007) e qualidade vocal (jitter aumentado p=0,022). Houve diferença significativa nas variáveis fonatórias (shimmer e harmonic-to-noise ratio) entre os grupos MG e GC na comparação do seguimento. Conclusão Indivíduos com MG apresentaram declínio no padrão de fala em um intervalo de quatro anos, com aumento no número de disártricos (leve e moderado). Mesmo com a estabilidade da doença, houve piora dos subsistemas respiratório, fonatório e articulatório. Não houve correlação entre o padrão de fala e as características clínicas da doença (gravidade e escala motora).

Timectomía transesternal extendida en pacientes con miastenia grave / Extended Transsternal Thymectomy in Patients with Myasthenia Gravis

Introducción: La miastenia grave adquirida es un trastorno de la transmisión neuromuscular, causado por la unión de autoanticuerpos con los componentes de la placa neuromuscular, más frecuentemente con el receptor de acetilcolina. Objetivo: Evaluar los resultados quirúrgicos inmediatos, obtenidos con la timectomía extendida en pacientes con miastenia grave timomatosa y no timomatosa. Métodos: Se realizó un estudio observacional, prospectivo y descriptivo para evaluar los resultados postoperatorios inmediatos de 21 pacientes intervenidos por miastenia grave entre junio de 2015 y mayo de 2020. Las variables estudiadas fueron edad, sexo, lesiones tímicas asociadas y resultados inmediatos: complicaciones y mortalidad ocurridas hasta 30 días de la intervención. Los datos fueron obtenidos de una base en Microsoft Access. Resultados: Dieciséis (76,2 por ciento) pertenecían al sexo femenino y 5 (23, 8 por ciento) al masculino. El mayor número correspondió a la MG con timoma, seguido por la MG e hiperplasia tímica (8) y uno con MG y restos tímicos. Se complicaron tres pacientes (14,3 por ciento). Uno presentó dos complicaciones: enfisema subcutáneo y septicemia, el siguiente, fiebre sin causa aparente y, el último, infección superficial de la herida. No hubo fallecidos. Conclusiones: La timectomía transesternal extendida permite extirpar el timo y la mayor parte de los tejidos adiposo y tímico aberrante, en el cuello y el mediastino. En pacientes tratados por equipos multidisciplinarios, con experiencia en esta cirugía, las complicaciones y la mortalidad tempranas usualmente son favorables(AU)

Introduction: Acquired myasthenia gravis (MG) is a neuromuscular transmission disorder caused by the union of autoantibodies and the components of the neuromuscular plaque, most frequently with the acetylcholine receptor. Objective: To assess the immediate surgical outcomes of extended thymectomy in patients with thymomatous and nonthymomatous myasthenia gravis. Methods: An observational, prospective and descriptive study was carried out to assess the immediate postoperative outcomes of 21 patients operated on for myasthenia gravis between June 2015 and May 2020. The variables studied were age, sex, associated thymic lesions, and immediate outcomes: complications and mortality that occurred up to thirty days after the intervention. The data were obtained from a Microsoft Access database. Results: Sixteen (76.2 percent) belonged to the female sex and five (23.8 percent) to the male sex. The highest number corresponded to MG with thymoma, followed by MG and thymic hyperplasia (8) and one with MG and thymic remains. Three patients (14.3 percent) were complicated. One had two complications: subcutaneous emphysema and septicemia; the next had unexplained fever; and the last one had superficial wound infection. There were no deaths. Conclusions: Extended transsternal thymectomy allows removal of the thymus and most of the adipose and aberrant thymic tissues in the neck and mediastinum. In patients treated by multidisciplinary teams, with experience in this surgery, outcomes regarding early complications and mortality are usually favorable(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.

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).

Successful use of sugammadex for caesarean section in a patient with myasthenia gravis / O uso bem-sucedido de sugamadex para cesariana em paciente com miastenia grave

Abstract Myasthenia gravis is an autoimmune disorder that is characterized by muscle weakness that fluctuates, worsening with exertion, and improving with rest. Diagnosis of myasthenia gravis is made following clinical and physical examination and is confirmed by serum immunoassays to measure autoantibody levels. Myasthenia gravis especially when associated with pregnancy is a high-risk disease, and its course is unpredictable. We described the second report about use of sugammadex after rocuronium for a caesarean delivery with myasthenia gravis, but, unlike our case that formerly was diagnosed with myasthenia gravis, the patient was extubated on postoperative successfully and we did not encounter any respiratory problems.

Resumo Miastenia grave (MG) é uma doença autoimune caracterizada por fraqueza muscular que flutua, piora com o esforço físico e melhora com o repouso. O diagnóstico de MG é feito após exame clínico e físico e confirmado por imunoensaios séricos para medir os níveis de autoanticorpos. MG, especialmente quando associada à gravidez, é uma doença de alto risco e de curso imprevisível. Descrevemos o segundo relato sobre o uso de sugamadex após rocurônio para uma cesariana com miastenia grave, mas, ao contrário de nosso caso, que foi previamente diagnosticado com miastenia grave, a paciente foi extubada com sucesso no pós-operatório sem qualquer problema respiratório.

Takotsubo syndrome associated with myasthenic crisis. A case report / Síndrome de takotsubo asociado a crisis miasténica. Presentación de un caso

Abstract: Introduction: The takotsubo syndrome is a rare clinical entity commonly associated with elderly women (ratio 6:1), easily confused with an acute ischemic syndrome, strangely associated with a myasthenic crisis. It is characterized with chest pain, elevated biomarkers, ST segment elevation, absence of coronary flow obstruction, and a characteristic deformity (anteroapical dyskinesia) of the left ventricle; these changes associated with a stressor. Case report: A woman of 69 years, diagnosed with myasthenia gravis six years ago, who entered in the intensive care unit with a suspected diagnosis of acute coronary syndrome and respiratory failure which required mechanical ventilation. Coronary angiography discarded a coronary disease. Ventriculography revealed a systolic anteroapical deformation. Treatment was initiated with acetylcholinesterase inhibitors and plasmapheresis with partial response, it required the use of vasoactive amines, with a suitable progressive cardiovascular and neurological outcome, with echocardiographic resolution. Conclusions: The takotsubo syndrome can be associated with myasthenia gravis and myasthenic crisis. The prognosis depends on early diagnosis, appropriate differential diagnosis, immediate treatment of myasthenic crisis, and management of the hemodynamic consequences of the takotsubo syndrome.

Resumen: Introducción: El síndrome de takotsubo es una entidad clínica poco frecuente asociada comúnmente con las mujeres de edad avanzada (relación 6:1), puede fácilmente confundirse con un síndrome isquémico agudo, extrañamente asociado con una crisis miasténica. Se caracteriza por dolor torácico, biomarcadores elevados, elevación del segmento ST, ausencia de obstrucción del flujo coronario, y una deformidad característica (discinesia anteroapical) del ventrículo izquierdo; estos cambios asociados con un factor de estrés. Caso clínico: Una mujer de 69 años, con diagnóstico de miastenia gravis hace seis años, que ingresó en la Unidad de Cuidados Intensivos con un diagnóstico de sospecha de síndrome coronario agudo e insuficiencia respiratoria que requirió de ventilación mecánica. La angiografía descartó una enfermedad coronaria. La ventriculografía reveló una deformación anteroapical sistólica. Se inició el tratamiento con inhibidores de la acetilcolinesterasa y plasmaféresis con respuesta parcial, se requirió el uso de aminas vasoactivas, con un resultado progresivo cardiovascular y neurológico adecuado, con resolución ecocardiográfica. Conclusiones: El síndrome de takotsubo puede estar asociada con miastenia gravis y la crisis miasténica. El pronóstico depende del diagnóstico precoz, el diagnóstico diferencial adecuado, el tratamiento inmediato de la crisis miasténica, y la gestión de las consecuencias hemodinámicas del síndrome takotsubo.

Síntomas de miastenia grave en un paciente con antecedente de timectomía por timoma invasor / Symptoms of myasthenia gravis in a patient with a history of thymectomy for invasive thymoma

Introducción. La miastenia grave es una enfermedad autoinmunitaria mediada por anticuerpos. Entre 10 y 15 % de quienes la padecen tienen timoma y su presencia se asocia con una mayor gravedad de los síntomas, crisis miasténicas y fracaso del tratamiento de primera línea. La timectomía se recomienda en pacientes jóvenes con miastenia grave generalizada y en todos los pacientes con timoma. Caso clínico. Se presenta el caso de una mujer de 43 años que, en el 2005, presentó una primera crisis miasténica asociada con un timoma invasor que se trató con timectomía y radioterapia. Durante los siguientes tres años, presentó síntomas graves y dos crisis más, que obligaron a suministrarle respiración mecánica asistida e inmunoglobulina. Al cabo del tratamiento, no se evidenciaron signos de recurrencia en las tomografías de tórax con contraste. Entre el 2009 y el 2012, la gravedad de los síntomas fue menor. En el 2013, estos se exacerbaron y una resonancia magnética de tórax con contraste reveló una lesión en el mediastino anterior, ya observada en el 2011, sugestiva de tejido residual o fibrosis. Se inició el tratamiento usual con inmunoglobulina y se hizo una tomografía por emisión de positrones cuyos resultados no fueron concluyentes, por lo que se llevó a cabo una nueva resección y se constató que no había recurrencia del tumor. Conclusiones. Los pacientes con miastenia grave y aquellos con timoma asociado, deben someterse a la timectomía como parte del tratamiento. Sin embargo, la exacerbación de los síntomas o su reaparición después del procedimiento no necesariamente implica una nueva alteración en el timo.

Introduction: Myasthenia gravis is an antibody-mediated autoimmune disease. Approximately 10-15% of patients present with a thymoma, the presence of which is associated with greater severity of symptoms, myasthenic crisis, and irresponsiveness to front-line therapy. A thymectomy is recommended in young patients with generalized myasthenia gravis and in all patients presenting with thymoma. Clinical case: The patient was a 43-year-old woman, who first showed symptoms of myasthenic crisis in 2005 and presented with invasive thymoma managed with thymectomy and radiotherapy. In the subsequent three years, the patient presented with severe symptoms and two myasthenic crises that required mechanical ventilation and immunoglobulin treatment. Contrast chest computed tomography examinations showed no recurrence. Between 2009 and 2012, the patient experienced decreased symptom severity. In 2013, the patient presented with an exacerbation of symptoms; a contrast chest magnetic resonance scan showed a lesion in the anterior mediastinum, previously observed in 2011, suggestive of residual tissue as opposed to fibrosis. Regular management was started with immunoglobulins; a positron emission tomography scan was inconclusive, requiring a new resection, which showed no evidence of tumor recurrence. Conclusions: Patients with myasthenia gravis and those with myasthenia-related thymoma both share thymectomy as an element of treatment. However, following the procedure, exacerbation or reappearance of symptoms does not necessarily represent new alterations in the thymus.

Miastenia grave generalizada: reporte de un caso / Generalized-myasthenia gravis: a case report

Mujer de 17 años de edad, con diagnostico reciente de miastenia gravis, en tratamiento irregular con piridostigmina y prednisona; que acudió por presentar debilidad muscular generalizada, disfagia, diplopía y ptosis palpebral. El examen físico encontró una tiroides aumentada de tamaño. Los exámenes auxiliares mostraron T4 libre y TSH normales, pero los anticuerpos antirreceptor de acetilcolina fueron positivos. Se realizó timectomía, pero en el postoperatorio, la paciente presentó crisis miasténica con insuficiencia respiratoria, por lo que fue sometida a ventilación mecánica. Después de dos semanas, la paciente fue dada de alta sin presentar debilidad muscular, disfagia o ptosis palpebral. El tratamiento al alta fue piridostigmina. La miastenia grave es una enfermedad autoinmune que se caracteriza por debilidad muscular fluctuante. En los casos de inicio temprano (menores de 40 años), esta asociada a hiperplasia del timo y otras enfermedades autoinmunes. La timectomía es el tratamiento de elección en pacientes menores de 50 años.

A 17-year-old woman presented with generalized weakness, dysphagia, ptosis, and diplopia. Recently, she was diagnosed with myasthenia gravis, and her treatment included pyridostigmine and prednisone. Physical examination showed goiter. Thyroid hormones were normal, but the autoantibodies against the postsynaptic acetylcholine receptor were positive. She underwent thymectomy, and developed respiratory insufficiency secondary to myasthenic crisis. After two weeks, she was discharged without complaints. Her long-term treatment included pyridostigmine. Myasthenia gravis is a rare autoimmune disease. The clinical hallmark is fatigable weakness. Patients with early- onset disease (< 40 years), are more often female, have enlarged, hyperplastic thymus glands, and other organ- specific autoantibodies might be present. Thymectomy is the treatment of choice in patients with disease onset before the age of 50 years.

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

Miastenia gravis del adulto / Myathenia graviss del adultery

La miastenia gravis (MG) es una enfermedad neuromuscularautoinmuney crónica caracterizada por grados variables de debilidad de los músculos esqueléticos del cuerpo. La denominación proviene del latín y el griego, y significa literalmente «debilidad muscular grave¼. Debuta con un cuadro insidioso caracterizado por ptosis palpebral bilateral y disminución de fuerza muscular, que rápidamente se recuperan con el descanso pero que reaparece al reiniciar el ejercicio. Suele iniciarse en los músculos peri oculares. En pocas ocasiones el inicio es agudo.

Myasthenia gravis (MG) is a chronic autoimmune neuromuscular disease characterized by varying degrees of weakness of the skeletal muscles of the body. The name comes from Latin and Greek, and literally means "grave muscle weakness. " Insidious debut with a picture characterize by bilateral ptosis and decreased muscle strength, which quickly recover with rest but comes back when you restart the exercise. It usually begins in the periocular muscles. Rarely, onset is acute.

Miastenia grave ocular en el anciano: presentación de un caso / Ocular myasthenia gravis in old people: a case report

La miastenia grave es un trastorno de la transmisión neuromuscular de origen autoinmunitario, producida por la disminución del número de los receptores de acetilcolina de la placa motora, se caracteriza por debilidad y fatiga muscular fluctuante principalmente de los músculos inervados por los pares craneales, su manifestación es evidente durante la actividad continuada y mejora con el reposo o tras la administración de medicamentos anticolinestarásicos. Se presenta un paciente de 79 años de edad, raza blanca, sexo masculino, con antecedente de padecer de diabetes mellitus tipo II para lo cual se controla con dieta, quien hace aproximadamente dos meses nota caída de los dos parpados, primero el derecho y después el izquierdo, además presenta dificultad para tragar, principalmente los alimentos sólidos y con el transcurso del día siente debilidad muscular, motivo por el cual asiste a consulta. Al examen físico se observa ptosis palpebral bilateral, parálisis del tercer par en su porción extrínseca. Se presenta este caso por su aparición en una edad y sexo no tan frecuente.

Myasthenia gravis is an autoimmune neuromuscular disorder of neuromuscular transmission produced by the decrease of the acetylcholine receptors numbers of motor plaque, which is characterized by fluctuating muscle weakness and fatigue, mainly from innervated muscles by the cranial pairs. Its manifestation is evident during continuous activity and it improves after periods of rest or by the administration of anti-cholinesterase medications. A case of a 79 year-old black male with mellitus type II diabetes antecedents who is controlled by means of a diet is presented. At about two months he noticed a drooping of the right eyelid first and the left later. Also, he has difficulty in swallowing, basically, solid food and during the day he feels muscular weakness, so he visits the doctor. In the physical examination bilateral ptosis is observed, paralysis of the third pair in its extrinsic portion.

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)

Miastenia grave y embarazo: presentación de un caso / Myasthenia gravis and pregnancy: presentation of a case

La miastenia grave (MG) es una enfermedad autoinmune de la placa motora del músculo estriado, caracterizada por fatiga y debilidad muscular fluctuante. Su prevalencia mundial ha aumentado desde 1950. En Cuba ha sido reportada una tasa de prevalencia e incidencia de 29,2 y 4,5, respectivamente. Su evolución durante el embarazo es totalmente impredecible. Del 10 al 20 por ciento de los recién nacidos de madres miasténicas pueden padecerla. Se reportó el caso de una embarazada de 32 años de edad asistida en el Hospital Ginecobstétrico Docente Julio R. Alfonso Medina, de Matanzas, en 2010, a la cual se le diagnosticó en el primer trimestre de gestación miastenia grave estadio IIb; iniciándose tratamiento con prednisona y piridostigmina. No hubo morbilidad perinatal, ni complicaciones maternas al recibir atención por un equipo multidisciplinario.

The myasthenia gravis is an autoimmune disease of the striated muscle motor plate, characterized by tiredness and weakness of the fluctuant muscle. Its prevalence around the world has increased since 1950. The prevalence and incidence rates reported in Cuba are 29,2 and 4,5 respectively. Its evolution during pregnancy is totally unpredictable. From 10 to 20 percent of the newborns from myastenic mothers may suffer it. We reported the case of a pregnant woman aged 32 years old, assisted at the Teaching Gynecoobstetric Hospital Julio R. Alfonso Medina of Matanzas, in 2010. She was diagnosed in the first gestational trimester with myasthenia gravis stage IIb, beginning the treatment with prednisone and pyridostigmine. There was not perinatal morbidity or maternal complications when receiving care from a multidisciplinary team.

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.

Miastenia grave ocular / Severe ocular myastenia

La miastenia grave es una enfermedad autoinmune de la unión neuromuscular que se caracteriza por la variabilidad de la debilidad muscular, que empeora o está provocada por el ejercicio del músculo o de los grupos musculares implicados, mientras que el descanso la mejora. La primera descripción de la enfermedad data de 1672, su relación con una afección del timo no se estableció hasta 1901. La enfermedad es poco frecuente, afecta a todas las etnias por igual, y no tiene predilección geográfica. Se reporta un paciente masculino de 28 años de edad atendido en el Servicio de Oftalmología Pediátrica del Instituto Cubano de Oftalmología Ramón Pando Ferrer por presentar visión doble, ptosis palpebral ligera en ojo derecho que empeora con el transcurso del día. Por el interrogatorio y el cuadro clínico se sospecha de una miastenia grave, se remite al clínico y el neurólogo. En los estudios realizados de neuroimágenes, tomografía y electroencefalograma no se encontraron alteraciones. Se decide valorar el caso con el servicio de Neuroftalmología de nuestro centro y por el Instituto de Neurología y es confirmado el diagnóstico

Myasthenia gravis is an autoimmune illness of the neuromuscular joint that is characterized by the variability of the muscular weakness that worsens or caused by exercising of the muscle or the involved muscular groups; resting helps in improving the condition of the muscle. The first description of the illness dates back to 1672, but its linking to a thymus disorder was not discovered until 1901. This illness is not very frequent, affects equally to all the races, and it does not predominate in any particular geographic region. This paper presented a male patient aged 28 years who went to the Ophthalmologic Service at Ramón Pando Ferrer Cuban Institute of Ophthalmology because he suffered from double vision, slight palpebral ptosis in his right eye that became worse as the day went by. After the questions asked to the patient and the analysis of the clinical picture, it was suspected that the patient was affected by myasthenia gravis, and then he was referred to the clinician and the neurologist. The studies based on the neuroimaging analysis, the tomography and the electroencephalogram did not show any alteration. It was decided to evaluate the case with the assistance of the neurophthalmology service in our center and the Institute of Neurology; the diagnosis was confirmed

Surgical correction of strabismus in Lambert-Eaton myasthenic syndrome: case reports / Correção cirúrgica do estrabismo na síndrome de Lambert-Eaton: relato de dois casos

Lambert-Eaton myasthenic syndrome is a rare and acquired autoimmune disorder. We describe two female patients with medial rectus paresis as the only ocular manifestation. After a unilateral medial rectus recession and lateral rectus resection procedure, both patients recovered normal adduction. To our knowledge, this is the first report of surgery for extraocular muscle paresis in Lambert-Eaton myasthenic syndrome.

A síndrome de Lambert-Eaton é um distúrbio autoimune raro e adquirido. Apresentamos duas pacientes com paresia do reto medial como única manifestação ocular. Após retrocesso do reto lateral e ressecção do reto medial, unilateral, ambas as pacientes apresentaram normalização da adução. Até onde sabemos, este é o primeiro relato de cirurgia para paresia do reto medial na síndrome de Lambert-Eaton.

Haptoglobin study in myasthenia gravis / Estudo sobre a haptoglobina na miastenia grave

OBJECTIVE: A cross-sectional study of haptoglobin (Hp) in myasthenia gravis (MG) was designed, with the objective to identify its values and correlate them with different disease status. METHOD: 46 patients were enrolled in the study, all having disease severity established according to the quantitative myasthenia gravis strength scores (QMGSS). Based on the functional scale determined by Myasthenia Gravis Foundation of America (MGFA) recommendations, patients were classified as having: complete stable remission (CSR; n=10); minimal manifestations-0 (MM0; n=6), minimal manifestations-1 (MM1; n=4); pharmacological remission (PR; n=6). Two other groups participated: thymomatous patients (T; n=10) and patients without imunosuppression or thymectomy, until the assessment for Hp (WIT; n=10). Hp dosage was done by immunonephelometry, blindly to clinical data. Student's t-test, Anova test and linear regression were employed for statistical analyses. RESULTS: Statistically significant differences occurred between CSR+MM0xWIT groups (86.62x157.57, p<0.001) and PR+MM1xWIT groups (73.93x157.57, p<0.001). Linear regression showed correlation between Hp levels and QMGSS (r=0.759, p<0.001). CONCLUSION: Our results suggest that Hp may be useful in clinical practice as a disease severity marker in MG.

OBJECTIVO: Desenhou-se estudo transversal sobre a haptoglobina (Hp) na miastenia grave (MG) com o objetivo de identificar seus valores e correlacioná-los a diferentes condições na doença. MÉTODO: 46 pacientes foram incluídos, todos tendo a gravidade da doença estabelecida segundo escores internacionais (QMGSS). Os pacientes tiveram seu estado funcional determinado de acordo com a Myasthenia Gravis Foundation of América (MGFA) e classificados em: remissão completa estável (CSR; n=10); mínima manifestação-0 (MM0; n=6), mínima manifestação-1 (MM1; n=4); remissão farmacológica (PR; n=6). Dois outros grupos participaram: pacientes timomatosos (T; n=10) e pacientes sem imunossupressão ou timectomia, até o momento da inclusão no estudo (WIT; n=10). A dosagem de Hp foi realizada por imunonefelometria, de modo cego quanto à clínica. As análises estatísticas incluíram o teste de Student, Anova e regressão linear. RESULTADOS: Observou-se diferença significativa entre os grupos CSR+MM0xWIT (86,62x157,57, p<0,001) e entre PR+MM1xWIT (73,93x157,57, p<0,001). A regressão linear mostrou correlação positiva entre os valores de Hp e os escores QMGSS (r=0,759, p<0,001). CONCLUSÃO: O estudo sugere que valores altos de Hp se correlacionaram a maior gravidade da MG.

Megaesôfago secundário a miastenia grave em umacadela da raça Pastor Alemão / Megaesophagus secondary to myasthenia gravis in a female German shepherd dog

Megaesôfago é uma causa comum de regurgitação sendo uma das possíveis etiologias a miastenia gravecaracterizada por uma desordem neuromuscular que resulta em fraqueza dos músculos esqueléticos, doesôfago, da laringe e da faringe. O presente relato descreve um caso de uma cadela da raça PastorAlemão, com histórico de fraqueza muscular e vômitos freqüentes, que foi diagnosticada comomegaesôfago secundário à miastenia grave. A radiografia do animal apresentou um acentuadomegaesôfago cervical e torácico. Após a instituição do protocolo terapêutico recomendado o animalapresentou melhora visível ao caminhar, porém as regurgitações pioraram e começou a apresentar tosse.Foi realizado novo exame radiográfico com presença, além de megaesôfago, de pneumonia aspirativa. Aproprietária optou pela eutanásia do animal e a necropsia revelou severa pneumonia e dilatação acentuadada porção caudal do esôfago, caracterizando um megaesôfago de grande dimensão, colaborando comsubsídios para um prognóstico desfavorável do caso.

Megaesophagus is a common cause of regurgitation being one of the possible etiologies the myasthenia gravis characterized by a neuromuscular disorder that result in weakness of the skeletal muscles, of the esophagus, of the larynx and of the pharynx. The present report describes a case of a female German shepherd dog, with report of muscles weakness and frequent vomits, which was diagnosed as a megaesophagus secondary to myasthenia gravis. The radiograph of the animal presented an accentuated cervical and thoracic megaesophagus. After the institution of the recommended therapeutic protocol the animal presented visible improvement when walking, however the regurgitations worsened and it began to present cough. New radiograph was accomplished with presence, besides megaesophagus, of aspiration pneumonia. The owner opted for the euthanasia of the animal and the necropsy revealed severe pneumonia and an accentuated dilation of the posterior portion of the esophagus, collaborating with subsidies for an unfavorable prognostic of the case.