Leticia de la Caridad Christian López
Full Text Available La miastenia gravis es una entidad clínica de origen autoinmune, cuya terapia habitual se realiza con drogas anticolinérgicas, la timectomía o la terapia con esteroides. Fue el objetivo del trabajo el conocer los cambios que se producen en el tamaño del área tímica, con el uso de esteroides, en una paciente de 2 años de edad, que presentaba miastenia gravis juvenil, con una hiperplasia tímica. Esta glándula alcanzó un área máxima de 1 928 mm. Con el uso de la prednisona a 60 mg por semanas se produjo una timectomía medicamentosa, con la reducción del área tímica a 439 mm y remisión total de la sintomatología. Se realizaron las mediciones periódicamente durante un año de los cambios del área de este órgano. No se produjeron recaídas de la enfermedad de base. La ultrasonografía demostró ser un método útil, por ser rápido, barato y no invasiva y permite un ajuste adecuado de la dosis de esteroides que se administrará.Myasthenia gravis is a clinical agent of autoimmune origin, whose habitual therapy includes anticholinergic drugs, thymectomy, or therapy with steroids. The objective of this paper was to know the changes that occur in the size of the thymic area in a 2-year-old patient with juvenile myasthenia gravis and with thymic hyperplasia. This gland reached a maximum area of 1 928 mm and with the use of 60 mg of prednisone per week a medicamentosus thymectomy was performed with the reduction of the thymic area to 439 mm and total remission of the symptomatology. The changes of the area of this organ were periodically measured during a year. There were no relapses of the base disease. The ultrasonography proved to be a useful method, since it is fast, cheap and noninvasive and allows an adequate control of the steroids dose to be administered.
Myasthenia gravis is a disease that causes weakness in your voluntary muscles. These are the muscles that ... gets worse with activity, and better with rest. Myasthenia gravis is an autoimmune disease. Your body's immune ...
Kishimoto, Gustavo; Murakami, Koji; Con, Sergio A.; Yamasaki, Erena; Domeki, Yasushi; Tsubaki, Masahiro; Sakamoto, Setsu
El seguimiento postoperatorio tiene un rol importante para la sobrevida del paciente después de la resección curativa del cáncer colorrectal. OBJETIVOS: Describir las características del seguimiento con tomografía por emisión de positrones (PET) integrado a tomografía axial computarizada (CT) (PET/CT) y su impacto en los sobrevivientes de cáncer colorrectal (CCR) posterior a la resección con intención curativa en un hospital universitario en la prefectura de Tochigi, Japón. MATERIAL Y MÉTODOS...
Sâmia Araújo de Sousa Studart
Full Text Available 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.
Full Text Available
Introducción: El virus del papiloma humano es el principal factor de riesgo asociado con el desarrollo de cáncer cervical, uno de los cánceres más comunes en mujeres colombianas. En Colombia, donde las tasas de incidencia de cáncer cervical son altas, se tiene poca información acerca de la prevalencia de la infección y no se tienen datos de seguimiento sobre la persistencia o eliminación de la infección y los factores de riesgo asociados. En este reporte se presentan los resultados de la prevalencia de la infección por HPV y los factores de riesgo asociados en mujeres que tuvieron una citología normal al comienzo del estudio así como el seguimiento de 227 mujeres positivas para la infección por HPV durante un periodo de cinco años, en la ciudad de Bogotá, Colombia.
Materiales y Métodos: 1859 mujeres con citología normal firmaron un consentimiento escrito y respondieron a un cuestionario estructurado. La presencia de HPV fue analizada mediante PCR con el uso de los iniciadores GP5+/ GP6+, que amplifican para un fragmento de 140 pb de la región L1/HPV (1 y tipificadas por medio del ensayo de EIA para la identificación de 37 diferentes tipos de HPV (2.
Se calcularon riesgos relativos para eliminación de la infección por HPV mediante el uso de curvas de supervivencia.
Resultados: La prevalencia de la infección fue de 14.8%; 9% de las mujeres fueron infectadas por HPV de alto riesgo, 3.1% por HPV de bajo riesgo, 2.3% por ambos tipos virales y 0.4% por HPV indeterminados. Se identificaron 32 diferentes tipos de HPV, siendo el HPV16, 58, 56, 81 y 18, los tipos mas comunes. La prevalencia de la infección por HPVs de alto riesgo entre
... Institutes of Health (NIH), the leading supporter of biomedical research in the world. Although there is no cure ... understand the fundamental processes in neuromuscular development. This research could reveal new therapies for neuromuscular diseases like myasthenia gravis. Researchers are ...
... that medications can be discontinued. In a few cases, the severe weakness of myasthenia gravis may cause respiratory failure, which requires immediate emergency medical care. Clinical Trials Throughout the U.S. and Worldwide NINDS Clinical ...
Oscar Papazian; Israel Alfonso; Nayle Araguez
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 nerv...
Resources - myasthenia gravis ... The following organizations provide information on myasthenia gravis : Myasthenia Gravis Foundation of America -- www.myasthenia.org National Institute of Neurological Disorders and Stroke -- www.ninds.nih.gov/Disorders/Patient- ...
Rullán Silva, Cristóbal Daniel
La defoliación por insectos es la causa de daño más común que afecta a la salud forestal de coníferas y frondosas. Varios estudios han mostrado que el actual calentamiento global puede conducir a un incremento en la frecuencia, severidad y extensión de daños producidos por plagas forestales, con consecuencias productivas y ecológicas predecibles. La teledetección de defoliaciones por insectos se basa en la integración de los datos de campo con las capacidades espectrales y espaciales de los s...
Full Text Available Hybrid materials have been prepared in this work through the reactions of Si and Ti alkoxides (TEOS and TBT, respectively and polydimethil siloxane (PDMS. These reactions have been studied by means of FT-IR spectroscopy during the whole reaction time. The hydrolysis of TEOS molecule has been followed by the 880 cm-1 band, and the self-condensation reactions through the 1180 and 1150 cm-1 bands. Polycondesation reaction between Si-OH groups and PDMS molecules has been followed by the 850 cm-1 band. On the other hand, the hydrolysis reaction of TBT and the self-condensation of Ti-OH groups have been followed by the 1130 and 770-510 cm-1 bands, respectively. Finally the condensation reaction between Si-OH and Ti-OH groups have been studied by the 936 cm-1 band. Results have shown that hydrolysis and condensation reactions are depending on TBT concentration. The formation of Si-O-Si cross-linked structures increases with the TBT concentrations in the reaction. The selfcondensation reaction of Si-OH grups or Ti-OH grous is very reapid forming Si-O-Si and Ti-O-Ti bonds, respectively. However, the Si-O-Ti bonds which are formed during the first moments of reaction are also rapidly broken due to H2O molecules or the reaction medium. The evolution of PDMS linear and cyclic molecules is also studied.
Se han preparado materiales híbridos por medio de reacciones de hidrólisis y condensación de alcóxidos de Si y Ti (TEOS y TBT, respectivamente y de reacciones de copolimerización de éstos con polidimetilsiloxano (PDMS. Se han estudiado las citadas reacciones mediante espectroscopia FT-IR, desde el mismo comienzo hasta la obtención del material final. La hidrólisis del TEOS así como la autocondensación del os grupos Si-OH generados tanto para formar cadenas entrecruzadas como lineales se han seguido mediante las bandas situadas a 880, 1180 y 1150 cm-1, respectivamente. La policondensación de dichos grupos con PDMS se ha seguido por la banda a
Sánchez-Salmón, Aida; Ruibal, Álvaro
La Tomografía por Emisión de Positrones (PET) es una técnica de medicina nuclear, no invasiva, que utiliza radiofármacos y cámaras de detección de radiaciones algo diferentes a las empleadas en otras exploraciones isotópicas. La mayoría de las indicaciones son oncológicas, si bien, en nefrourología su uso ha sido menor debido a las características del radiofármaco hasta ahora más comúnmente usado, la 18F-FlúorDeoxiGlucosa (18F-FDG), un análogo de la glucosa que se elimina por vía renal, lo qu...
... increase the risk of myasthenia gravis , but the identity of these genes is unknown. Many factors likely ... Myasthenia Gravis Fact Sheet MalaCards: myasthenia gravis Merck Manual Home Health Handbook for Patients and Caregivers Myasthenia ...
Martínez Torre, S; Gómez Molinero, I; Martínez Girón, R
Myasthenia gravis is one of the most common disorders that affect neuromuscular transmission. It is currently one of the most understood and characterised autoimmune disorders Its typical symptoms are fluctuating weakness and fatigue that affects a combination of ocular muscles, bulbar functions, as well as limb and respiratory muscles, which are due to an immune attack against the postsynaptic membrane of the neuromuscular junction. The diagnosis of myasthenia gravis is based on clinical and serological test. It is a disease that can be effectively controlled with the current therapeutic lines, even achieving a complete remission. An update of this interesting disorder is now presented. Copyright © 2018 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España, S.L.U. All rights reserved.
Osmar Antonio Centurión
Full Text Available La técnica de mapeo endocárdico durante el estudio electrofisiológico ha sido extensamente empleada en el análisis de las taquicardias paroxísticas supraventriculares (TPS. Mediante este mapeo endocárdico y el análisis meticuloso de los electrogramas endocárdicos y los respectivos intervalos de conducción, es posible localizar el sitio de origen de las arritmias y así facilitar su tratamiento ablativo por medio de catéteres de radiofrecuencia. Se describe el caso de un hombre de 29 años con síndrome de Wolff-Parkinson-White (WPW que manifiestó que presentaba varios episodios documentados de TPS con serio compromiso hemodinámico que motivaba su frecuente internación en terapia intensiva. El mapeo electrofisiológico endocárdico meticuloso demostró la presencia de un haz anómalo de Kent auriculoventricular izquierdo en posición posteroseptal. El período refractario absoluto del haz anómalo de Kent fue de 240 ms. La primera emisión de radiofrecuencia a través de un catéter adecuadamente posicionado previo terminó la taquicardia. La taquicardia permaneció no inducible a partir de entonces. En estas dos décadas de seguimiento clínico, el paciente no ha presentado ni un solo episodio de taquicardia. La curación definitiva generada por la ablación del haz anómalo de Kent ha proporcionado un cambio drástico, total y beneficioso en la calidad de vida al paciente. Los beneficios clínicos y socioeconómicos son mayores cuanto más temprano en la evolución se realice el procedimiento de ablación de arritmias.
I. Díaz de Tuesta Revilla
Conclusiones: Sin costes adicionales para un hospital razonablemente informatizado, la telemedicina ahorra tiempo, mejora el acceso al especialista, adelanta decisiones y evita desplazamientos para trivialidades. Mejora la calidad. Es el futuro en la toma de decisiones.
Full Text Available 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
Good nutrition is important for everyone. This is especially true when you have a chronic disorder like myasthenia gravis ( ... difficulty with chewing and swallowing may interfere with nutrition and create safety issues. Diet modifications may be ...
... are individualized There are many effective treatments for myasthenia gravis. Spontaneous improvement and even remission (although uncommon) ... Created by Kellen Interactive Web Design © copyright 2010 Myasthenia Gravis Foundation of America, Inc.
This patent describes an improvement in a process for diagnosing myasthenia gravis. The process comprises the steps of preparing a complex of acetycholine receptor protein, toxin and a radioactive isotope, incubating the complex with a serum sample from a patient so as to join antibodies engendered in connection with myasthenia gravis to the complex, precipitating the complex joined with antibody with anti-immunoglobulin and measuring radioactivity, from the radioactive isotope, of the precipitated complex. The improvement is that the acetylcholine receptor protein is derived from cells of the TE671 Line
This patent describes an improvement in a process for diagnosing myasthenia gravis. The process comprises the steps of preparing a complex of acetycholine receptor protein, toxin and a radioactive isotope, incubating the complex with a serum sample from a patient so as to join antibodies engendered in connection with myasthenia gravis to the complex, precipitating the complex joined with antibody with anti-immunoglobulin and measuring radioactivity, from the radioactive isotope, of the precipitated complex. The improvement is that the acetylcholine receptor protein is derived from cells of the TE671 Line.
Weijnen, FG; Kuks, JBM; van der Glas, HW; Oudenaarde, [No Value; Bosman, F
Masticatory muscle electromyograms (EMGs) were recorded while patients with bulbar myasthenia gravis chewed artificial food and compared with those of patients with ocular myasthenia gravis, patients in clinical remission who had previously suffered from bulbar myasthenia gravis and healthy
Facio, María Laura; Madalena, Leticia; Bacqué, María del Carmen; Idiarte, Laura; Pandolfo, Marcela; Angerosa, Margarita; Alejandre, Mariel; Fraind, Susana; Bresciani, Pablo; Pizzolato, Marco
La nefropatía crónica del trasplante (NCT) se caracteriza por fibrosis intersticial y atrofia tubular, pero su etiología es diversa. El objetivo del trabajo fue evaluar el seguimiento cualitativo de proteínas urinarias en pacientes con más de seis años de trasplante renal y compararlo con parámetros de laboratorio y con biopsia renal. Se evaluaron 17 pacientes durante un año, a través de creatinina sérica, proteinuria y fraccionamiento proteico por electroforesis en geles de poliacrilamida (S...
Swick, H.M.; Preston, D.F.; McQuillen, M.P.
A study was conducted to determine whether 67 Ga scans could be used for the detection of thymomas and to investigate the activity of the thymus gland in patients with myasthenia gravis. Scans of the anterior mediastinum proved to be a reliable way to detect thymomas. The scans were positive in eight patients including three with myasthenia gravis and histologically proved thymomas, three others with severe myasthenia gravis and thymic tumors, and two with histologically proved thymomas not associated with myasthenia. Activity on 67 Ga scans was not directly related to the increased activity of the thymus gland that is presumed to be associated with myasthenia gravis
Swick, H.M. (Univ. of Kentucky, Lexington); Preston, D.F.; McQuillen, M.P.
A study was conducted to determine whether /sup 67/Ga scans could be used for the detection of thymomas and to investigate the activity of the thymus gland in patients with myasthenia gravis. Scans of the anterior mediastinum proved to be a reliable way to detect thymomas. The scans were positive in eight patients including three with myasthenia gravis and histologically proved thymomas, three others with severe myasthenia gravis and thymic tumors, and two with histologically proved thymomas not associated with myasthenia. Activity on /sup 67/Ga scans was not directly related to the increased activity of the thymus gland that is presumed to be associated with myasthenia gravis. (HLW)
... 9 Animals and Animal Products 2 2010-01-01 2010-01-01 false Beef with gravy and gravy with beef. 319.313 Section 319.313 Animals and Animal Products FOOD SAFETY AND INSPECTION SERVICE, DEPARTMENT OF... Dehydrated Meat Food Products § 319.313 Beef with gravy and gravy with beef. “Beef with Gravy” and “Gravy...
Soyoral, Lokman; Goktas, Ugur; Cegin, Muhammed Bilal; Baydi, Volkan
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. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Publicado por Elsevier Editora Ltda. All rights reserved.
Aragonès, J M; Altimiras, J; Roura, P; Alonso, F; Bufill, E; Munmany, A; Alfonso, S; Illa, I
The reported prevalence of myasthenia gravis ranges between 5 and 24 cases per 100,000, and people over 65years account for less than 50% of all cases. The prevalence and clinical characteristics of myasthenia gravis in the county of Osona were studied in patients younger and older than 65. The study draws from the county-based prospective myasthenia gravis register implemented by the Neurology Department at Hospital General de Vic in 1991. The prevalence of myasthenia gravis was 32.89×10 5 inhabitants (95%CI, 23.86-41.91). The standardized prevalence (European population) was 35.47×10 5 inhabitants (95%CI, 26.10-44.84). The ratio of women to men was 1.3. Overall, the group of patients older than 65 accounted for 62.75% of all cases. The prevalence of myasthenia gravis increased considerably in older age groups. No cases were registered among patients under 25years old, prevalence was 21.87×10 5 in the 25 to 64 age group, and prevalence in patients over 65 years increased to 122.35×10 5 . The clinical characteristics prior to treatment and at the cut-off date are similar (P>.05) in patients younger than 65 and those aged 65 and older. These figures show the highest prevalence rate reported to date. This high prevalence is due to the rate observed among patients older than 65. These results provide a new warning that myasthenia gravis may be underdiagnosed in the elderly population. Copyright © 2014 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.
Cierre espontáneo de agujero macular idiopático:: seguimiento por tomografía de coherencia óptica y microperimetría MP1 Spontaneous closure of the idiopathic macular hole.: Follow-up of this case by optical coherence tomography and microperimetry MP1
Julio César Molina Martín
Full Text Available Se presenta un caso de cierre espontáneo de agujero macular idiopático seguido por tomografía de coherencia óptica y microperimetría MP1 antes y después del cierre. El cierre espontáneo de un estadio 4 no ocurre con frecuencia, sin embargo, puede aparecer fundamentalmente en pacientes con diámetro superior del agujero menor a 150 micras. La tomografía de coherencia óptica y la microperimetría constituyen herramientas útiles en el diagnóstico, pronóstico y seguimiento de esta entidad.The case of spontaneous closure of a stage 4 idiopathic macular hole at followed up by Optical Coherence Tomography and microperimetry MP1 before and after the closure was presented. The spontaneous closure of a stage 4 macular hole is rare but it can occur in patients with hole upper diameters less than 150 µm. The OCT and the microperimetry MP1 are very useful tools in the diagnosis, prognosis and follow-up of this maculopathy.
Adriana Patricia Suraniti
Full Text Available Miastenia Gravis is a neuromuscular disease caused by auto antibodies. Early Clinical and biochemical diagnosis and treatment is demanded in the assurementof quality and time of life in all dogs. In this study we describe the conventional diagnosis methods and therapy in 32 dogs with suspected myasthenia gravis and propose the administration of bromide of piridostigmin as another use full diagnosis method in dogs.
Eric Efrain Solano-Uscanga
Full Text Available En muchas de las veces, las instrucciones para realizar tareas correspondientes a cursos online, a distancia o virtuales no son entendidas adecuadamente. El uso de instrucciones es común en los diferentes ámbitos donde se desenvuelve el ser humano, aunque no todas las instrucciones tienen las mismas cualidades generando, por tanto, diferentes efectos sobre la conducta. En el presente estudio se analiza la relación entre la complejidad de la tarea y el seguimiento de instrucciones empleando como pretexto la construcción de diseños con piezas Lego con cuatro niveles de complejidad distintos. Se trabaja con 15 estudiantes universitarios, empleando listas de chequeo para evaluar el éxito en el seguimiento de instrucciones. Los resultados muestran que un mejor seguimiento de instrucciones a medida que la instrucción se complejiza.
Weijnen, FG; Kuks, JBM; van der Glas, HW; Wassenberg, MWM; Bosman, F
Objectives - The aim was to study tongue force in patients with bulbar myasthenia gravis and compare it with that of patients with ocular myasthenia gravis, patients in clinical remission who previously suffered from bulbar myasthenia gravis, and healthy subjects. Material and methods - Tongue force
Vázquez-Roque, F J; Hernández-Oliver, M O; Medrano Plana, Y; Castillo Vitlloch, A; Fuentes Herrera, L; Rivero-Valerón, D
Radical or extended thymectomy is an effective treatment for myasthenia gravis in the adult population. There are few reports to demonstrate the effectiveness of this treatment in patients with juvenile myasthenia gravis. The main objective of this study was to show that extended transsternal thymectomy is a valid option for treating this disease in paediatric patients. Twenty-three patients with juvenile myasthenia gravis underwent this surgical treatment in the period between April 2003 and April 2014; mean age was 12.13 years and the sample was predominantly female. The main indication for surgery, in 22 patients, was the generalised form of the disease (Osserman stage II) together with no response to 6 months of medical treatment. The histological diagnosis was thymic hyperplasia in 22 patients and thymoma in one patient. There were no deaths and no major complications in the postoperative period. After a mean follow-up period of 58.87 months, 22 patients are taking no medication or need less medication to manage myasthenic symptoms. Extended (radical) transsternal thymectomy is a safe and effective surgical treatment for juvenile myasthenia gravis. Copyright © 2015 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.
MARCIO F. DE CARVALHO
Full Text Available Os autores descrevem o caso de uma mulher branca de 24 anos de idade admitida com lupus eritematoso sistêmico (com 4 anos de evolução de doença e início recente de miastenia gravis. São discutidos os principais diagnósticos diferenciais para a fraqueza muscular e a fadiga apresentadas por esta paciente. Uma revisão de literatura abordando a associação de miastenia gravis e lupus eritematoso é feita, com ênfase às características clínicas desses pacientes e ao papel do timoma e timectomia no desenvolvimento de lupus eritematoso em pacientes previamente miastênicos.We report the case of a 24-year-old white woman admitted with a four year diagnosis of systemic lupus erythematosus and the recent onset of myasthenia gravis discussing the main differential diagnosis of weakness and fatigue in this patient. A review of literature approaching the association of myasthenia gravis and systemic lupus erythematosus is also done with emphasis on the clinical characteristics of these patients and the role of thymoma and thymectomy in the development of systemic lupus erythematosus in myasthenic patients.
Dong Hyun Seo
Full Text Available The actual incidence of multiple thymoma is unknown and rarely reported because it remains controversial whether the cases represent a disease of multicentric origin or a disease resulting from intrathymic metastasis. In this case, a patient underwent total thymectomy for multiple thymoma with myasthenia gravis via bilateral video-assisted thoracic surgery. A well-encapsulated multinodular cystic mass, measuring 57 mm×50 mm×22 mm in the right lobe of the thymus, and a well-encapsulated mass, measuring 32 mm×15 mm×14 mm in the left lobe, were found. Both tumors were type B2 thymoma. Few cases of multiple thymoma with myasthenia gravis have ever been reported in the literature. We report a case of synchronous multiple thymoma associated with myasthenia gravis.
Aydin, Yener; Ulas, Ali Bilal; Mutlu, Vahit; Colak, Abdurrahim; Eroglu, Atilla
In recent years, thymectomy has become a widespread procedure in the treatment of myasthenia gravis (MG). Likelihood of remission was highest in preoperative mild disease classification (Osserman classification 1, 2A). In absence of thymoma or hyperplasia, there was no relationship between age and gender in remission with thymectomy. In MG treatment, randomized trials that compare conservative treatment with thymectomy have started, recently. As with non-randomized trials, remission with thymectomy in MG treatment was better than conservative treatment with only medication. There are four major methods for the surgical approach: transcervical, minimally invasive, transsternal, and combined transcervical transsternal thymectomy. Transsternal approach with thymectomy is the accepted standard surgical approach for many years. In recent years, the incidence of thymectomy has been increasing with minimally invasive techniques using thoracoscopic and robotic methods. There are not any randomized, controlled studies which are comparing surgical techniques. However, when comparing non-randomized trials, it is seen that minimally invasive thymectomy approaches give similar results to more aggressive approaches. PMID:28416933
Currier, R.D.; Routh, A.; Hickman, B.T.; Douglas, M.A.
Twenty-eight patients with progressive myasthenia gravis without thymoma received treatment of 3000 rads (30 Gy) to the anterior mediastinum, and a followup was conducted for five to 18 years. Twenty-four patients had generalized myasthenia, and four had ocular myasthenia gravis. Twenty patients with generalized myasthenia survived the several month post-treatment period and improved, but four died during that period. The improvement lasted a median of 1.5 years, and older patients had longer remissions than younger patients. The four patients who had ocular myasthenia did not change after treatment. Mediastinal irradiation produces a temporary remission in generalized myasthenia
Currier, R.D.; Routh, A.; Hickman, B.T.; Douglas, M.A.
Twenty-eight patients with progressive myasthenia gravis without thymoma received treatment of 3000 rads (30 Gy) to the anterior mediastinum, and a followup was conducted for five to 18 years. Twenty-four patients had generalized myasthenia, and four had ocular myasthenia gravis. Twenty patients with generalized myasthenia survived the several month post-treatment period and improved, but four died during that period. The improvement lasted a median of 1.5 years, and older patients had longer remissions than younger patients. The four patients who had ocular myasthenia did not change after treatment. Mediastinal irradiation produces a temporary remission in generalized myasthenia.
Full Text Available Resumen: En este trabajo se presenta una estrategia de control en tiempo discreto para el seguimiento de trayectorias de un robot móvil tipo (2,0 controlado remotamente. La estrategia de control se desarrolló mediante un enfoque de modos deslizantes, considerando el modelo discreto exacto del vehículo en el cual se incluyen los efectos del retardo de transporte causado por la propagación de las señales sobre una red de comunicación. El esquema de control garantiza el seguimiento de trayectorias predeterminadas obteniéndose convergencia asintótica de los errores de seguimiento. La estrategia propuesta es evaluada mediante una serie de resultados por simulación. Palabras clave: Robot móvil, retardos de transporte, control en tiempo discreto, modos deslizantes
Nilson Lopes da Fonseca Junior
Full Text Available OBJETIVO: Demonstrar a sensibilidade e especificidade do teste do gelo no diagnóstico diferencial de ptose palpebral por miastenia gravis. MÉTODOS: Estudo prospectivo tipo ensaio clínico com grupo controle. Foi realizado o teste do gelo em pacientes portadores de ptose palpebral. Os pacientes foram divididos em 2 grupos, sendo o grupo I constituído por pacientes com miastenia gravis e o grupo II (controle formado por pacientes portadores de ptose congênita, miogênica não-miastênica ou aponeurótica. RESULTADOS: Todos os pacientes do grupo I tiveram aumento da fenda palpebral de, no mínimo, 3 mm após a aplicação do gelo. Nenhum paciente do grupo II apresentou incremento da fenda palpebral após o teste. CONCLUSÃO: O teste do gelo mostrou-se específico para detecção de ptose palpebral de causa miastênica.PURPOSE: To demonstrate the sensitivity and the specificity of the ice test in the differential diagnosis of ptosis in myasthenia gravis. METHODS: Prospective trial with a control group. The patients were instructed to hold a frozen ice pack on the closed ptotic eyelid. They were divided into 2 groups, with group I consisting of patients with myasthenia gravis and group II (control consisting of patients with congenital, non-myasthenic myogenic or aponeurotic ptosis. RESULTS: All patients in group I had increased palpebral fissure for at least 3 mm after the application of ice pack. No patient in group II showed increased palpebral fissure after the ice test. CONCLUSION: Ice test proved to be specific for the detection of myasthenic ptosis.
MYASTHENIA GRAVIS MEDICATION INFORMATION CARD Drugs to be Avoided or Used with Caution in MG My Name _______________________________________________ ... the MGFA web site; reference document “Medications and Myasthenia Gravis (A Reference for Health Care Professionals.” www. ...
Cejvanovic, S; Vissing, J
OBJECTIVE: Myasthenia gravis (MG) is characterized by fatigue and fluctuating muscle weakness as a result of impaired neuromuscular transmission (NMT). Although MG is a prototypic fatiguing disorder, little is known about how the condition affects fixed weakness, and if present, whether weakness...
Helgeland, G.; Petzold, A.F.S.; Luckman, S.P.; Gilhus, N.E.; Plant, G.T.; Romi, F.R.
Introduction: Myasthenia gravis (MG) is an autoimmune disease with weakness in striated musculature due to anti-acetylcholine receptor (AChR) antibodies or muscle specific kinase at the neuromuscular junction. A subgroup of patients has periocular symptoms only; ocular MG (OMG). Matrix
Evaluación de un programa de educación de tratamiento intensivo en adolescentes con diabetes tipo 1: Seguimiento por dos años Evaluation of an intensive treatment education program for type 1 diabetes in a group of adolescents. A two year follow up
Andrea G. Krochik
Full Text Available Durante la década pasada, se publicaron numerosos trabajos demostrando que el tratamiento intensivo de la diabetes tipo 1 puede prevenir y retrasar el desarrollo de las complicaciones microvasculares asociadas a la misma. Sin embargo en el grupo de niños y adolescentes se hallaron algunos problemas como el incremento de la frecuencia de los episodios de hipoglucemia y el excesivo aumento de peso. El propósito del presente estudio fue describir los resultados del tratamiento intensificado llevado a cabo durante 2 años en una población de niños y adolescentes argentinos con diabetes tipo 1. Fueron seleccionados 25 pacientes mayores de 10 años de edad con diabetes de más de 1 año de evolución. Todos ellos realizaron un programa de educación durante una semana de internación. El seguimiento posterior fue mensual durante los siguientes dos años. El esquema de tratamiento insulínico consistió en 4 a 5 controles preprandiales diarios, dos dosis de insulina NPH cubriendo requerimientos basales y cuatro dosis de insulina regular preprandial ajustadas de acuerdo al cálculo de hidratos de carbono a ingerir, la actividad física y el valor de glucemia. La media de edad fue de 13.5 años (r: 10-19 años. El tiempo medio transcurrido desde el diagnóstico hasta la intensificación del tratamiento fue de 3.8 años ( r: 1.2 -9 años. La dosis media total de NPH disminuyó significativamente desde 0.9 U/kg en el inicio a 0.8 U/kg al año de seguimiento (p = 0.04, sin diferencias al segundo año de seguimiento (0.85 U/kg. Por el contrario, la dosis de insulina regular administrada en forma preprandial en normoglucemia aumentó de 0 a 0.21 U/kg en el primer año (p 0.0001 y a 0.69 U/kg a los dos años (p NS. La media de HbA1C mostró una significativa reducción desde 10±1.62% a 8.53±1.04% en el primer año (p = 0.03 y 8.72± 0.81% a los dos años. El Z score de IMC aumentó significativamente de 0.7 ± 0.9 a 1.06 ± 1.15 luego de un año (p = 0
Full Text Available Ocular myasthenia gravis is an autoimmune disorder of the neuromuscular junction. Diplopia and ptosis are common symptoms at the onset of ocular myasthenia gravis. It may occur due to the antibodies developed against various drugs. We are reporting a case of ocular myasthenia gravis which was developed in a patient post angiography which may be due to antibody developed against the dye used in angiography.
Myasthenia gravis is a rare long-term neurological condition that is characterised by fluctuating skeletal muscle weakness and fatigue, as well as respiratory difficulties. It is both an acquired autoimmune disease and a chronic neuromuscular disorder. Because of its rarity, myasthenia gravis is relatively unknown and may be unfamiliar to many nurses. While there are various types of myasthenia, this article focuses on myasthenia gravis, exploring its symptoms, diagnosis and treatment, and examining the nurse's role in managing the condition. The symptoms of myasthenic crisis and cholinergic crisis are also explained, and the experience of patients with myasthenia gravis in hospital and community settings is illustrated using case studies.
Tratamento cirúrgico por via anterior na mielopatia cervical espondilótica com seguimento mínimo de dez anos Tratamiento quirúrgico por vía anterior en la mieolopatía cervical espondilótica con seguimiento mínimo de diez años Anterior decompression and fusion for spondilotic cervical mielopathy with a minimal ten-year follow-up
Rui Peixoto Pinto
Full Text Available OBJETIVO: a mielopatia cervical espondilótica (MEC é uma causa frequente de disfunção da medula espinhal na população adulta. O tratamento implica em descompressão cirúrgica precoce. O objetivo foi apresentar um estudo retrospectivo da descompressão anterior e artrodese para MEC com um seguimento mínimo de dez anos. MÉTODOS: pacientes operados entre Janeiro de 1990 e Dezembro de 1994 foram avaliados por sexo, idade, número de níveis operados, avaliação funcional pela escala de Nurick pré-operatória um ano após cirurgia e após a revisão final que ocorreu em 2004, evidência de consolidação e complicações. RESULTADOS: foram avaliados 91 pacientes, 69 do sexo masculino, 22 do sexo feminino, com uma média de idade de 56,6 anos (42-86 e um seguimento médio de 11,9 anos. Ocorreram cinco óbitos: três pacientes no pós-operatório imediato, um no primeiro ano e um durante o restante período. Em média, foram operados 2,7±1,0 níveis por paciente (1-4. O valor médio de Nurick pré-operatório foi de 3,8±0,9. Houve uma melhoria significativa do estado neurológico um ano após a cirurgia (2,2±1,1; pOBJETIVO: la mielopatía cervical espondilótica (MEC es una causa frecuente de disfunción de la médula espinal en la población adulta. El tratamiento implica una descompresión quirúrgica precoz. El objetivo es presentar un estudio retrospectivo de la descompresión anterior y artrodesis para MEC con un seguimiento mínimo de diez años. MÉTODOS: pacientes operados entre Enero de 1990 y Diciembre de 1994 fueron evaluados según el sexo, la edad, el número de niveles operados, la evaluación funcional por la escala de Nurick pre operatoria un año después de la cirugía y después de la revisión final que fue en el 2004, evidencia de consolidación y complicaciones. RESULTADOS: fueron evaluados 91 pacientes, 69 del sexo masculino, 22 del sexo femenino, con un promedio de edades de 56.6 años (42 a 86 y un seguimiento
Suzuki, Masayuki; Takashima, Tsutomu; Itoh, Hiroshi
In this report, preoperative CT diagnosis of the thymus was reevaluated in 15 operated patients with myasthenia gravis. Using GE CT/T 8800 wholebody scanner, pre- and/or post-contrast study was performed from the apex to the diaphragm level. Five cases of thymoma, 5 out of 6 cases of thymic hyperplasia and 3 cases of normal thymus were correctly diagnosed. One hyperplasia was diagnosed as normal and one normal thymus as hyperplasia. At the CT evaluation of the thymus in patients with myasthenia gravis, it is easy to detect thymoma, but it is often difficult to differentiate thymic hyperplasia and normal thymus due to the aging changes in the gland and the presence of localized hyperplasia. (author)
Kuroda, Yoshikazu; Miyamoto, Mariko; Maki, Masako; Yamazaki, Toshiro.
35 cases of myasthenia gravis were studied with RI scintigraphy. 67 Ga-citrate was used in 34 patients and 76 Se-selenomethionine in 5 patients for thymus scintigraphy. RI scintigram was negative in non-tumorous thymus, regardless of the severity of illness and it was positive in seven of nine patients with thymomas. RI accumulation in thymus was found both in benign and malignant thymomas. RI seems to accumulated in lymphocytic and mixed thymus more than epithelial type. (author)
Kuroda, Yoshikazu; Miyamoto, Mariko (Tokyo Metropolitan Fuchu Hospital (Japan)); Maki, Masako; Yamazaki, Toshiro
35 cases of myasthenia gravis were studied with RI scintigraphy. /sup 67/Ga-citrate was used in 34 patients and /sup 76/Se-selenomethionine in 5 patients for thymus scintigraphy. RI scintigram was negative in non-tumorous thymus, regardless of the severity of illness and it was positive in seven of nine patients with thymomas. RI accumulation in thymus was found both in benign and malignant thymomas. RI seems to accumulated in lymphocytic and mixed thymus more than epithelial type.
Almeida Fábio Henrique Souza
Full Text Available A Miastenia Gravis é uma doença auto-imune caracterizada por auto-anticorpos contra receptores nicotínicos de acetilcolina da placa neural. O tratamento clínico básico para as formas generalizadas é feito com drogas anticolenesterásicas, mas em algumas fases podem ser necessários outras modalidades de tratamento, tais como a corticoterapia e a plasmaferese. O tratamento cirúrgico, a timectomia, é uma forma de abordar a causa base da doença e tem resultados bastante encorajadores, visto que é atribuída ao timo a produção dos auto-anticorpos. Apresentamos neste trabalho o quadro clínico, os resultados cirúrgicos e as alteração anátomo-patológicas dos 90 casos estudados.
This is a 10 year retrospective review of patients managed at the Ahmadu Bello Teaching hospital, Zaria, Nigeria for myasthenia gravis. Only 4 patients were identified from the hospitals records. Myasthenia gravis appears uncommon in this environment. Reasons for the apparent rarity of this condition and other ...
Renard, Dimitri; Hedayat, Amir; Gagnard, Corinne
Laryngeal myasthenia gravis is a relatively rare variant of myasthenia gravis. A vast portion of patients with initial laryngeal myasthenia gravis develop involvement of ocular and/or extra-ocular muscles during the years after symptom onset although a minority of laryngeal myasthenia gravis patients continues to have isolated laryngeal muscle involvement for several years. We present a 58-year-old woman with recurrent episodic isolated dysphonia (associated with diffuse bilateral vocal cord paresis on laryngoscopy) since the age of 32. Dysphonia became permanent since 6 months. A diagnosis of laryngeal myasthenia gravis was made based on abnormal single-fiber electromyography and spectacular response to pyridostigmine treatment. Repetitive nerve stimulation was normal and anti-acetylcholine receptor and anti-muscle specific tyrosine kinase antibodies were absent. This case shows that laryngeal myasthenia gravis can be isolated during 26 years of follow-up. We propose that even when myasthenia gravis seems unlikely as underlying mechanism of isolated dysphonia (because of lack of antibodies, normal repetitive nerve stimulation, and absence of extra-laryngeal involvement after years of follow-up), single-fiber electromyography should be performed and myasthenia gravis treatment should be tried. Copyright © 2014 Elsevier B.V. All rights reserved.
Anestesia peridural torácica para cirurgia plástica de mama em paciente portadora de miastenia gravis: relato de caso Anestesia peridural torácica para cirugía plástica de mama en paciente portadora de miastenia gravis: relato de caso Thoracic epidural anesthesia for mammaplasty in myasthenia gravis patient: case report
Fabiano Timbó Barbosa
Full Text Available JUSTIFICATIVA E OBJETIVOS: A miastenia gravis é uma doença crônica, auto-imune, caracterizada pela fraqueza da musculatura esquelética em decorrência da diminuição dos receptores de acetilcolina na junção neuromuscular. O objetivo deste relato é mostrar um caso de paciente com miastenia gravis submetida a anestesia peridural torácica para cirurgia plástica de mama. RELATO DO CASO: Paciente com 51 anos, portadora de miastenia gravis foi submetida a anestesia peridural torácica com bupivacaína e fentanil. Não houve sinais de depressão respiratória. A paciente recebeu alta hospitalar após 36 horas. CONCLUSÕES: O presente caso sugere como conduta anestésica para o paciente portador de miastenia gravis a anestesia peridural como técnica única, sem a obrigatoriedade de intubação orotraqueal.JUSTIFICATIVA Y OBJETIVOS: La miastenia gravis es una enfermedad crónica, auto-inmune, caracterizada por la debilidad de la musculatura esquelética resultante de la disminución de los receptores de acetilcolina en la unión neuromuscular. El objetivo de este relato es mostrar el caso de una paciente con miastenia gravis sometida a anestesia peridural torácica para una cirugía plástica de mama. RELATO DEL CASO: Paciente del sexo femenino, 51 años, portadora de miastenia gravis fue sometida a anestesia peridural torácica con bupivacaína y fentanil. No hubo señales de depresión respiratoria. La paciente recibió alta hospitalaria después de 36 horas. CONCLUSIONES: Este actual caso sugiere como conducta anestésica para el paciente portador de miastenia gravis la anestesia peridural como única técnica, sin la obligatoriedad de intubación orotraqueal.BACKGROUND AND OBJECTIVES: Myasthenia gravis is a chronic autoimmune disease characterized by skeletal muscles weakness promoted by decreased acetylcholine receptors in the neuromuscular junction. This report aimed at describing a case of myasthenia gravis patient submitted to thoracic
Full Text Available 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, piorando com o esforço físico e melhorando 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 um parto cesário 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. Keywords: Myasthenia gravis, Sugammadex, Pregnancy, Palavras-chave: Miastenia grave, Sugammadex, Gravidez
Mariana Inés Ybarra
Full Text Available OBJECTIVE: To investigate the prevalence of psychiatric disorders in patients with myasthenia gravis (MG. METHOD: Forty-one patients with MG answered to a structured psychiatric interview (MINI-Plus. RESULTS: Eleven (26.1% patients were diagnosed with a depressive disorder and 19 (46.3% were diagnosed with an anxiety disorder. Patients with dysthymia were older (p=0.029 and had longer disease duration (p=0.006. Patients with social phobia also had longer disease duration (p=0.039. CONCLUSION: Psychiatric disorders in MG are common, especially depressive and anxiety disorders.
Nair, Akshay Gopinathan; Patil-Chhablani, Preeti; Venkatramani, Devendra V; Gandhi, Rashmin Anilkumar
Myasthenia gravis (MG) is a disease that affects the neuro-muscular junction resulting in classical symptoms of variable muscle weakness and fatigability. It is called the great masquerader owing to its varied clinical presentations. Very often, a patient of MG may present to the ophthalmologist given that a large proportion of patients with systemic myasthenia have ocular involvement either at presentation or during the later course of the disease. The treatment of ocular MG involves both the neurologist and ophthalmologist. Thus, the aim of this review was to highlight the current diagnosis, investigations, and treatment of ocular MG. PMID:25449931
Barnett, Carolina; Merkies, Ingemar S J; Katzberg, Hans; Bril, Vera
The Quantitative Myasthenia Gravis Score and the Myasthenia Gravis Composite are two commonly used outcome measures in Myasthenia Gravis. So far, their measurement properties have not been compared, so we aimed to study their psychometric properties using the Rasch model. 251 patients with stable myasthenia gravis were assessed with both scales, and 211 patients returned for a second assessment. We studied fit to the Rasch model at the first visit, and compared item fit, thresholds, differential item functioning, local dependence, person separation index, and tests for unidimensionality. We also assessed test-retest reliability and estimated the Minimal Detectable Change. Neither scale fit the Rasch model (X2p Myasthenia Gravis Composite had lower discrimination properties than the Quantitative Myasthenia Gravis Scale (Person Separation Index: 0.14 and 0.7). There was local dependence in both scales, as well as differential item functioning for ocular and generalized disease. Disordered thresholds were found in 6(60%) items of the Myasthenia Gravis Composite and in 4(31%) of the Quantitative Myasthenia Gravis Score. Both tools had adequate test-retest reliability (ICCs >0.8). The minimally detectable change was 4.9 points for the Myasthenia Gravis Composite and 4.3 points for the Quantitative Myasthenia Gravis Score. Neither scale fulfilled Rasch model expectations. The Quantitative Myasthenia Gravis Score has higher discrimination than the Myasthenia Gravis Composite. Both tools have items with disordered thresholds, differential item functioning and local dependency. There was evidence of multidimensionality in the QMGS. The minimal detectable change values are higher than previous studies on the minimal significant change. These findings might inform future modifications of these tools.
María Bárbara Eizaguirre; Florencia Aguirre; Cecilia Yastremiz; Sandra Vanotti; Andrés Villa
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...
JORGE S. REIS FILHO
Full Text Available Thymomas are tumors characterized by a remarkable morphological heterogeneity and variable clinical behavior. This tumor has unique clinical associations, most notably with hematological abnormalities and myasthenia gravis. According with the Müller-Hermelink criteria, there are significant differences between the histological types of thymomas and the association with myasthenia gravis. Among the different histological types, medullary thymoma is the least frequent variant associated with this autoimmune disease. In this report we describe a case of medullary thymoma presenting in a 71-year- old woman with a myasthenic syndrome.Os timomas são tumores caracterizados por grande heterogeneidade morfológica e comportamento clínico variável. Este tumor apresenta associações clínicas singulares, principalmente com doenças hematológicas e com a miastenia gravis. De acordo com a classificação de Müller-Hermelink, existem diferenças significativas entre as variedades histológicas dos timomas e sua associação com a miastenia gravis. Entre os diferentes tipos histológicos, o timoma medular é a variante menos frequentemente associada com esta doença autoimune. Neste relato, nós descrevemos caso de timoma medular em uma paciente de 71 anos de idade com síndrome miastênica.
Miarzynska, M.; Szlezak, L.; Fibak, J.; Wolski, M.; Lis-Podrzycka, E.; Miarzynski, K.
The rare case of malignant tumor of thymus - Thymoma malignum was described. The initial diagnosis was difficult, because of the irregular symptoms of myasthenia gravis. The diagnostic difficulties, treatment and clinical features of this neoplasm were also discussed. (author)
... Many different drugs have been associated with worsening myasthenia gravis (MG). However, these drug associations do not ... review article for health professionals, found at the myasthenia.org website. For this app, we are only ...
BURGOS ROMERO, LUIS
Convenios con empresa. La obra prevé la construcción de un edificio de 41 viviendas VP + 2 viviendas libres, constando de 2 plantas sótano para uso de aparcamiento y trasteros, planta baja con local comercial, 8 plantas con 5 viviendas por planta y una planta ático con 3 viviendas. La obra está en su fase de estructura, habiéndose realizado las plantas de sótano. Burgos Romero, L. (2011). Seguimiento de la construcción de edificio 43 viviendas Av/ Juan XXIII Valencia. http://hdl.handle.net...
Velásquez-Jones, Luis; Medeiros, Mara; Valverde-Rosas, Saúl; Jiménez-Triana, Clímaco; del Moral-Espinosa, Irma; Romo-Vázquez, José Carlos; Franco-Alvarez, Isidro
Introducción: El raquitismo dependiente de vitamina D tipo I es una enfermedad hereditaria rara debida a una mutación en el gen CYP27B1 que codifica la enzima 1α-hidroxilasa. Se caracteriza por la presentación de raquitismo hipocalcémico grave desde la edad de la lactancia debido al déficit de producción del metabolito activo de la vitamina D, la 1α,25-dihidroxivitamina D3. Caso clínico: Presentamos el caso de un paciente con raquitismo diagnosticado a los 11 meses de edad y el seguimiento...
Denis Bernardi Bichuetti
Full Text Available Myasthenia gravis (MG is an autoimmune disease characterized by fluctuating muscle weakness, caused by impaired neuromuscular transmission. Patients with MG can present other autoimmune diseases in association, commonly hypo or hyperthyroidism. The association of MG to demyelinating disease is rare and has been described before. We report on three Brazilian patients with MG that presented distinct demyelinating diseases, two monophasic and one recurrent neuromyelitis optica, several years after the diagnosis of MG, and discuss their clinical courses.Miastenia gravis (MG é doença autoimune caracterizada por episódios de fraqueza muscular alternados com melhora, causada por bloqueio da junção neuromuscular. Pacientes com MG podem apresentar outras doenças autoimunes, comumente hipo ou hipertiroidismo, e a associação de MG com doenças desmielinizantes é raramente descrita. Relatamos três pacientes brasileiros com MG que desenvolveram doenças desmielinizantes, dois monofásicos e um neuromielite óptica recorrente, vários anos após o diagnóstico de MG e discutimos seus cursos clínicos.
Jagathesan, Tania; O'Brien, Michael D
Myasthenia gravis is an autoimmune condition where antibodies form against the acetylcholine receptors at the neuromuscular junction, eventually causing damage to the motor end plate. The clinical features include muscle fatigability as well as ocular, bulbar, and limb weakness, which can have implications on the role of a pilot or air traffic controller. This retrospective study reviewed the United Kingdom Civil Aviation Authority (UK CAA) experience of myasthenia gravis. A search of the United Kingdom Civil Aviation Authority medical records database from 1990 to 2016 identified 11 individuals with a diagnosis of myasthenia gravis. Data were extracted for the class of medical certificate, age at diagnosis, symptoms, acetylcholine receptor antibody status, treatment, the time from diagnosis to loss of medical certification, and the reasons for loss of certification. There were two Class 1 certificate holders (for professional flying) and six Class 2 certificate holders (for private pilot flying) and three air traffic controllers. The mean and median ages at diagnosis were 53 and 57 yr, respectively, with a range of 28-67 yr. The mean and median intervals from diagnosis to loss of certification were 22 and 11 mo, respectively, with a range of 0 to 108 mo. The aeromedical implications of myasthenia gravis, including complications, types of treatment, and functional impact, are considered. A policy for medical certification following a diagnosis of myasthenia gravis is proposed.Jagathesan T, O'Brien MD. Myasthenia gravis and its aeromedical implications. Aerosp Med Hum Perform. 2017; 88(1):30-33.
Wolfe, Gil I; Kaminski, Henry J; Aban, Inmaculada B; Minisman, Greg; Kuo, Hui-Chien; Marx, Alexander; Ströbel, Philipp; Mazia, Claudio; Oger, Joel; Cea, J Gabriel; Heckmann, Jeannine M; Evoli, Amelia; Nix, Wilfred; Ciafaloni, Emma; Antonini, Giovanni; Witoonpanich, Rawiphan; King, John O; Beydoun, Said R; Chalk, Colin H; Barboi, Alexandru C; Amato, Anthony A; Shaibani, Aziz I; Katirji, Bashar; Lecky, Bryan R F; Buckley, Camilla; Vincent, Angela; Dias-Tosta, Elza; Yoshikawa, Hiroaki; Waddington-Cruz, Márcia; Pulley, Michael T; Rivner, Michael H; Kostera-Pruszczyk, Anna; Pascuzzi, Robert M; Jackson, Carlayne E; Garcia Ramos, Guillermo S; Verschuuren, Jan J G M; Massey, Janice M; Kissel, John T; Werneck, Lineu C; Benatar, Michael; Barohn, Richard J; Tandan, Rup; Mozaffar, Tahseen; Conwit, Robin; Odenkirchen, Joanne; Sonett, Joshua R; Jaretzki, Alfred; Newsom-Davis, John; Cutter, Gary R
Thymectomy has been a mainstay in the treatment of myasthenia gravis, but there is no conclusive evidence of its benefit. We conducted a multicenter, randomized trial comparing thymectomy plus prednisone with prednisone alone. We compared extended transsternal thymectomy plus alternate-day prednisone with alternate-day prednisone alone. Patients 18 to 65 years of age who had generalized nonthymomatous myasthenia gravis with a disease duration of less than 5 years were included if they had Myasthenia Gravis Foundation of America clinical class II to IV disease (on a scale from I to V, with higher classes indicating more severe disease) and elevated circulating concentrations of acetylcholine-receptor antibody. The primary outcomes were the time-weighted average Quantitative Myasthenia Gravis score (on a scale from 0 to 39, with higher scores indicating more severe disease) over a 3-year period, as assessed by means of blinded rating, and the time-weighted average required dose of prednisone over a 3-year period. A total of 126 patients underwent randomization between 2006 and 2012 at 36 sites. Patients who underwent thymectomy had a lower time-weighted average Quantitative Myasthenia Gravis score over a 3-year period than those who received prednisone alone (6.15 vs. 8.99, Pmyasthenia gravis. (Funded by the National Institute of Neurological Disorders and Stroke and others; MGTX ClinicalTrials.gov number, NCT00294658.).
Ko, Eun Sook; Jeon, Kyung Nyeo; Bae, Kyung Soo; Yoo, Jin Jong; Kang, Duk Sik
A thymoma often occurs in patients with myasthenia gravis, but the development of multiple thymoma is very rare. The authors report the radiologic and pathologic findings of multiple invasive thymoma in a 59-year-old male with myasthenia gravis
Ko, Eun Sook; Jeon, Kyung Nyeo; Bae, Kyung Soo; Yoo, Jin Jong [College of Medicine, Gyeongsang National Univ., Jinju (Korea, Republic of); Kang, Duk Sik [College of Medicine, Kyungpook National Univ., Daegu (Korea, Republic of)
A thymoma often occurs in patients with myasthenia gravis, but the development of multiple thymoma is very rare. The authors report the radiologic and pathologic findings of multiple invasive thymoma in a 59-year-old male with myasthenia gravis.
Utsugisawa, Kimiaki; Nagane, Yuriko
Many patients with myasthenia gravis (MG) still find it difficult to maintain daily activities due to chronic residual fatigability and long-term side effects of oral corticosteroids, since full remission is not common. Our analysis demonstrated that disease severity, oral corticosteroids, and depressive state are the major factors negatively associated with QOL, and that QOL of MM status patients taking CSR and is a target of treatment. In order to achieve early MM or better status with prednisolne strategy that can achieve early improvement by performing an aggressive therapy using combined treatment with plasmapheresis and high-dose intravenous methylprednisolone and then maintain an improved status using low-dose oral corticosteroids and calcineurin inhibitors.
Barragan, J. N.
Full Text Available Durante el proceso de vinculación de trabajos de investigación en centros educativos en las áreas de estudio de contaduría y administración, ha sido posible el llegar a constatar que en varios de estos centros educativos, no se da un seguimiento formal y profesional a egresados de las diferentes carreras de contaduría y administración, y las pocas universidades que tiene implementado un sistema de seguimiento a egresados, no le otorgan crédito, ni mucho menos importancia como instrumento de planeación estratégica. Se limitan simplemente a la creación de bancos de datos, comúnmente llamados de “ex alumnos”, cuyo principal objetivo es servir de directorio actualizado para envío de invitaciones a eventos de capacitación y fiestas y celebraciones institucionales. Como se menciona anteriormente, no se ha sabido apreciar por parte de las escuelas de contaduría y administración, la importancia que tiene un programa de seguimiento de egresados, para el desarrollo y soporte de la planeación estratégica de estos centros educativos, y sobre todo me llama la atención, que no se ha sido posible apreciar, la real falta de orientación, o por que no llamarle “tutoría”, que leude requerir un egresado al enfrentarse al campo profesional, además de constituir una invaluable fuente de retroalimentación y medida de evaluación para la institución, sobre el nivel académico de sus egresados, y en consecuencia, monitor del progreso obtenido en su práctica profesional.
José Roberto Támara-Ramírez
Conclusiones. Es necesario que los programas de HIV/sida establezcan sistemas de seguimiento y rastreo, con el fin de establecer estrategias para mejorar la retención de los pacientes y, por ende, su calidad de vida a largo plazo.
Moreira Ana Tereza Ramos
Full Text Available A miastenia gravis é uma doença crônica, caracterizada por fatigabilidade anormal de músculos estriados, podendo acometer grupos musculares isolados ou tornar-se generalizada. Os autores descrevem um caso de miastenia gravis congênita generalizada e oftalmoplegia parcial em um paciente de 10 anos de idade, portador de sintomas sistêmicos motores e de ausência na adução, abdução e elevação em ambos os olhos e com ptose palpebral bilateral, sendo reduzida à função de infraversão. O paciente foi diagnosticado aos dois anos e seis meses, sendo iniciado tratamento com piridostigmina em doses subterapêuticas, sem alteração importante no quadro clínico. Aos quatro anos de idade, procurou esta instituição, sendo ajustada à dose da medicação, seguindo-se melhora significativa dos sintomas motores sistêmicos, melhora parcial da ptose palpebral e sem alteração na oftalmoplegia externa.
Gilhus, Nils Erik; Verschuuren, Jan J
Myasthenia gravis is an autoimmune disease that is characterised by muscle weakness and fatigue, is B-cell mediated, and is associated with antibodies directed against the acetylcholine receptor, muscle-specific kinase (MUSK), lipoprotein-related protein 4 (LRP4), or agrin in the postsynaptic membrane at the neuromuscular junction. Patients with myasthenia gravis should be classified into subgroups to help with therapeutic decisions and prognosis. Subgroups based on serum antibodies and clinical features include early-onset, late-onset, thymoma, MUSK, LRP4, antibody-negative, and ocular forms of myasthenia gravis. Agrin-associated myasthenia gravis might emerge as a new entity. The prognosis is good with optimum symptomatic, immunosuppressive, and supportive treatment. Pyridostigmine is the preferred symptomatic treatment, and for patients who do not adequately respond to symptomatic therapy, corticosteroids, azathioprine, and thymectomy are first-line immunosuppressive treatments. Additional immunomodulatory drugs are emerging, but therapeutic decisions are hampered by the scarcity of controlled studies. Long-term drug treatment is essential for most patients and must be tailored to the particular form of myasthenia gravis. Copyright © 2015 Elsevier Ltd. All rights reserved.
Ramirez Benitez, Juan Rafael; Ramos Berumen, Carlos; Beltran Adan, Jose; Lagunas Mendoza, Javier [Instituto de Investigaciones Electricas, Cuernavaca, Morelos (Mexico)]. E-mail: email@example.com; firstname.lastname@example.org; email@example.com
The Instituto de Investigaciones Electricas (IIE) has been interested in the Parabolic Dish technology for electricity generation in Mexico, then through its Non Conventional Energy Department has dedicated special tasks concerning to the knowledge and development of such technology. The structural component, reflective surface support and the tracking system which allows concentrate the solar energy into the receiver have been designed and manufactured. For the mechanical device and control of the solar tracking have been projected a set with servomotors, an electronic control as well as an interface for the equipment configuration and follow-up. In order for getting the following of the apparent movement of the sun, information on the sun paths through the year was analyzed and in consequence elevation and azimuth angles were determined. Using that approaches, for fixing the sun position sensors are not used and then only the control algorithm and the electronic device developed at the IIE were implemented. In this paper the sun tracking system and the electronic control device are presented. [Spanish] La tecnologia de plato parabolico para la generacion de electricidad en Mexico, ha sido de gran interes para el Instituto de Investigaciones Electricas, por lo que a traves de la Gerencia de Energias No Convencionales se ha avocado a la tarea de conocer y desarrollar esta tecnologia. Se ha disenado y fabricado la parte estructural, la base de la superficie reflejante y el sistema de seguimiento que permite concentrar la energia solar en el receptor. Para el mecanismo y control del seguidor solar se ha propuesto un mecanismo que utiliza servomotores, un control electronico asi como una interfaz para configurar el equipo y monitorear sus variables. Para lograr el seguimiento del movimiento aparente del sol se analizo la informacion en literatura de las trayectorias del sol a lo largo del ano, logrando asi determinar los angulos de altitud y azimut para acotar el
Meriggioli, Matthew N; Sanders, Donald B
Myasthenia gravis is an autoimmune disorder of the neuromuscular junction. A number of molecules, including ion channels and other proteins at the neuromuscular junction, may be targeted by autoantibodies leading to abnormal neuromuscular transmission. In approximately 85% of patients, autoantibodies, directed against the postsynaptic nicotinic acetylcholine receptor can be detected in the serum and confirm the diagnosis, but in general, do not precisely predict the degree of weakness or response to therapy. Antibodies to the muscle-specific tyrosine kinase are detected in approximately 50% of generalized myasthenia gravis patients who are seronegative for anti-acetylcholine receptor antibodies, and levels of anti-muscle-specific tyrosine kinase antibodies do appear to correlate with disease severity and treatment response. Antibodies to other muscle antigens may be found in the subsets of myasthenia gravis patients, potentially providing clinically useful diagnostic information, but their utility as relevant biomarkers (measures of disease state or response to treatment) is currently unclear. PMID:22882218
Richards, Jason; Howard, James F
Myasthenia gravis (MG) is generally caused by antibodies directed against the neuromuscular junction, including antibodies against the postsynaptic nicotinic acetylcholine receptor (AChR). Pathologic abnormalities of the thymus gland, including thymoma, are associated with MG. We report a 56-year-old woman who presented with double vision. Single fiber EMG confirmed myasthenia gravis. AChR, striational muscle and MuSK antibodies were absent in the serum. Chest CT demonstrated a malignant thymoma. We report the first case of seronegative myasthenia gravis associated with malignant thymoma. The case challenges the conventional wisdom that all patients with thymoma associated MG test positive for antibodies against AChR. Copyright © 2017 Elsevier B.V. All rights reserved.
Full Text Available El seguimiento de los titulados, y más concretamente el índice de ocupación de los mismos, se contempla como un indicador de la calidad docente de las titulaciones impartidas por los centros de educación superior. De lo que se deduce que cuanto mayor sea ese índice de ocupación mayor será la calidad de la enseñanza. Sin embargo, esa relación no es directa, sino que está mediatizada por muchas otras variables. El grado de inserción laboral de los titulados depende más de la estructura del mercado de trabajo que de la calidad de la enseñanza recibida.
Full Text Available Autoimmune diseases (AIDs are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as “self” are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto’s thyroiditis and Graves disease. They are frequently associated with other organ or non-organ specific AIDs, such as myasthenia gravis (MG. In fact, ATD seems to be the most associated pathology to MG. The etiology of both diseases is multifactorial and it is due to genetic and environmental factors, and each of them has specific characteristics. The two pathologies show many commonalities, such as the organ-specificity with a clear pathogenic effect of antibodies, the pathological mechanisms, such as deregulation of the immune system and the implication of the genetic predisposition. They also show some differences, such as the mode of action of the antibodies and therapies. In this review that focuses on ATD and MG, the common features and the differences between the two diseases are discussed.
Lopomo, Angela; Berrih-Aknin, Sonia
Autoimmune diseases (AIDs) are the result of specific immune responses directed against structures of the self. In normal conditions, the molecules recognized as “self” are tolerated by immune system, but when the self-tolerance is lost, the immune system could react against molecules from the body, causing the loss of self-tolerance, and subsequently the onset of AID that differs for organ target and etiology. Autoimmune thyroid disease (ATD) is caused by the development of autoimmunity against thyroid antigens and comprises Hashimoto’s thyroiditis and Graves disease. They are frequently associated with other organ or non-organ specific AIDs, such as myasthenia gravis (MG). In fact, ATD seems to be the most associated pathology to MG. The etiology of both diseases is multifactorial and it is due to genetic and environmental factors, and each of them has specific characteristics. The two pathologies show many commonalities, such as the organ-specificity with a clear pathogenic effect of antibodies, the pathological mechanisms, such as deregulation of the immune system and the implication of the genetic predisposition. They also show some differences, such as the mode of action of the antibodies and therapies. In this review that focuses on ATD and MG, the common features and the differences between the two diseases are discussed. PMID:28751878
Full Text Available Objetivo: Seguimiento neurofisiológico en 10 pacientes con injertos de nervios periféricos. Lugar: Unidad de Neurofisiología Clínica del Hospital Nacional Guillermo Almenara Irigoyen, EsSalud. Material y Métodos: Se estudió 10 pacientes de 6 a 39 años, con lesiones de nervios periféricos en miembros superiores, en quienes se realizó injertos. Se hizo control neurofisiológico periódico con electromiografía, velocidad de conducción nerviosa motora y sensitiva. Resultados: Se encontró una excelente evolución de los 10 casos, demostrada por el seguimiento electrofisiológico, en algunos de ellos con recuperación precoz de la conducción nerviosa motora e incluso sensitiva, como en los casos de lesión cubital (casos 4, 7 y 8 y cubital y mediano (caso 10. Esta recuperación tuvo un correlato clínico, con cambios neurovegetativos, motores y sensitivos. Conclusiones: La electrofisiología juega un papel importante en el seguimiento postoperatorio de las lesiones de los nervios periféricos en las que se realiza injertos. El tiempo de recuperación neurofisiológica y clínica de un injerto nervioso es muy variable, entre 1 y 2 años o más. Sorprende que la fisiología clínica no siempre guarde una relación proporcional con la electrofisiología, pues en algunos casos la recuperación clínica es aceptable, mientras que en otros es desproporcionada a la gran lentitud de la velocidad de conducción nerviosa.
Clark, T. E.; Liu Boyang; Love, S. T.; Xiong, C.; Veldhuis, T. ter
Higgs boson decays in flexible brane world models with stable, massive gravi-vectors are considered. Such vectors couple bilinearly to the standard model fields through either the standard model energy-momentum tensor, the weak hypercharge field strength, or the Higgs scalar. The role of the coupling involving the extrinsic curvature is highlighted. It is found that within the presently allowed parameter space, the decay rate of the Higgs into two gravi-vectors (which would appear as an invisible Higgs decay) can be comparable to the rate for any of the standard model decay modes.
José Lamartine de Assis
Full Text Available Two pairs of siblings with myasthenia gravis, belonging to two different families, are reported. This is the only record of familial myasthenia during the past twenty years, in a total of 145 patients seen at the Neurological Clinic of the São Paulo Medical School. In spite of the fact that myasthenia gravis does not show hereditary characteristics, the peculiar features of the four cases justify the present report. The two pairs of siblings were born from non myasthenic nor consanguineous parents. The disease started at birth showing bilateral partial eyelid ptosis in all patients. The course of the illness has been favorable. There was no thymoma.
Invernaderos: Seguimientos de Temperatura y Oxígeno Entre diciembre del 2001 y mayo del 2002 se realizaron dos series de experimentos para evaluar el efecto de las temperaturas altas en la supervivencia y el crecimiento del camarón utilizando invernaderos.
Weijnen, FG; Wokke, JHJ; Kuks, JBM; van der Glas, HW; Bosman, F
Masticatory muscle strength was quantified in patients with bulbar myasthenia gravis and compared with that of patients with ocular myasthenia gravis, patients in clinical remission (whether or not pharmacological) who previously suffered from bulbar myasthenia gravis, and healthy subjects. Maximal
Wang, Lili; Zhang, Yun; He, Maolin
Clinical predictors for myasthenia gravis relapse and ocular myasthenia gravis secondary generalization during the first two years after disease onset remain incompletely identified. This study attempts to investigate the clinical predictors for the prognosis of Myasthenia Gravis. Eighty three patients with myasthenia gravis were concluded in this study. Baseline characteristics were analyzed as predictors. Relapse of myasthenia gravis developed in 26 patients (34%). Generalization developed in 34 ocular myasthenia gravis patients (85%). Other autoimmune diseases were observed more commonly in relapsed myasthenia gravis (P = 0.012). Second generalization group contained more late onset patients (P = 0.021). Ocular myasthenia gravis patients with thymus hyperplasia progressed more rapidly than those with other thymus pathology (P = 0.027). Single onset symptom of ocular myasthenia gravis such as ptosis or diplopia predicted early progression than concurrence of ptosis and diplopia (P = 0.027). Treatment effect including glucocorticoid, pyridostigmine, thymectomy, IVIG, immunosuppressive drugs did not show significant difference between the relapsed and non-relapsed groups. The treatment outcome also showed no difference between the single OMG and second generalized groups. Occurrence of associated autoimmune disease can serve as a potential predictor for myasthenia gravis relapse. Either ptosis or diplopia, as well as thymic hyperplasia can predict generalization in the first six months.
According to a generalization of the gravi-magnetic hypothesis (GMH) any neutral mass moving in a curvilinear path with respect to an inertial frame creates a magnetic field, dependent on the curvature radius of the path. A simple astrophysical consequence of the generalized GMH is suggested considering the special cases of binary pulsars and binary neutron stars
Marine Corps Inst., Washington, DC.
Developed as part of the Marine Corps Institute (MCI) correspondence training program, this course on vegetables, soups, sauces, gravies, and beverages is designed to increase Marine Corps cooks' effectiveness as food handlers, using the proper techniques in the preparation of these items. Introductory materials include specific information for…
Kuks, JBM; Skallebaek, D
Plasmapheresis plays an important role in the acute management of patients with severe myasthenia gravis. Although plasmapheresis is now in use for more than 20 years, some controversies remain about the indication and the place in the therapy. It is generally found that the effect starts one week
Kim, Guk Hee [Insung Hospital, Chuncheon (Korea, Republic of); Kang, Eun Young; Lee, Nam Joon; Suh, Won Hyuck [Korea University College of Medicine, Seoul (Korea, Republic of)
The relationship between myasthenia gravis and the thymus was well establish and myasthenia gravis occurs in the presence of thymic hyperplasia or thymoma or occasionally in histologically normal thymus. Since not every patients with myasthenia gravis is a candidate for thymectomy, unless a thymoma is present, the differentiation of normal and hyperplastic thymus from thymoma becomes important. Authors reviewed retrospectively clinical records and chest CT scans of 18 patients with myasthenia gravis who underwent thymectomy during recent 5 years, to evaluate the role of CT scan. The results were as follows. 1 Of total 18 cases, 5 cases had normal thymus, 6 cases had thymic hyperplasia, 4 cases had benign thymoma and 3 cases had malignant thymoma. 2. Of 5 cases of normal thymus, no false positive cases were noted in CT scan. 3. Of 6 cases of thymic hyperplasia, CT findings were normal except 1 cases of thickened left thymic lobe. 4. Of 7 cases of thymoma, no false negative cases were noted in CT scan. 5. CT findings of benign thymoma were round or oval shaped, discrete, slightly enhancing soft tissue mass in anterior mediastinum. 6. CT findings of malignant thymoma were lobulated contoured, infiltrative, soft tissue mass lesion in anterior mediastinum with calcifications, pleural tumor implants, and SVC compression. CT yielded valuable information on differential diagnosis of thymoma, thymic hyperplasia and normal thymus. Also CT was a highly sensitive method in the detection of thymoma and determining the extent and invasiveness.
Kim, Guk Hee; Kang, Eun Young; Lee, Nam Joon; Suh, Won Hyuck
The relationship between myasthenia gravis and the thymus was well establish and myasthenia gravis occurs in the presence of thymic hyperplasia or thymoma or occasionally in histologically normal thymus. Since not every patients with myasthenia gravis is a candidate for thymectomy, unless a thymoma is present, the differentiation of normal and hyperplastic thymus from thymoma becomes important. Authors reviewed retrospectively clinical records and chest CT scans of 18 patients with myasthenia gravis who underwent thymectomy during recent 5 years, to evaluate the role of CT scan. The results were as follows. 1 Of total 18 cases, 5 cases had normal thymus, 6 cases had thymic hyperplasia, 4 cases had benign thymoma and 3 cases had malignant thymoma. 2. Of 5 cases of normal thymus, no false positive cases were noted in CT scan. 3. Of 6 cases of thymic hyperplasia, CT findings were normal except 1 cases of thickened left thymic lobe. 4. Of 7 cases of thymoma, no false negative cases were noted in CT scan. 5. CT findings of benign thymoma were round or oval shaped, discrete, slightly enhancing soft tissue mass in anterior mediastinum. 6. CT findings of malignant thymoma were lobulated contoured, infiltrative, soft tissue mass lesion in anterior mediastinum with calcifications, pleural tumor implants, and SVC compression. CT yielded valuable information on differential diagnosis of thymoma, thymic hyperplasia and normal thymus. Also CT was a highly sensitive method in the detection of thymoma and determining the extent and invasiveness
Full Text Available Ocular myasthenia gravis in conjunction with thyroid disorders, although rare, has been reported in the past. However, the similarity in the presentation of both the entities and the tendency of myasthenia gravis to get overlooked easily, even by experienced clinicians, necessitates a thorough knowledge, a strong consideration, and a vigilant approach, to aid in its diagnosis. We discuss a case of a female in a thyrotoxic state, with symptoms of ocular myasthenia gravis, and a brief overview of this entity.
Poornima A Shah
Full Text Available Man-in-the-barrel syndrome (MBS is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG at rest and brief duration (3-6 ms bi/triphasic motor unit potentials (MUPs on submaximal effort apart from a decremental response on repetitive nerve stimulation (RNS at 2 Hz. While electrophysiology is an important tool in the diagnosis of myasthenia gravis, pathological EMG patterns do not exclude the diagnosis of myasthenia gravis.
Shah, Poornima A; Wadia, Pettarusp Murzban
Man-in-the-barrel syndrome (MBS) is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG) at rest and brief duration (3-6 ms) bi/triphasic motor unit potentials (MUPs) on submaximal effort apart from a decremental response on repetitive nerve stimulation (RNS) at 2 Hz. While electrophysiology is an important tool in the diagnosis of myasthenia gravis, pathological EMG patterns do not exclude the diagnosis of myasthenia gravis. PMID:27011638
Goienetxea Soto E
Full Text Available El Seguimiento Farmacoterapéutico (SFT es un servicio profesional que aborda de manera global los problemas de salud y los medicamentos que utiliza el paciente, centrándose en la valoración de la necesidad, efectividad y seguridad de la farmacoterapia. Es, por tanto, el farmacéutico el profesional idóneo para llevar a cabo esta actividad, ya que es el experto en el medicamento y tiene el conocimiento, habilidades y actitudes necesarias para abordar todos los campos de actuación. Así queda ampliamente recogido en las competencias profesionales del título de Grado en Farmacia y respaldado por la normativa sanitaria vigente, así como por diversos organismos oficiales nacionales e internacionales, que recomiendan su implantación en farmacia comunitaria. La implementación de programas sanitarios específicos impulsados por las administraciones públicas, dirigidos a la implantación del servicio de SFT en farmacia comunitaria, supondría una mejora del uso de los medicamentos y la salud de los pacientes, así como un impulso al desarrollo profesional del farmacéutico comunitario y su integración en el Sistema Nacional de Salud (SNS.
Spasojevic, Ivana; Hajdukovic, Danica; Komarcevic, Milena; Petrovic, Stanislava; Jovanovic, Jelena; Ciric, Aleksandra
Myasthenia gravis is an autoimmune disease caused by antibodies leading to the destruction of nicotinic acetylcholine receptors on the neuromuscular junction. It is characterized by muscle weakness that gets aggravated with physical activity and improves at rest. Myasthenia Gravis Foundation of America made the clinical classification of Myasthenia gravis which is still in use today. "Tensilon test" is still the gold standard for the diagnosis of Myasthenia gravis. In addition to this test repeated muscular stimulation can be used as well as the analysis of specific autoantibodies. Treatment of Myasthenia Gravis. In conservative treatment of Mysthenia gravis anticholinesterases, immunosuppressants and plasmapheresis can be used. If conservative treatment does not lead to the desired remission, surgical treatment is indicated. The most accepted indication for thymectomy is the presence of thymoma with generalized form of Myasthenia gravis in adults. How to Distinguish Myasthenic From Cholinergic Crisis.'The following is important to make a difference between these two crises: knowledge of the events that preceded the crisis, the size of pupils as well as the presence of muscarinic signs and tensilon test. Specific Features of Anesthesia in Patients with Myasthienia Gravis. Mechanism of the disease development is the reason'for the increased sensitivity or resistance of these patients to certain types of drugs used in anesthesia. Protocol of Perioperative Anesthesia in Patients with Myasthenia Gravis. Based on 35 years of experience in the surgical treatment of patients with Myasthenia gravis anesthesiologists at the Department of Thoracic Surgery, Institute for Pulmonary Diseases of Vojvodina. made the protocol of anesthesia and perioperative treatment for these patients. Anesthesiologists may have to deal with a patient with myasthenia gravis in different types of surgical interventions. The protocol for anesthesia and perioperative management of these patients
Eizaguirre, María Bárbara; Aguirre, Florencia; Yastremiz, Cecilia; Vanotti, Sandra; Villa, Andrés
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. age 43.9 ± 14.8, years of education 10.9 ± 3.3. 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.
María Bárbara Eizaguirre
Full Text Available 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
Full Text Available Objective. To present a case of maternal myasthenia gravis in pregnancy and give a systematic review of the literature. Case. We report the case of a 38-year-old parturient with a life-threatening complication of immune-mediated myasthenia gravis shortly after an elective cesarean section on patient's request under spinal anesthesia at 35 + 3 weeks of gestation. The newborn was transferred to the pediatric unit for surveillance and did not show any signs of muscular weakness in the course of time. The mother developed a respiratory insufficiency on the second day postpartum. The myasthenic crisis led to a progressive dyspnoea within minutes, which exacerbated in a secondary generalized seizure with cardiac-circulatory arrest. After successful cardiopulmonary resuscitation, the patient was transferred to intensive care. The interdisciplinary therapeutic approach included ventilatory assistance via endotracheal intubation, parenteral pyridostigmine, azathioprine, and steroids. By interdisciplinary measures, a stable state was regained. Conclusion. Myasthenia gravis especially when associated with pregnancy is a high-risk disease. As this disease predominantly occurs in women of reproductive age, it is important to be aware of this condition in obstetrics and its interdisciplinary diagnostic and therapeutic management.
Silvia EstellÃ©s MartÃnez
Full Text Available Resumen: Este trabajo presenta el modelado de un quadrotor como un sistema multicuerpo llevado a cabo mediante el software Vehicle- Sim, en el que los diferentes componentes del sistema son descritos mediante una estructura paterno-filial seÃ±alando las restricciones fÃsicas entre ellos. Los modelos estructural y aerodinÃ¡mico han sido desarrollados mediante este software, ampliamente utilizado en la simulaciÃ³n del comportamiento dinÃ¡mico de vehÃculos.Sobre el modelo resultante se he desarrollado un algoritmo de control basado en la metodologia PVA con la finalidad de obtener un seguimiento de trayectoria mediante acciones de control suaves. Empleando la metodologÃa convencional de control PVA no es posible estabilizar el vehÃculo en todos los rangos de posicionamiento lateral (y y longitudinal (x. En este artÃculo los autores muestran como esta limitaciÃ³n en el diseÃ±o de una estrategia de control PVA convencional es solventada con una modificaciÃ³n consistente en sustituir los parÃ¡metros constantes del PVA clÃ¡sico por funciones dependientes del desplazamiento.El sistema de control es implementado para adecuarse a los requerimientos de las actuaciones y se diseÃ±a sobre la plataforma de simulaciÃ³n multidominio Simulink. Con la finalidad de obtener una importante mejora en la respuesta de posicionamiento, se im- plementa un generador de trayectorias continuas.Una vez que el modelo es desarrollado y el sistema de control implementado, los autores presentan el modelo matemÃ¡tico y los resultados de las simulaciones realizadas. Ãstas validan el empleo tanto de la metodologÃa de control PVA aplicada, como de la alimentaciÃ³n de trayectorias predefinidas, no sÃ³lo para la posiciÃ³n, sino tambiÃ©n para la velocidad y aceleraciÃ³n. Abstract: In this work the authors present the modelling of a quadrotor as a multibody system carried out with the software VehicleSim, in which the different
BACK GROUND: Myasthenia gravis (MG) is generally a rare disorder and may thus be easily misdiagnosed. Misdiagnosis is even more likely when the presentation is atypical. OBJECTIVE: To present and discuss an unusual presentation of myasthenia gravis METHOD: A 67-year-old man presented with progressive ...
Background : Nemaline rod disease is a congenital myopathy, presentation of which may mimic myasthenia gravis. Methods : We report a suspected case of nemaline rod disease in a female adolescent who presented with features similar to myasthenia gravis but failed to respond effectively to its conventional management ...
Myasthenia gravis (MG) is an acquired autoimmune disorder causing skeletal muscle fatigue and weakness. This is a report of one woman and her daughter presenting with myasthenia and gravis and Grave\\'s disease. It highlights possible hereditary component of this condition which has not been commonly reported in ...
Impacto de la vacuna conjugada en la incidencia de meningitis por Haemophilus influenzae en el Distrito Federal de Brasil: resultados de tres años de seguimiento Impact of anti-Hib conjugate vaccine on the incidence of Haemophilus influenzae meningitis in Brazil's Federal District: results of a three-year follow-up
Helen Selma de Abreu Freitas
Full Text Available INTRODUCCIÓN: Haemophilus influenzae del serotipo b (Hib es todavía un importante agente causal de procesos infecciosos. Su variante encapsulada es la causa de formas invasoras de enfermedad. En algunas poblaciones aborígenes, la incidencia de enfermedades causadas por Hib es mayor de 400 por 100 000 niños menores de 5 años. En los decenios de 1970 y 1980, tras la identificación de anticuerpos protectores contra la cápsula de Hib, se desarrollaron vacunas contra este microorganismo. OBJETIVO: Estimar el impacto que ha tenido desde su introducción, en marzo de 1998, la vacunación contra Hib en el Distrito Federal de Brasil. MÉTODO: Con los datos de base poblacional del Sistema de Vigilancia de la Secretaría de Salud del Distrito Federal de Brasil, se compararon las tasas de incidencia de meningitis correspondientes a los tres años anteriores y posteriores a la introducción de la vacuna. Se compararon también los cambios en su tendencia. RESULTADOS: Al comparar los dos períodos se advierte una reducción de aproximadamente 90% en la incidencia de meningitis por Hib, cambio que no ocurre en el caso de las meningitis ocasionadas por otros agentes bacterianos. Se registró un aumento proporcional de los casos entre los niños de 6 meses de edad y menores, debido a la reducción de la incidencia entre los mayores de esa edad. CONCLUSIÓN: La introducción de la vacuna conjugada en el Distrito Federal de Brasil redujo de 168 por 100 000 (1995-1997 a 15 por 100 000 (1999-2001 la incidencia de meningitis por Hib entre niños de 7 a 35 meses. Esto representa una reducción de 91,1%.INTRODUCTION: Type b Haemophilus influenzae (Hib continues to be an important causative agent of various infectious processes, and its encapsulated strains cause invasive disease. In some aboriginal populations, the incidence of Hib infections in children under five is greater than 400 per 100 000. In the seventies and eighties, vaccines against Hib were
Anestesia ambulatorial para radioterapia em paciente portador de miastenia gravis: relato de caso Anestesia ambulatorial para radioterapia en paciente portador de miastenia gravis: relato de caso Outpatient anesthesia for radiotherapy in a patient with myasthenia gravis: case report
Raquel Marcondes Bussolotti
Full Text Available JUSTIFICATIVA E OBJETIVOS: A miastenia gravis (MG é doença auto-imune rara, caracterizada pela redução de receptores nicotínicos na junção neuromuscular, com incidência de 14/100.000. O objetivo deste relato foi descrever o caso de paciente portador de miastenia gravis submetido à anestesia geral balanceada, para tratamento radioterápico de carcinoma espinocelular de parótida, em regime ambulatorial. RELATO DO CASO: Paciente do sexo masculino, 87 anos, 87 kg, estado físico ASA III, com história de miastenia gravis; acidente vascular encefálico prévio (AVE; marca-passo por bloqueio atrioventricular total; hipertensão arterial sistêmica (HAS. Foram programadas sete sessões de radioterapia de 20 minutos para tratamento de tumor de parótida, sob anestesia geral. Na sala de radioterapia foi monitorizado com cardioscópio, monitor de pressão arterial não-invasiva, oxímetro de pulso, e submetido à anestesia geral com propofol e sevoflurano. Após a indução, foi mantido em ventilação espontânea com cânula de Guedel e cateter nasofaríngeo com O2 /sevoflurano, para acomodação da máscara imobilizadora. Na sala de recuperação pós-anestésica, não apresentou complicações. CONCLUSÕES: A escolha dos anestésicos e o acompanhamento clínico criterioso permitiram a boa evolução do paciente, com AVE prévio, cardiopata e idoso, submetido à anestesia geral balanceada para procedimento de aplicação de radioterapia, em regime ambulatorial.JUSTIFICATIVA Y OBJETIVOS: La Miastenia Gravis (MG es una enfermedad auto inmune rara, caracterizada por la reducción de receptores nicotínicos en la junción neuromuscular, con incidencia de 14/100.000. El objetivo de este relato fue el de describir el caso del paciente portador de miastenia gravis sometido a la anestesia general balanceada, para tratamiento radioterápico de carcinoma espinocelular de parótida en régimen ambulatorial. RELATO DEL CASO: Paciente del sexo masculino
Mazzoli, Marco; Ariatti, Alessandra; Valzania, Franco; Kaleci, Shaniko; Tondelli, Manuela; Nichelli, Paolo F; Galassi, Giuliana
50%-60% of patients with ocular myasthenia gravis (OMG) progress to generalized myasthenia gravis (GMG) within two years. The aim of our study was to explore factors affecting prognosis of OMG and to test the predictive role of several independent clinical variables. We reviewed a cohort of 168 Caucasian patients followed from September 2000 to January 2016. Several independent variables were considered as prognostic factors: gender, age of onset, results on electrophysiological tests, presence and level of antibodies against acetylcholine receptors (AChR Abs), treatments, thymic abnormalities. The primary outcome was the progression to GMG and/or the presence of bulbar symptoms. Secondary outcomes were either achievement of sustained minimal manifestation status or worsening in ocular quantitative MG subscore (O-QMGS) or worsening in total QMG score (T-QMGS), assessed by Myasthenia Gravis Foundation of America (MGFA) quantitative scores. Changes in mental and physical subscores of health-related quality of life (HRQoL) were assessed with SF-36 questionnaire. Variance analysis was used to interpret the differences between AChR Ab titers at different times of follow up among the generalized and non-generalized patients. Conversion to GMG occurred in 18.4% of patients; it was significantly associated with sex, later onset of disease and anti-AChR Ab positivity. Antibody titer above the mean value of 25.8 pmol/mL showed no significant effect on generalization. Sex and late onset of disease significantly affected T-QMGS worsening. None of the other independent variables significantly affected O-QMGS and HRQoL. Sex, later onset and anti-AChR Ab positivity were significantly associated with clinical worsening.
Jens A. Petersen
Full Text Available We report a 54-year-old woman who suffered a commotio cerebri, whiplash injury and a chest trauma with sternal fracture due to a high-velocity car accident. Two months later, she developed unilateral ptosis and blurred vision, which worsened during the day. Multiple diagnoses were suggested, ranging from thoracic outlet syndrome towards depression. Symptoms persisted and five years later, the patient consulted a neurologist. Laboratory analysis revealed significantly elevated levels of antibodies to acetylcholine receptors, and the diagnosis of myasthenia gravis was made. Speculatively, the damage of retrosternal thymic remnants due to a sternal fracture might have precipitated the condition or exacerbated subclinical disease.
Esguerra de Cárdenas Irene
Full Text Available
El "programa de apoyo y seguimiento al hogar de personas con antecedentes de enfermedad mental" se inició formalmente en marzo de 1993 en la Clínica Nuestra Señora de la Paz. Surgió de la necesidad de implementar acciones concretas al egreso de los pacientes de la institución, tendientes a reforzar la responsabilidad de éstos en su tratamiento y manejo de la enfermedad en el hogar y además de ofrecer un recurso de apoyo para escuchar sus inquietudes y preocupaciones. El propósito del programa es proporcionar al enfermo mental y a su familia un apoyo permanente al egresar de la Institución, en lo relacionado con el manejo de dificultades o problemas que se presenten durante el proceso de la enfermedad, el tratamiento y la reinserción del paciente al hogar y al medio social. Dentro de las estrategias de trabajo se contemplan reuniones semanales con grupos de pacientes próximos a salir de la clínica, reuniones periódicas de reforzamiento con personas inscritas al programa, seguimiento telefónico o mediante entrevistas individuales y/o familiares. Hasta el momento han asistido a las reuniones de egreso cerca de 400 pacientes y se han inscrito voluntariamente al programa 150 personas.
A number of papers of the author have been summarized devoted to gravi-inertial systems of reference in which the following problems are solved: a) analogs of inertial systems of reference (ISR), gravi-ISR, have been introduced into the general relativity the ory (GRT); b) using transformations between such ISR as symmetry transformation, obtained by variational methods are values with clear physical sense; c) using the gravi-ISR basis as the zero level of the deformation reading, the theory of elasticity in GRT has been constructed and someof its applications considered. The results are compared with those of other authors
Bettini, Mariela; Gonorazky, Hernan; Chaves, Marcelo; Fulgenzi, Ernesto; Figueredo, Alejandra; Christiansen, Silvia; Cristiano, Edgardo; Bertini, Enrico S; Rugiero, Marcelo
Cases of acquired rippling muscle disease in association with myasthenia gravis have been reported. We present three patients with iRMD (immune-mediated rippling muscle disease) and AChR-antibody positive myasthenia gravis. None of them had thymus pathology. They presented exercise-induced muscle rippling combined with generalized myasthenia gravis. One of them had muscle biopsy showing a myopathic pattern and a patchy immunostaining with caveolin antibodies. They were successfully treated steroids and azathioprine. The immune nature of this association is supported by the response to immunotherapies and the positivity of AChR-antibodies. Copyright © 2016 Elsevier B.V. All rights reserved.
Carvajal Escobar Yesid
Full Text Available El sistema de monitoreo y seguimiento permite identificar, a través de indicadores e índices, la sostenibilidad del recurso hídrico en el sector agrícola de la microcuenca Centella (Dagua-Valle del Cauca. Este modelo de monitoreo permite evaluar continua y sistemáticamente el progreso y los cambios ocasionados por la ejecución de un conjunto de actividades en un período de tiempo determinado. De igual manera, a través de este sistema es posible realizar un seguimiento y verificar en qué medida se cumplen las metas propuestas de sostenibilidad del recurso hídrico en el sector agrícola a nivel: biofísico, tecnológico, político-institucional y socioeconómico.
Wilmar Loaiza Cerón
Full Text Available El sistema de monitoreo y seguimiento permite identificar, a través de indicadores e índices, la sostenibilidad del recurso hídrico en el sector agrícola de la microcuenca Centella (Dagua-Valle del Cauca. Este modelo de monitoreo permite evaluar continua y sistemáticamente el progreso y los cambios ocasionados por la ejecución de un conjunto de actividades en un período de tiempo determinado. De igual manera, a través de este sistema es posible realizar un seguimiento y verificar en qué medida se cumplen las metas propuestas de sostenibilidad del recurso hídrico en el sector agrícola a nivel: biofísico, tecnológico, político-institucional y socioeconómico.
Liang, T; Boulos, M I; Murray, B J; Krishnan, S; Katzberg, H; Umapathy, K
Myasthenia gravis (MG) is an autoimmune neuromuscular disorder resulting from skeletal muscle weakness and fatigue. An early common symptom is fatigable weakness of the extrinsic ocular muscles; if symptoms remain confined to the ocular muscles after a few years, this is classified as ocular myasthenia gravis (OMG). Diagnosis of MG when there are mild, isolated ocular symptoms can be difficult, and currently available diagnostic techniques are insensitive, non-specific or technically cumbersome. In addition, there are no accurate biomarkers to follow severity of ocular dysfunction in MG over time. Single-fiber electromyography (SFEMG) and repetitive nerve stimulation (RNS) offers a way of detecting and measuring ocular muscle dysfunction in MG, however, challenges of these methods include a poor signal to noise ratio in quantifying eye muscle weakness especially in mild cases. This paper presents one of the attempts to use the electric potentials from the eyes or electrooculography (EOG) signals but obtained from three different forms of sleep testing to differentiate MG patients from age- and gender-matched controls. We analyzed 8 MG patients and 8 control patients and demonstrated a difference in the average eye movements detected between the groups. A classification accuracy as high as 68.8% was achieved using a linear discriminant analysis based classifier.
Sieb, J P
This paper provides a thorough overview of the current advances in diagnosis and therapy of myasthenia gravis (MG). Nowadays the term ‘myasthenia gravis’ includes heterogeneous autoimmune diseases, with a postsynaptic defect of neuromuscular transmission as the common feature. Myasthenia gravis should be classified according to the antibody specificity [acetylcholine, muscle-specific receptor tyrosine kinase (MuSK), low-density lipoprotein receptor-related protein 4 (LRP4), seronegative], thymus histology (thymitis, thymoma, atrophy), age at onset (in children; aged less than or more than 50 years) and type of course (ocular or generalized). With optimal treatment, the prognosis is good in terms of daily functions, quality of life and survival. Symptomatic treatment with acetylcholine esterase inhibition is usually combined with immunosuppression. Azathioprine still remains the first choice for long-term immunosuppressive therapy. Alternative immunosuppressive options to azathioprine include cyclosporin, cyclophosphamide, methotrexate, mycophenolate mofetil and tacrolimus. Rituximab is a promising new drug for severe generalized MG. Emerging therapy options include belimumab, eculizumab and the granulocyte– macrophage colony-stimulating factor. One pilot study on etanercept has given disappointing results. For decades, thymectomy has been performed in younger adults to improve non-paraneoplastic MG. However, controlled prospective studies on the suspected benefit of this surgical procedure are still lacking. In acute exacerbations, including myasthenic crisis, intravenous immunoglobulin, plasmapheresis and immunoadsorption are similarly effective. PMID:24117026
Maria F. Finnis
Full Text Available Myasthenia gravis (MG is an autoimmune disease in which antibodies are directed against the postsynaptic membrane of the neuromuscular junction, resulting in muscle weakness and fatigability. Juvenile myasthenia gravis (JMG is a rare condition of childhood and has many clinical features that are distinct from adult MG. Prepubertal children in particular have a higher prevalence of isolated ocular symptoms, lower frequency of acetylcholine receptor antibodies, and a higher probability of achieving remission. Diagnosis in young children can be complicated by the need to differentiate from congenital myasthenic syndromes, which do not have an autoimmune basis. Treatment commonly includes anticholinesterases, corticosteroids with or without steroid-sparing agents, and newer immune modulating agents. Plasma exchange and intravenous immunoglobulin (IVIG are effective in preparation for surgery and in treatment of myasthenic crisis. Thymectomy increases remission rates. Diagnosis and management of children with JMG should take account of their developmental needs, natural history of the condition, and side-effect profiles of treatment options.
Whereas the coexistence of different autoimmune or rheumatologic diseases with myasthenia gravis (MG) is well documented, its combination with sarcoidosis is extremely rare. Presented here is an interesting case with coexisting MG and sarcoidosis.
Kirzinger, Lukas; Khomenko, Andrei; Schulte-Mattler, Wilhelm; Backhaus, Roland; Platen, Sabine; Schalke, Berthold
Adult and pediatric patients suffering from MuSK (muscle-specific kinase) -antibody positive myasthenia gravis exhibit similar features to individuals with acetylcholine receptor (AChR) antibodies, but they differ in several characteristics such as a predominant bulbar, respiratory and neck weakness, a generally worse disease severity and a tendency to develop muscle atrophy. Muscle atrophy is a rare phenomenon that is usually restricted to the facial muscles. We describe a girl with MuSK-antibody positive myasthenia gravis who developed a myopathy with severe generalized muscular weakness, muscle atrophy, and myopathic changes on electromyography. This is the first published example of a generalized myopathic syndrome in myasthenia gravis. We review the relevant literature and discuss the hypothesis of a mitochondrial myopathy as a pathogenic mechanism in MuSK-antibody positive myasthenia gravis. Copyright Â© 2016 Elsevier Inc. All rights reserved.
J Gordon Millichap
Full Text Available A late infantile case of myasthenia gravis (MG with anti-muscle-specific receptor tyrosine kinase (MuSK high antibody (Ab titer is reported from Kyushu University, Fukuoka; and Nagasaki University, Japan.
Shah, Poornima A; Wadia, Pettarusp Murzban
Man-in-the-barrel syndrome (MBS) is an uncommon presentation due to bilateral, predominantly proximal muscle weakness that has not been described to be associated with myasthenia gravis. We describe a case of myasthenia gravis presenting as MBS. Additionally, he had significant wasting of the deltoids bilaterally with fibrillations on electromyography (EMG) at rest and brief duration (3-6 ms) bi/triphasic motor unit potentials (MUPs) on submaximal effort apart from a decremental response on r...
Croitoru Cristina Georgiana
Full Text Available Myasthenia gravis is one of the neurological diseases with a relatively recent history, full of mistakes, in which the British and German neurology schools have attempted to find answers when confronted with the unknown. The paper aims at making a historical account of the disease from its discovery in the 16th century, when the first case of myasthenia gravis was medically diagnosed, to the beginning of the 20th century, when the dawn of modern therapy started to show.
Full Text Available El término penfigoide de membranas mucosas designa un grupo de enfermedades vesículo-bulosas autoinmunes crónicas, que afectan predominantemente mucosas. El presente artículo describe dos casos clínicos de penfigoide de membranas mucosas con compromiso gingival y tuvo como objetivo resaltar la importancia de un correcto diagnóstico de la lesión y discutir la conducta terapéutica. Fueron realizados diversos exámenes complementares como, examen de Nikolsky, biopsia incisional, investigación de células LE y de anticuerpos antinucleares, además de la evaluación de médicos especializados en dermatología y oftalmología. Fue fundamental la substitución de dentífricos, la orientación nutricional y psicológica, el cuidado con la higiene, además del uso de corticoides tópicos. Hubo periodos de remisión y exacerbación del cuadro clínico durante el seguimiento, fue necesario el ajuste en la terapéutica y refuerzo en los cuidados con la higiene bucal. Es muy importante la interacción multidisciplinaria en la atención de estos casos para el control del tratamiento y seguimiento, además de reforzar todas las orientaciones y cuidados en lo que se refiere a la salud bucal y cautela en el uso de corticoides.
Vrinten, C.; van der Zwaag, A.M.; Weinreich, S.S.; Scholten, R.J.; Verschuuren, J.J.
BACKGROUND: Myasthenia is a condition in which neuromuscular transmission is affected by antibodies against neuromuscular junction components (autoimmune myasthenia gravis, MG; and neonatal myasthenia gravis, NMG) or by defects in genes for neuromuscular junction proteins (congenital myasthenic
Lichiardopol, Corina; Herlea, V; Ioan, Virginia; Tomulescu, V; Mixich, F
Both hypergonadotropic hypogonadism and myasthenia gravis can be parts of type II autoimmune polyendocrine syndrome and association between the two disorders has been reported in few cases. A 14 year old male patient with a personal history of bilateral cryptorchidism and ptosis was referred for delayed puberty. Clinical examination revealed eunuchoid habitus, small, soft testes, gynecomastia, ptosis, a myasthenic deficit score of 22.5 points and an IQ of 84 points. Decreased testosterone (0.064 ng/mL) and elevated LH (64.5 mUI/mL) were consistent with hypergonadotropic hypogonadism and karyotype was normal: 46,XY. Thyroid function, haematologic evaluation, BUN, electrolytes, and glycemia were in the normal range. Therapy consisted of anticholinesterase inhibitors, immunosuppressants, corticotherapy, testosterone; thoracoscopic thymectomy was performed showing thymic lymphoid hyperplasia on histopathologic examination. Myasthenic score improved (12.5 points), progressive virilization occurred, and a year later the patient presented with cushingoid features and obesity.
Jordan, Berit; Schweden, Tabea L K; Mehl, Theresa; Menge, Uwe; Zierz, Stephan
Cognitive fatigue has frequently been reported in myasthenia gravis (MG). However, objective assessment of cognitive fatigability has never been evaluated. Thirty-three MG patients with stable generalized disease and 17 healthy controls underwent a test battery including repeated testing of attention and concentration (d2-R) and Paced Auditory Serial Addition Test. Fatigability was based on calculation of linear trend (LT) reflecting dynamic performance within subsequent constant time intervals. Additionally, fatigue questionnaires were used. MG patients showed a negative LT in second d2-R testing, indicating cognitive fatigability. This finding significantly differed from stable cognitive performance in controls (P fatigue was significantly higher in MG patients compared with controls (P fatigue is not correlated with objective findings. Muscle Nerve 56: 449-457, 2017. © 2016 Wiley Periodicals, Inc.
Full Text Available INTRODUCCIÓN El farmacéutico tiene que demostrar que es capaz de realizar e implementar unos servicios de calidad y su eficiencia antes de poder exigir la financiación de estos servicios. El objetivo general del presente estudio es analizar los costes derivados de la prestación de un servicio de seguimiento para pacientes con enfermedad cardiovascular y evaluar la posible eficiencia en el mismo. MATERIAL Y MÉTODOS Para la recogida y análisis de datos se utilizó el programa Cuídate Corazón® (Laboratorios Lácer y para establecer un posible precio al servicio se aplicó la estrategia de Kotler, utilizando el método orientado al coste. Como evaluación de la efectividad del servicio se contabilizaron los RNM detectados al paciente y la aceptación por parte del médico de la intervención del farmacéutico. Se realizó una encuesta para ver el grado de satisfacción del paciente. RESULTADOS El coste por paciente y mes del servicio para cada farmacia resultó ser de 2,48 . Se detectaron 10 RNM de los que en un 78% la intervención farmacéutica fue aceptada por el médico, que hizo cambios en la medicación. El paciente resulta estar muy satisfecho por este servicio, que mejora su percepción del farmacéutico como profesional sanitario y cree que el farmacéutico debe ser remunerado por el servicio. DISCUSIÓN Se considera que se ha generado un valor añadido a la atención del paciente, creando una demanda del servicio prestado. Las farmacias participantes seguirán trabajando con el programa Cuídate Corazón ®, y establecerán una remuneración para el farmacéutico por parte del paciente, ya que se considera que esto responsabiliza al profesional y mejora la implicación del paciente en beneficio de su salud
Ramos Berumen, Carlos; Ramirez Benitez, Juan Rafael; Lopez Perez, Manuel de Jesus; Beltran Adan, Jose; Lagunas Mendoza, Javier [Instituto de Investigaciones Electricas, Cuernavaca, Morelos (Mexico)
A solar concentrator prototype of Parabolic Trough (PT) for solar process heat generation has been designed, manufactured and put into operation by the Instituto de Investigaciones Electricas. By means of a sun tracking mechanism controlled by an automatic device, the PT is moving from East to West during the day then this way the solar energy is focused continually over the lineal receiver. Such movement is reached thanks to the electronic module fitted with special software. In this paper, the design of an electronic module based on calculation algorithm of the sun position along the day and over the year which controls the PT sun tracking is presented. The main purpose of the development is to integrate a control system which is using the algorithm of low cost (it was tested in a commercial system with success). [Spanish] El Instituto de Investigaciones Electricas (IIE) ha disenado, fabricado y puesto en operacion un prototipo de concentrador solar de canal parabolico para la generacion de calor de proceso. La tecnologia termosolar a concentracion de canal parabolica mantiene el concentrador moviendose de este a oeste durante el dia, enfocando continuamente el sol en el receptor lineal, mediante un mecanismo y un control automatico que permite el seguimiento aparente del sol. Se ha desarrollado el software para el control del mecanismo de seguimiento solar. En este articulo se presenta el diseno de un modulo electronico que controla el mecanismo de seguimiento solar de un canal parabolico, el cual tiene sus bases en un algoritmo que calcula la posicion del sol durante todo el dia y a lo largo del ano. El proposito principal es integrar un sistema de control de bajo costo, que utilice el algoritmo desarrollado por el IIE, el cual fue probado con exito en un sistema comercial para el seguimiento solar de un canal parabolico.
Full Text Available Abstract Background The myasthenia gravis is twice as common in women as in men and frequently affects young women in the second and third decades of life, overlapping with the childbearing years. Generally, during pregnancy in one third of patients the disease exacerbates, whereas in two thirds it remains clinically unchanged. Complete remission can occur in some patients. Methods To describe the clinical course, delivery and neonatal outcome of 18 pregnant women with the diagnosis of myasthenia gravis. Retrospective chart review of pregnant patients with myasthenia gravis, followed at the National Institute of Perinatology in Mexico City over an 8-year period. Data was abstracted from the medical records on the clinical course during pregnancy, delivery and neonatal outcome. Results From January 1, 1996 to December 31, 2003 18 patients with myasthenia gravis were identified and included in the study. The mean ± SD maternal age was 27.4 ± 4.0 years. During pregnancy 2 women (11% had an improvement in the clinical symptoms of myasthenia gravis, 7 women (39% had clinical worsening of the condition of 9 other patients (50% remained clinically unchanged. Nine patients delivered vaginally, 8 delivered by cesarean section and 1 pregnancy ended in fetal loss. Seventeen infants were born at mean ± SD gestational age of 37.5 ± 3.0 weeks and a mean birth weight of 2710 ± 73 g. Only one infant presented with transient neonatal myasthenia gravis. No congenital anomalies were identified in any of the newborns. Conclusions The clinical course of myasthenia gravis during pregnancy is variable, with a significant proportion of patients experiencing worsening of the clinical symptoms. However, neonatal transient myasthenia was uncommon in our patient population.
Daysi Ruiz Fuentes
Full Text Available El perfeccionamiento de los procesos que se desarrollan en las Instituciones de Educación Superior resulta hoy prioridad del Estado en Ecuador. La sociedad ecuatoriana exige a las universidades respuestas atinadas a los problemas que subsisten en los diversos contextos laborales. Hoy el país requiere de procesos formativos pertinentes, que se enriquezcan sistemáticamente con las experiencias prácticas. El seguimiento a graduados es un proceso que permite una vinculación continua de la universidad con el entorno. Además de constatar la calidad de los profesionales que se forman, a la vez que lo perfecciona, al tomar en cuenta criterios valorativos de graduados y empleadores, entre otros actores. La presente investigación se desplegó en la carrera de Economía de la Facultad de Ciencias Económicas de la Universidad Laica “Eloy Alfaro” de Manabí, y su objetivo general estuvo encaminado al perfeccionamiento del seguimiento a graduados en esa carrera. En su desarrollo se aplicaron métodos teóricos, empíricos y estadísticos, y el resultado final es un procedimiento que declara explícitamente los principales actores de tan importante proceso, además de analizar aristas anteriormente no tratadas en este tipo de estudios, como el necesario acercamiento a los egresados. El procedimiento final propuesto, de aplicarse, debe contribuir a mejorar los resultados formativos de la carrera de Economía objeto de estudio, cuestión relevante, si se tiene en cuenta que este profesional merece especial atención, por ser el encargado de elevar a niveles superiores la economía nacional.PALABRAS CLAVE: proceso de formación profesional; seguimiento a graduados; carrera de Economía.PROCESS OF TRACKING GRADUATES IN THE FACULTY OF ECONOMIC SCIENCES OF THE LAY UNIVERSITY ELOY ALFARO FROM MANABÍ ABSTRACTThe improvement of the processes that take place in the institutions of higher education today is priority of the State in Ecuador. Ecuadorian
María del Mar Velasco Gámez
Full Text Available Based on recent financial scandals such as Enron (2001 and World-Com (2002 Corporate governance has become a key indicator of good management and social responsibility of the companies. This paper reflections are made on the degree to which the recommendations in the Unified Code of Good Spanish Governance are complied with by companies listed on the FTSE4Good Ibex, checking if these companies are putting the practice of good corporate governance to use as one of the dimensions that sets them apart from other companies. The results of our research highlight a high, albeit selective, degree of compliance with the recommendations.
ONAIDY ALINA BOHÓRQUEZ GAMBA
Full Text Available Este artículo ofrece una visión global del estado del arte de cinco trabajos de grado en modalidad de pasantía, realizados por estudiantes de pregrado de la Universidad Nacional de Colombia, Facultad de Enfermería, entre 2004 y 2007, desarrollados en los servicios de puerperio de cuatro hospitales de Bogotá, dentro del proyecto " Seguimiento domiciliario a la madre y al recién nacido durante el puerperio" . Refiere la vinculación de madres en periodo de puerperio, sus neonatos, padres y familias como grupo de apoyo, a quienes les realizaron seguimiento mediante llamadas telefónicas y visitas domiciliarias, para identificar factores de riesgo y signos de alarma que les comprometieran la salud y la vida. Congruentes con los hallazgos en las valoraciones de enfermería y el seguimiento, los estudiantes y docentes ejecutaron acciones de promoción de la salud y prevención de la enfermedad, mediante sesiones educativas en espacios institucional y domiciliario, dirigidas a fortalecer e iniciar oportunamente cuidados de enfermería, promover la participación de la familia, motivar la consulta temprana y ofrecer apoyo en lactancia materna, estilos de vida saludable, fortalecimiento del vínculo afectivo madre-hijo-padre, respetando las prácticas culturales. Las intervenciones de cuidado ejecutadas por estudiantes y docentes fueron estrategias para participar en la disminución de las tasas de morbilidad y mortalidad materna y neonatal por causas evitables. Además los trabajos evidenciaron que el cuidado de enfermería sobrepasa los espacios hospitalarios y reviste importancia en escenarios de vida cotidiana como el hogar.
Hugh J Freeman; Helen R Gillett; Peter M Gillett; Joel Oger
Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a glutenfree diet, but developed profound and generalized motor weakness with acetylcholine receptor antibody positive myasthenia gravis, a disorder reported to occur in about 1 in 5000. This possible relationship between myasthenia gravis and celiac disease was further explored in serological studies. Frozen stored serum samples from 23 acetylcholine receptor antibody positive myasthenia gravis patients with no intestinal symptoms were used to screen for celiac disease. Both endomysial and tissue transglutaminase antibodies were examined. One of 23 (or, about 4.3%) was positive for both IgA-endomysial and IgA tissue transglutaminase antibodies. Endoscopic studies subsequently showed duodenal mucosal scalloping and biopsies confirmed the histopathological changes of celiac disease. Celiac disease and myasthenia gravis may occur together more often than is currently appreciated. The presence of motor weakness in celiac disease may be a clue to occult myasthenia gravis, even in the absence of intestinal symptoms.
March, Katherine L; Samarin, Michael J; Sodhi, Amik; Owens, Ryan E
Purpose Pembrolizumab, a monoclonal antibody which inhibits the programmed cell death 1 receptor, has been shown to efficaciously enhance pre-existing immune responses to malignancies. However, safety concerns must also be considered as pembrolizumab use has been associated with several life-threatening immune-related adverse events (irAEs). We report a fatal case of pembrolizumab-induced myasthenia gravis in a patient with no prior myasthenia gravis history. Case report A 63-year-old male presented with right eyelid drooping, puffiness, blurred vision, and shortness of breath two weeks after an initial infusion of pembrolizumab. He was subsequently diagnosed with new onset acetylcholine-receptor positive myasthenia gravis. Despite aggressive treatment with corticosteroids, pyridostigmine, intravenous immunoglobulin, and plasmapheresis, the patient clinically deteriorated and ultimately expired from acute respiratory failure after a 12-day hospitalization. Discussion Current package labeling for pembrolizumab warns against various irAEs associated with its use including pneumonitis, colitis, and endocrinopathies. To date, only one case of new onset myasthenia gravis and two case reports of myasthenia gravis exacerbation have been identified. This case further highlights the mortality risk associated with development of irAEs. Conclusion While rare, evidence for the development of MG associated with pembrolizumab is growing. Prompt recognition of symptoms and discontinuation of pembrolizumab is necessary to help improve prognosis.
Full Text Available 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.
Asensio Castañeda, Eva María; Blanco Fernández, María Ascensión
La asignatura de Libre Elección “Desarrollo de Competencias” ofertada en la Universidad Europea de Madrid, está destinada a alumnos que cursan cualquier titulación que se imparte en dicha universidad. Su objetivo es que, por medio del uso de nuevas metodologías y herramientas de evaluación continua y seguimiento tales como la carpeta de aprendizaje, el alumno adquiera y desarrolle competencias tan importantes como: la gestión eficaz del tiempo, habilidades comunicativas, trabajo en equipo, li...
Blasco, Xavier; García-Nieto, Sergio; Reynoso-Meza, Gilberto
Resumen: Este documento muestra en detalle el proceso de simulación y evaluación multiobjetivo de las propuestas al Concurso en Ingeniería de Control 2012, Control autónomo del seguimiento de trayectorias de un vehículo cuatrirrotor, organizado por el Comité Español de Automática (CEA). El concurso busca ofrecer una herramienta de trabajo adicional que pueda servir para reforzar la educación en control y orientar a los alumnos de ingeniería sobre el tipo de problemas que un ingeniero de contr...
Lee, John-Ih; Jander, Sebastian
Myasthenia gravis is the most frequent acquired disorder of neuromuscular transmission. In the majority of cases, pathogenic antibodies against components of the postsynaptic muscle endplate membrane can be detected. In recent years there have been significant advances in the pathophysiological understanding and therapy of the disease. Areas covered: PubMed searches were conducted for the term 'myasthenia gravis' cross-referenced with the terms 'immunology', 'subgroups', 'antibody', 'ocular', 'thymoma', 'treatment' and 'thymectomy'. Additionally, we summarized the current state of immunopathology and therapy. Expert commentary: Immunological research defined new target antigens at the postsynaptic neuromuscular junction which along with clinical features allow a refined definition of disease subgroups. Overall the prognosis of myasthenia gravis with best possible symptomatic, immunosuppressive and supportive treatment is good but new immunomodulatory treatment options are developed for patients who do not respond well to the first line therapy. For most patients individually adapted long-term drug therapy is needed.
Komatsu, Midori; Tanaka, Makoto; Morimatsu, Mitsunori; Hirai, Shunsaku; Heshiki, Atsuko
We investigated whether CT had any advantage over pneumomediastinography (PMG) for the visualization of thymus in twenty-one patients with myasthenia gravis (MG). In two cases of thymoma which had been suspected with chest radiography, CT demonstrated an anterior mediastinal tumor distinguishable from other mediastinal organs, and for these cases PMG was not performed. Excluding three patients who were submitted to PMG only, CT of the anterior mediastinum was carried out in sixteen patients of MG, revealing thymic shadows in seven (44%). PMG followed by conventional tomography was done subsequently in three of these seven cases, for whom thymectomy was indicated because of uncontrollable myasthenic symptoms, and in all patients finger-like thymic shadows were disclosed. The vertical extension of thymus was more easily demonstrable by PMG than CT. PMG was carried out in six of nine patients in whom CT was negative, and in all cases thymic shadows were obvious with subsequent conventional tomography. Consequently, false negative rate of CT was at least 38% (6/16) with regard to the visualization of the nontumorous thymus. Although CT of the anterior mediastinum is useful as a screening method because of its non-invasiveness, its negative result does not rule out an absence of the pathologic thymus in view of its high false negative rate. In this regard, PMG is still necessary for the final determination of the thymic configuration in the MG patients. (J.P.N.)
Andersen, Jintana B; Gilhus, Nils Erik; Sanders, Donald B
Information from myasthenia gravis (MG) patients treated and evaluated for at least 2 years between 1980 and 2014 was reviewed to assess the effect of demographics, antibody status and titer, thymus histology, and clinical severity on outcome after 2, 5, and 10 years of treatment. Among 268 patients, 74% had acetylcholine receptor antibodies, 5% had muscle specific tyrosine kinase-antibodies, and 22% had neither. Optimal outcome was achieved by 64% of patients at 2 years of follow-up, 73% at 5 years, and 75% after 10 years. Optimal outcome was achieved more often in patients with late onset, in those who had thymectomy, and in those with ocular-only disease at maximum severity. The only consistent independent predictor of optimal outcome was onset after age 50 years on multivariate analysis. Prognosis is favorable for the majority of MG patients, regardless of age, maximum disease severity, or antibody status. Muscle Nerve, 2016 Muscle Nerve 54: 1041-1049, 2016. © 2016 Wiley Periodicals, Inc.
Rodolico, Carmelo; Parisi, Daniela; Portaro, Simona; Biasini, Fiammetta; Sinicropi, Stefano; Ciranni, Annamaria; Toscano, Antonio; Messina, Sonia; Musumeci, Olimpia; Vita, Giuseppe; Girlanda, Paolo
Myasthenia gravis (MG) is an autoimmune disorder presenting with fluctuating, fatigable muscle weakness. Initial symptoms classically involve ocular and proximal limb muscles. Rarely, MG may onset with unusual features, so it can be misdiagnosed with other neuromuscular diseases. To describe unusual and atypical presentations of MG in a large cohort of patients, considering and discussing diagnostic difficulties and pitfalls. We report on 21 out of 508 MG patients, coming to our department in the last 27 years and presenting with atypical or unusual features. The diagnosis was achieved performing a careful clinical examination, a proper neurophysiological assessment, the neostigmine test, the AChR and MuSK antibodies assay and chest CT-scan. Patients with atypical/unusual MG onset were the 4.4% of all MG patients population. We describe seven different clinical categories: asymmetric distal upper limbs weakness, foot drop, isolated triceps brachii weakness and foot drop, post exertional axial weakness with dropped head, acute facial dyplegia, limb-girdle MG and MG with sudden lower limbs weakness and recurrent falls. Atypical and unusual presentations may increase the risk to misdiagnose or delay MG diagnosis. Isolated limb-girdle presentation is the most frequent atypical form in our series.
Wang, Zhen; Yan, Yaping
Myasthenia gravis (MG) and neuromyelitis optica (NMO) are autoimmune channelopathies of the peripheral neuromuscular junction (NMJ) and central nervous system (CNS) that are mainly mediated by humoral immunity against the acetylcholine receptor (AChR) and aquaporin-4 (AQP4), respectively. The diseases share some common features, including genetic predispositions, environmental factors, the breakdown of tolerance, the collaboration of T cells and B cells, imbalances in T helper 1 (Th1)/Th2/Th17/regulatory T cells, aberrant cytokine and antibody secretion, and complement system activation. However, some aspects of the immune mechanisms are unique. Both targets (AChR and AQP4) are expressed in the periphery and CNS, but MG mainly affects the NMJ in the periphery outside of CNS, whereas NMO preferentially involves the CNS. Inflammatory cells, including B cells and macrophages, often infiltrate the thymus but not the target—muscle in MG, whereas the infiltration of inflammatory cells, mainly polymorphonuclear leukocytes and macrophages, in NMO, is always observed in the target organ—the spinal cord. A review of the common and discrepant characteristics of these two autoimmune channelopathies may expand our understanding of the pathogenic mechanism of both disorders and assist in the development of proper treatments in the future. PMID:29312313
Marcos R. G. de Freitas
Full Text Available A forma familiar da miastenia gravis é condição relativamente rara, ocorrendo em cerca de 3,4% dos pacientes miastênicos. Os autores registram os casos de dois irmãos não-gêmeos, apresentando miastenia gravis com acometimento predominantemente ocular desde o nascimento. Um terceiro irmão faleceu na infância, provavelmente com a mesma afecção. Os pais eram consanguíneos. Estudo da incidência da forma familiar da miastenia e de seus aspectos clínicos, genéticos e terapêuticos complementa os registros.
Full Text Available Fundamento La evolución del riesgo cardiovascular estimado, puede servir para valorar la efectividad de las diferentes intervenciones terapéuticas que se realizan en pacientes con seguimiento habitual en las consultas del médico de familia. El objetivo de este trabajo es comparar diferentes sistemas de evaluación del efecto de las intervenciones preventivas en la evolución del riesgo coronario en el seguimiento a largo plazo de personas hipertensas en Atención Primaria. Métodos: Estudio descriptivo longitudinal con seguimiento de 8.42 pacientes hipertensos de 34 a 70 años durante 6 años en dos centros de atención primaria, con una intervención de mejora de calidad (ciclo de mejora en el último año en uno de ellos. El seguimiento mínimo en atención primaria previo al inicio del estudio fue de dos años. Las variables principales fueron edad y sexo, presión arterial, lípidos, tabaquismo, diabetes y riesgo cardiovascular (RCV(Framingham-Wilson en la situación real y considerando constante la edad en el primer supuesto y los factores de riesgo en el segundo y el riesgo relativo. Resultados: Se encontró un descenso de la presión arterial sistólica y diastólica de 11,78 mmHg (IC95%:10,51-13,05 y 8,83 mmHg (IC95%:8,13-9,53 respectivamente y LDL-Colesterol 15,94 mg/dl (IC95%:11,77-20,12, un ascenso del HDLColesterol de 7,53 mg/dl (IC95:6,39-8,66, disminución del tabaquismo del 31% y un aumento de diabéticos. El riego coronario disminuyó 1,40(IC95%:0,87-1,93 puntos porcentuales, el RCV con edad constante descendió 3,84(IC95%:3,35-4,33 y con factores de riesgo constantes incrementó 3,06(IC95%:2,82- 3,29. El Riesgo relativo descendió de 2,50 a 1,85. Conclusiones: El envejecimiento puede enmascarar el efecto logrado por la atención sanitaria en el control del riesgo cardiovascular absoluto. El riesgo relativo podría ser una alternativa para monitorizar el seguimiento.
Álvarez-Cordovés, M M; Mirpuri-Mirpuri, P G; Pérez-Monje, A
Myasthenia gravis is an autoimmune disorder of neuromuscular transmission involving the production of autoantibodies directed against skeletal muscle receptors, in most cases of acetylcholine. Clinically it is characterized by the appearance of muscle weakness after prolonged activity, which tends to recover after a period of rest, or administration of acetylcholinesterase inhibitors. It is a relatively rare disease, although the prevalence has increased by improved diagnosis and increased longevity of the population. The diagnosis can be based on evidence after it is suspected using pharmacological, immunological or electrophysiology tests. Treatment can be divided into: symptomatic, short term and long term. We report the case of a patient who complained of diplopia, this muscle weakness being the most common initial symptom of the disease. Copyright © 2012 Sociedad Española de Médicos de Atención Primaria (SEMERGEN). Publicado por Elsevier España. All rights reserved.
Miguel Ángel Martín González
Full Text Available Introducción: el tratamiento videotoracoscópico permite la exéresis del timo y de la grasa peritímica, como en la cirugía abierta, pero con las ventajas del mínimo acceso. Objetivo: evaluar los resultados de esta vía con nuestros primeros pacientes. Métodos: se realizó un estudio descriptivo y prospectivo de 10 pacientes con miastenia gravis desde enero de 2007 hasta agosto de 2008, en el hospital "Hermanos Ameijeiras". Los resultados se presentaron en por cientos y para la relación de las variables se empleó chi cuadrado. Resultados: según la clasificación de Osserman de la miastenia, 8 se ubicaron en el grado II B, y 2 en el II A. El 50 % (5 de ellos tenía un timoma asociado. En el 50 % se emplearon 3 puertos de entrada. El tiempo quirúrgico varió de 60 a 180 minutos. Hubo un 37,5 % (3 de complicaciones posoperatorias sin mortalidad. En el informe anatomopatológico de la pieza (4 el 50 % tenía un timoma, todos en el estadio I de Masaoka. El 50 % (4 se encuentra en remisión y el otro 50 % en mejoría significativa. Conclusiones: la timectomía videotoracoscópica tiene grandes ventajas, ya que sin cambiar la técnica quirúrgica los pacientes se benefician de todas las ventajas del mínimo acceso.Introduction: the video-laparoscopy treatment allows the thymus exeresis and the peri-thymic fat like in the open surgery, but with the advantages of a minimal access. Objective: to assess the results of this route achieved in our first patients. Methods: a prospective and descriptive study was conducted in 10 patients presenting with myasthenia gravis from January, 2007 to August, 2008 admitted in the "Hermanos Ameijeiras" Clinical Surgical Hospital. The results were showed in percentages and for the relation of variables chi² was used. Results: according to the Osserman's classification of the myasthenia, 8 were located in the IIB degree and 2 in the IIA one. The 50 % (5 of them had an associated thymoma. In the 50 % three
Mourão, Aline Mansueto; Gomez, Rodrigo Santiago; Barbosa, Luiz Sergio Mageste; Freitas, Denise da Silva; Comini-Frota, Elizabeth Regina; Kummer, Arthur; Lemos, Stella Maris Aguiar; Teixeira, Antonio Lucio
The aims of the current study were 1) to evaluate the reliability and validity of the Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale and 2) to investigate the quality of life of Brazilian patients with myasthenia gravis and its determinants. This cross-sectional study included 69 patients with myasthenia gravis who underwent neurological evaluation and completed questionnaires regarding quality of life (the 36-item Short Form of the Medical Outcomes Study and the 15-item Myasthenia Gravis Quality of Life Scale), anxiety and depressive symptoms. The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale showed high internal consistency and good concurrent validity with the 36-item Short Form of the Medical Outcomes Study and its subscales. Determinants of quality of life in Brazilian patients with myasthenia gravis included the current status of myasthenia gravis as assessed by the Myasthenia Gravis Composite, the current prednisone dose and the levels of anxiety and depression. The Brazilian version of the 15-item Myasthenia Gravis Quality of Life Scale is a valid instrument. Symptom severity, prednisone dosage and anxiety and depression levels impact the quality of life of patients with myasthenia gravis.
cardiomyopathy, may occur3,4 and cardiac and respiratory involvement have been documented as indices of worse ... The heart is not involved in myasthenia gravis and electrocardiographic findings remain normal .... transmission and of Motor neurons. In: Berhman. RE, Kliegman RM, Jenson HB eds. Nelson extbook of ...
Full Text Available Introduction. Autoimmune polyglandular syndrome type 2 is defined as adrenal insufficiency associated with autoimmune primary hypothyroidism and/or with autoimmune type 1 diabetes mellitus, but very rare with myasthenia gravis. Case report. We presented a case of an autoimmune polyglandular syndrome, type 2 associated with myasthenia gravis. A 49-year-old female with symptoms of muscle weakness and low serum levels of cortisol and aldosterone was already diagnosed with primary adrenal insufficiency. Primary hypothyroidism was identified with low values of free thyroxine 4 (FT4 and raised values of thyroidstumulating hormone (TSH. The immune system as a cause of hypothyroidism was confirmed by the presence of thyroid antibodies to peroxidase and TSH receptors. Myasthenia gravis was diagnosed on the basis of a typical clinical feature, positive diagnostic tests and an increased titre of antibodies against the acetylcholine receptors. It was not possible to confirm the immune nature of adrenal insufficiency by the presence of antibodies to 21- hydroxylase. The normal morphological finding of the adrenal glands was an indirect confirmation of the condition as well as the absence of other diseases that might have led to adrenal insufficiency and low levels of both serum cortisol and aldosterone. Hormone replacement therapy, anticholinergic therapy and corticosteroid therapy for myasthenia gravis improved the patient’s general state of health and muscle weakness. Conclusion. This case report indicates a need to examine each patient with an autoimmune disease carefully as this condition may be associated with another autoimmune diseases.
Background. Myasthenia gravis (MG) is a treatable autoimmune disease characterised by fatiguable weakness of skeletal muscles. More than 85% of MG patients have antibodies to the acetylcholine receptor (AChR) at the neuromuscular junction or are seropositive for MG (SPMG). In the developed world the incidence of ...
Aim:To evaluate the management of post thymectomy myasthenia gravis (MG) patient in our centre; to highlight those aspects of patient care that could improve outcome and to serve as a bench mark for developing a standardized protocol for management. Method: A retrospective study of 5 cases of post thymectomy MG ...
Sehgal, Shekhar; Rebello, Roshan; Wolmarans, Louise; Elston, Marianne
We present two patients with Graves' disease and concurrent myasthenia gravis. The impact of the dual diagnosis on the clinical course and the potential for a delayed diagnosis of myasthenia gravis is discussed. Patient 1, a 28-year-old man was diagnosed with Graves' disease following his second respiratory arrest. His history was strongly suggestive of a second pathology. Patient 2, a 66-year-old Cantonese woman with established Graves' disease presented with thionamide-related neutropaenia. Examination revealed bilateral ptosis and right lateral rectus palsy. Both patients had thyrotoxicosis secondary to Graves' disease with concurrent myasthenia gravis. Although neuromuscular weakness is common in Graves' disease, coexisting myasthenia gravis (MG) is rare and can cause profound morbidity. Ocular signs in both diseases may cause diagnostic confusion although ptosis suggests coexisting MG. In both cases, the thyrotoxicosis delayed the diagnosis of MG. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.
Holtsema, H; Mourik, J; Rico, RE; Falconi, [No Value; Kuks, JBM; Oosterhuis, HJGH
We carried out an epidemiological study on thr prevalence and annual incidence of myasthenia gravis on tropical islands Curacao and Aruba in the period 1980 1995. Twenty-one patients (seven men and 14 women) were identified. The point prevalence increased from 29 per million in 1980 to about 70 per
Full Text Available Abstract Ectopic cervical thymoma is rare and is often misdiagnosed as a thyroid tumor or other malignancy. Ectopic thymic tissue can be found along the entire thymic descent path during embryogenesis. However, a thymoma arising from such ectopic thymic tissue is extremely rare. Herein we report a patient with ectopic cervical thymoma and myasthenia gravis (MG and discuss the management.
Full Text Available An important problem in management of the case with myasthenia gravis (MG is the control of exacerbation. There are several possible causes of exacerbation of MG including the use of drug. Here, the authors report a case of MG exacerbation and diarrhea associated with erythromycin treatment.
Barnett, Carolina; Bril, Vera; Kapral, Moira; Kulkarni, Abhaya V; Davis, Aileen M
To study responsiveness and meaningful change of the Myasthenia Gravis Impairment Index (MGII) and its relative efficiency compared to other measures. We enrolled 95 patients receiving prednisone, IV immunoglobulin (IVIg), or plasma exchange (PLEX) and 54 controls. Patients were assessed with the MGII and other measures-including the Quantitative Myasthenia Gravis Score, Myasthenia Gravis Composite, and Myasthenia Gravis Activities of Daily Living-at baseline and 3-4 weeks after treatment. Statistical markers of responsiveness included between-groups and within-group differences, and we estimated the relative efficiency of the MGII compared to other measures. Patient-meaningful change was assessed with an anchor-based method, using the patient's impression of change. We determined the minimal detectable change (MDC) and the minimal important difference (MID) at the group and individual level. Treated patients had a higher change in MGII scores than controls (analysis of covariance p 1 favoring the MGII. The MGII demonstrated responsiveness to prednisone, IVIg, and PLEX in patients with myasthenia. There is a differential response in ocular and generalized symptoms to type of therapy. The MGII has higher relative efficiency than comparison measures and is viable for use in clinical trials. Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
Yang, Jing; Liu, Chanchan; Li, Tao; Li, Chengyan
To compare the post-thymectomy prognosis in different conditions of myasthenia gravis (MG) patients with thymus hyperplasia. Collecting medical record and carrying out the follow-up study of 123 myasthenia gravis patients with thymus hyperplasia who have underwent thymectomy during the period between 2003 and 2013. Dividing into different groups based on gender, age of onset, duration of disease and Myasthenia Gravis Association of America (MGFA) clinical classification to analyze different prognosis in different groups. Complete stable remission (CSR) was achieved in 71 of 123 patients (59.5%). There is no gender-related difference in achieving CSR. Patients with early onset of MG (≤40 years old) or disease duration less than 12 months had significantly higher CSR rates than those with late onset of MG (>40 years old) or disease duration more than 12 months respectively, while no difference was found in remission rate between MGFA clinical classification I and MGFA II. Myasthenia gravis patients with thymus hyperplasia who had thymectomy are proved to possess greater chance of achieving CSR. The onset age of disease and duration are the prognostic factors.
Gil García MI
Full Text Available En el contexto de un estudio de investigación sobre priorización de facilitadores para la implantación del Seguimiento Farmacoterapéutico en las farmacias comunitarias españolas, se realizó un grupo focal compuesto por miembros de Foro de Atención Farmacéutica en Farmacia Comunitaria. El objetivo era explorar sus opiniones sobre los facilitadores priorizados en dicho estudio que se habían definido como “Incentivos”, “Campañas externas”, “Experto en SFT” y “Profesionalidad del farmacéutico”. El facilitador prioritario del estudio, denominado “incentivos”, incluye fundamentalmente incentivos económicos, imprescindibles en la implantación y sostenibilidad del Seguimiento Farmacoterapéutico, aunque también hace referencia a otro tipo de incentivo como el reconocimiento profesional. Los participantes estaban de acuerdo con los resultados del estudio, que indican la necesidad de un pago previo para implantar nuevos servicios profesionales. Se considera que este pago queda justificado por los beneficios que la realización del Seguimiento Farmacoterapéutico aporta a la salud del paciente y a la reducción del gasto sanitario derivado de un buen uso de la medicación. Por consiguiente, se plantearon distintos métodos para incentivar económicamente a la farmacia que realiza Seguimiento Farmacoterapéutico. En cuanto al reconocimiento profesional, se consideró como un incentivo la acreditación de la farmacia que realiza SFT y que su provisión tenga relevancia a nivel curricular. Se confirma también como de gran importancia tanto la realización de campañas externas como la existencia de un experto farmacéutico profesional, preparado y acreditado para realizar nuevos SPF.
Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar
Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27-53 years. In our study 25 patients (32.89%) belonged to the age group of 21-30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA 1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% ( p =3.3x10 -8 ) to 94.6% ( p =2.2x10 -14 ) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA 1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low.
Lee, Ju-Yeun; Min, Ju-Hong; Han, Sueng-Han; Han, Jinu
We describe a 27-year-old pregnant female with new onset of conjugate gaze deficit during the third trimester of pregnancy. Repetitive nerve stimulation tests, neostigmine tests, and acetylcholine receptor antibody assays were all negative. The patient delivered a normal healthy baby at a local clinic via cesarean section. The baby became hypotonic and had respiratory failure several minutes after birth. The result of acetylcholine receptor antibody was negative in the neonate. The neonate became healthy spontaneously and was extubated after 21 days of ventilation care. Two months after delivery, the mother developed ptosis and generalized symptoms and subsequent workup revealed she was muscle specific kinase (MuSK) antibody positive. The neonate was presumed to have an anti-MuSK-mediated transient neonatal myasthenia gravis. Although MuSK antibody testing is rarely indicated in ocular myasthenia gravis, MuSK antibody testing is necessary in pregnant women who are presumed ocular myasthenia gravis to warn occurrence of transient neonatal myasthenia gravis. Copyright © 2017 Elsevier B.V. All rights reserved.
Yoo, Yung Ju; Han, Sang Beom; Yang, Hee Kyung; Hwang, Jeong-Min
Ocular myasthenia gravis is a localized form of myasthenia gravis, which is a postsynaptic disorder of the neuromuscular junction that causes fluctuating weakness of extraocular muscles resulting from autoimmune mechanisms. In women with myasthenia, changes in sex hormone levels and administration of corticosteroids can trigger or worsen symptoms of myasthenia gravis. To describe a case of seronegative ocular myasthenia gravis whose first symptom appeared a day after in vitro fertilization procedure. A 37-year-old woman suddenly developed mild ptosis and fluctuating diplopia that worsened in the evening. Before the development of symptoms, she had undergone in vitro fertilization procedure and had taken oral steroids. Ocular motility examination revealed an intermittent exotropia in primary gaze at both distance and near. The neostigmine test confirmed her diagnosis as ocular myasthenia gravis. When taking a history for young women with sudden onset of binocular diplopia, steroids and sex hormones should be taken into account, which may trigger or exacerbate symptoms of ocular myasthenia gravis.
Riesco Albizu, Miguel; Díaz Fondón, M. Ángeles
El seguimiento continuo del trabajo del alumno suele ser complicado cuando se trata prácticas de desarrollo de software. Además de ser tedioso y de difícil realización, puede ser una tarea considerablemente larga cuando el número de alumnos es elevado. En este trabajo se propone una combinación de técnicas, basadas en la utilización de un sistema de control de versiones, para hacer posible este seguimiento incluso con un número alto de alumnos. Peer Reviewed...
Caballero Biosca, Felipe
La contaminación de suelos y aguas subterráneas es uno de los problemas ambientales más extendidos en gran parte de los terrenos industriales de Cataluña. En este proyecto se ha analizado el proceso de gestión de la contaminación: caracterización, remediación y seguimiento de la descontaminación en suelos y aguas subterráneas por un caso de afección por organoclorados (percloroetileno) y otros contaminantes (hidrocarburos, selenio y cromo) en un emplazamiento industrial situado en una zona ag...
Overvik, E; Nilsson, L; Fredholm, L; Levin, O; Nord, C E; Gustafsson, J A
Lean pork meat was fried with or without the addition of frying-fat at 200 or 250 degrees C. The pan residues were collected by washing the hot pan with boiling water. When producing thickened gravy the water was substituted by a mixture of water and flour, milk and flour or cream and flour. The basic extracts were tested for mutagenicity in Ames' Salmonella test on strain TA98 with the addition of S9 mix. High amounts of mutagenicity were found in all samples. The amounts of mutagenicity in the pan residues were at a comparable level of the amounts found in the meat crusts. Thickening of the gravy caused only small changes in the mutagenicity.
Zeitlhofer, J.; Maida, E.M.; Mamoli, B.; Mayr, N.
In a retrospective study in 47 patients with myasthenia gravis acetylcholine-receptor-antibody-titers (AChR-AB) were correlated with the severity of the disease. In 18 patients the course of titers was studied and two groups of patients could be differentiated: patients with relative constant and patients with fluctuating titers. Age, age of begin of myasthenia and sex did not influence the titers. Also the duration of the disease and the severity of symptoms did not influence the level of AChR-AB-titers. In this retrospective study the influence of immunsuppressive therapy on the intra-individual course of AB-titers and their correlation with the clinical symptoms could not be judged. Measurement of AChR-AB is of value for the diagnosis of myasthenia gravis and important for judging the clinical course and the effect of therapy. (Author)
Myasthenia Gravis (MG) is a disorder affecting components of the neuromuscular junction. Epidemiological studies show rising incidence and prevalence rates. The aim of this study was to determine the incidence and prevalence of MG in the Republic of Ireland. Data sources included patient lists from consultant neurologists and ophthalmologists, a neuroimmunology laboratory, general practitioners and the Myasthenia Gravis Association. A total of 1,715 cases were identified, of which 706 definite, probable or possible autoimmune and congenital MG cases were included. The overall prevalence rate from the data obtained is 15.38\\/100,000. The study demonstrated a female preponderance (female:male of 1.3: 1) and some geographical variation within Ireland. The average incidence rate for the years 2000 to 2009 was 11.3 per year; the rate for the current decade is 18 per year. The increasing number of diagnoses may be due to improved access to diagnostic investigations and increasing awareness of the clinical manifestations.
Kassardjian, Charles D; Murray, Brian J; Kokokyi, Seint; Jewell, Dana; Barnett, Carolina; Bril, Vera; Katzberg, Hans D
The relationship between sleep and neuromuscular fatigue is understood poorly. The goal of this study was to evaluate the effects of napping on quantitative measures of neuromuscular fatigue in patients with myasthenia gravis (MG). Eight patients with mild to moderate MG were recruited. Patients underwent maintenance of wakefulness tests (MWT) and multiple sleep latency tests (MSLT). The Quantitative Myasthenia Gravis Score (QMGS) was measured before nap and after each nap to examine the effects of napping and sleep on neuromuscular weakness. Results showed that QMGS improves only after naps where patients slept more than 5 min but not where patients did not sleep or slept less than 5 min. Daytime napping mitigates neuromuscular fatigue in patients with MG, especially if patients slept for more than 5 min. Copyright © 2013 Wiley Periodicals, Inc.
Fernando Luiz Westphal
Full Text Available A doença de Castleman é um distúrbio linfoproliferativo atípico, de etiologia desconhecida, que pode estar associada a uma série de condições clínicas, inclusive doenças de caráter autoimune e neoplasias malignas. No presente relato, uma paciente de 72 anos foi encaminhada ao serviço de cirurgia torácica do Hospital Universitário Getúlio Vargas, localizado na cidade de Manaus (AM para a ressecção de um tumor de mediastino posterior. Três meses antes, havia sido internada em UTI com um quadro de dispneia intensa, ocasião na qual foi diagnosticada miastenia gravis. Após a ressecção da massa mediastinal, a análise histopatológica revelou doença de Castleman hialino-vascular complicada por sarcoma de células dendríticas foliculares. Até o momento da redação deste estudo, a paciente utilizava um anticolinesterásico e corticoides para o controle da miastenia gravis.Castleman's disease is an atypical lymphoproliferative disorder of unknown etiology, which might be associated with various clinical conditions, including autoimmune diseases and malignant neoplasms. We report the case of a 72-year-old female patient who was referred to the thoracic surgery department of Getúlio Vargas University Hospital, in the city of Manaus, Brazil, for the resection of a posterior mediastinal tumor. Three months prior, the patient had been admitted to the ICU with signs of severe dyspnea, at which time she was diagnosed with myasthenia gravis. After the resection of the mediastinal tumor, the histopathological examination revealed hyaline vascular-type Castleman's disease, complicated by follicular dendritic cell sarcoma. At this writing, the patient was being treated with an anticholinesterase agent and corticosteroids for the control of myasthenia gravis.
Héctor Alfonso Solano Moreno
Full Text Available Las lesiones en la médula espinal representan un porcentaje importante como causa de discapacidad en México y en el mundo, siendo la tercera causa con un porcentaje superior a 25%. Este tipo de pacientes sufre múltiples complicaciones y una de ellas es la que corresponde al sistema urológico al que muchas veces no se le da un seguimiento de manera regular. Dentro de las lesiones de las vías neurológicas, que inervan a la vejiga, pueden afectar a uno o varios aspectos de la fisiología, ya sea su fase de llenado, de almacenamiento o de vaciamiento, lo anterior según el área nerviosa alcanzada y la naturaleza de la lesión; estas lesiones deben ser manejadas por el mismo urólogo que debe seleccionar la opción terapéutica adecuada en su ámbito de competencia, tomando en cuenta los pros y contras de cada una de ellas, ya que esto tendrá una repercusión en la calidad de vida del paciente, así como continuar un programa de seguimiento para la detección de complicaciones de manera oportuna. Particularmente el cateterismo urinario es pilar fundamental para el manejo de las lesiones medulares postraumáticas, ya que puede contribuir a la disminución de las complicaciones.
Weijnen, FG; Bosman, F; van der Glas, HW; Kuks, JBM
Mastication was evaluated in patients with bulbar myasthenia gravis and compared with that of patients with ocular myasthenia gravis, patients in remission who previously suffered from bulbar symptoms, and healthy controls. Bulbar myasthenia gravis may impair mastication due to weakness of the
Guertler, K.F.; Janzen, R.W.C.; Hagemann, J.; Otto, H.F.
Computed tomography of the mediastinum was performed in 45 patients with myasthenia gravis. Surgery was carried out in fourteen. Amongst these, there were four thymomas, one thymolipoma, eight thymic hyperplasias and one normal thymus gland. A further patient, who did not have surgery, probably also had a thymic tumour. The normal thymus and thymic hyperplasia cannot be distinguished on computed tomography. Differentiation of small thymomas from normal thymus is not always possible. Invasion by thymomas can only be appreciated with large tumours.
Myasthenia gravis is an autoimmune neuromuscular disorder. There are several treatment options, including symptomatic treatment (acetylcholinesterase inhibitors), short-term immunosuppression (corticosteroids), long-term immunosuppression (azathioprine, cyclosporine, cyclophosphamide, methotrexate, mycophenolate mofetil, rituximab, tacrolimus), rapid acting short-term immunomodulation (intravenous immunoglobulin, plasma exchange), and long-term immunomodulation (thymectomy). This review explores in detail these different treatment options. Potential future treatments are also discussed. PMID:21845054
Tessitore, Adele; Vita, Maria Letizia; Cusumano, Giacomo; Congedo, Maria Teresa; Filotico, Mariella; Meacci, Elisa; Porziella, Venanzio; Margaritora, Stefano; Granone, Pierluigi
We describe the technique, the benefits and the drawbacks of an original video-assisted thymectomy (VAT), performed through an inframammary cosmetic incision and median sternotomy in myasthenia gravis (MG) patients. This procedure is clinically valuable and cosmetically satisfactory so as to be very well accepted by patients, especially by young women. Minimal-access thymectomy has become increasingly popular as surgical treatment for patients with nonthymomatous myasthenia gravis because of its comparable efficacy, safety, and lesser degree of tissue trauma with conventional open surgery. We report a review/interview of 180 MG patients treated between 1993 and 2005. According to Myasthenia Gravis Foundation of America (MGFA), complete stable remission (CSR) and pharmacologic remission (PR) were calculated at the end of a minimal period of 12 months. A clinical remission was obtained in 41.1% (CR 27.8%, PR 13.3%), who had been followed for at least 12 months from surgery. 95% of these patients judged their cosmetic results to be excellent or good. Thymectomy in MG video-assisted infra-mammary cosmetic incision has shown to be a useful surgical approach as demonstrated by the good functional and very good aesthetic results, associated with a very low morbidity and no mortality.
Full Text Available Vocal cord palsy can have myriad causes. Unilateral vocal cord palsy is common and frequently asymptomatic. Trauma, head, neck and mediastinal tumors as well as cerebrovascular accidents have been implicated in causing unilateral vocal cord palsy. Viral neuronitis accounts for most idiopathic cases. Bilateral vocal cord palsy, on the other hand, is much less common and is a potentially life-threatening condition. Myasthenia gravis, an autoimmune disorder caused by antibodies targeting the post-synaptic acetylcholine receptor, has been infrequently implicated in its causation. We report here a case of bilateral vocal cord palsy developing in a 68-year-old man with no prior history of myasthenia gravis 2 months after he was operated on for diverticulitis of the large intestine. Delay in considering the diagnosis led to endotracheal intubation and prolonged mechanical ventilation with attendant complications. Our case adds to the existing literature implicating myasthenia gravis as an infrequent cause of bilateral vocal cord palsy. Our case is unusual as, in our patient, acute-onset respiratory distress and stridor due to bilateral vocal cord palsy was the first manifestation of a myasthenic syndrome.
Myasthenia gravis, a rare disorder of the neuromuscular transmission, is increasingly acknowledged as a syndrome more than as a single disease. This review summarizes recent advances in pathophysiology which confirm the disease heterogeneity, and may help find disease-targeted and patient-targeted therapies. Antibodies to the acetylcholine receptor, the muscle-specific tyrosine kinase and the lipoprotein receptor protein 4, characterize disease subtypes with distinct clinical traits and immune-pathogenic mechanisms. Genome-wide approaches have identified susceptibility loci within genes that participate in the immune response. Regulatory T and B cells appear to be defective in myasthenia gravis. In patients with acetylcholine receptor antibodies, thymectomy associated with prednisone proved more effective than prednisone alone in a multicenter randomized trial. New therapeutic options target B cells, B-cell growth factors and complement inhibition, and are currently reserved for patients with refractory disease. In the recent past, there has been an active search for new antigens in myasthenia gravis, whereas clinical and experimental studies have provided new insights of crucial pathways in immune regulation, which might become the targets of future therapeutic interventions.
Almog, Yehoshua; Ben-David, Merav; Nemet, Arie Y
Myasthenia gravis may affect any of the six extra-ocular muscles, masquerading as any type of ocular motor pathology. The frequency of involvement of each muscle is not well established in the medical literature. This study was designed to determine whether a specific muscle or combination of muscles tends to be predominantly affected. This retrospective review included 30 patients with a clinical diagnosis of myasthenia gravis who had extra-ocular muscle involvement with diplopia at presentation. The diagnosis was confirmed by at least one of the following tests: Tensilon test, acetylcholine receptor antibodies, thymoma on chest CT scan, or suggestive electromyography. Frequency of involvement of each muscle in this cohort was inferior oblique 19 (63.3%), lateral rectus nine (30%), superior rectus four (13.3%), inferior rectus six (20%), medial rectus four (13.3%), and superior oblique three (10%). The inferior oblique was involved more often than any other muscle (pmyasthenia gravis can be difficult, because the disease may mimic every pupil-sparing pattern of ocular misalignment. In addition diplopia caused by paresis of the inferior oblique muscle is rarely encountered (other than as a part of oculomotor nerve palsy). Hence, when a patient presents with vertical diplopia resulting from an isolated inferior oblique palsy, myasthenic etiology should be highly suspected. Copyright © 2015 Elsevier Ltd. All rights reserved.
Yeh, J-H; Lin, C-M; Chiu, H-C; Bai, C-H
The objective of the study was to examine predictors for sleep-disordered breathing (SDB) in patients with myasthenia gravis (MG) using Watch-PAT. We prospectively studied 58 consecutive patients with MG without respiratory symptoms for a full-night Watch-PAT with concomitant recording of the MG score and acetylcholine receptor antibody concentration and analyzed potential risk factors of SDB. Twenty-four patients (41%) had definitive SDB, which was mild in 12 patients, moderate in six, and severe in six. Assessing risk factors with multivariate models, we found four significant predictors (BMI, age, male gender, and use of azathioprine); BMI was the most powerful predictor. The severity and prevalence of sleep-disordered breathing had no significant association with MG score, myasthenia stage, or seropositivity of acetylcholine receptor antibody. The prevalence of SDB in myasthenic patients with mild and moderate weakness was high when using the Watch-PAT. Both myasthenia-specific factors (use of azathioprine) and general predictors in terms of BMI, age, and male gender predisposed the development of SDB in patients with myasthenia gravis. Careful screening of patients with myasthenia gravis at risk of SDB using Watch-PAT might improve the quality of sleep and cardiovascular health through proper treatment of underlying SDB. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Barnett, Carolina; Bril, Vera; Kapral, Moira; Kulkarni, Abhaya; Davis, Aileen M
Myasthenia gravis is characterized by weakness and fatigability of different muscle groups, including ocular, bulbar and the limbs. Therefore, a measure of disease severity at the impairment level in myasthenia needs to reflect all the relevant impairments, as well as their variations with activity and fatigue. We conducted a qualitative study of patients with myasthenia, to explore their experiences and related impairments, aimed at developing a conceptual framework of disease severity at the impairment level in myasthenia gravis. Twenty patients representing the spectrum of disease participated in semi-structured interviews. Interviews were recorded and the transcripts were analyzed by content analysis using an inductive approach with line-by-line open coding. Themes were generated from these codes. Two main themes were identified: the severity of the impairments and fatigability (i.e., triggering or worsening of an impairment with activity). The impairments were further classified within body regions (ocular, bulbar and axial/limbs). Fatigability was described as a phenomenon affecting the whole body but also affecting specific impairments, and was associated with fluctuation of the symptoms. Patients were concerned that clinical examination at a single point in time might not reflect their true clinical state due to fatigability and fluctuations in severity. This conceptual framework reflects the relevance of both severity and fatigability in understanding impairment-based disease severity in myasthenia. This framework could inform the development of impairment measures in myasthenia gravis.
Full Text Available BACKGROUND: Myasthenia gravis is characterized by weakness and fatigability of different muscle groups, including ocular, bulbar and the limbs. Therefore, a measure of disease severity at the impairment level in myasthenia needs to reflect all the relevant impairments, as well as their variations with activity and fatigue. We conducted a qualitative study of patients with myasthenia, to explore their experiences and related impairments, aimed at developing a conceptual framework of disease severity at the impairment level in myasthenia gravis. METHODS: Twenty patients representing the spectrum of disease participated in semi-structured interviews. Interviews were recorded and the transcripts were analyzed by content analysis using an inductive approach with line-by-line open coding. Themes were generated from these codes. RESULTS: Two main themes were identified: the severity of the impairments and fatigability (i.e., triggering or worsening of an impairment with activity. The impairments were further classified within body regions (ocular, bulbar and axial/limbs. Fatigability was described as a phenomenon affecting the whole body but also affecting specific impairments, and was associated with fluctuation of the symptoms. Patients were concerned that clinical examination at a single point in time might not reflect their true clinical state due to fatigability and fluctuations in severity. CONCLUSIONS: This conceptual framework reflects the relevance of both severity and fatigability in understanding impairment-based disease severity in myasthenia. This framework could inform the development of impairment measures in myasthenia gravis.
Leonardo H. Mendonça Oliveira
Full Text Available 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, pOBJECTIVO: 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
Full Text Available The Dandy-Walker syndrome (DWS is a rare posterior fossa malformation. The DWS can occur associated with other brain or systemic malformations, but ocular abnormalities in this disease are rare and clinical findings mimicking myasthenia gravis have not been described to date. We report a 23-year-old woman who presented mild limitation of the ocular movements with progressive palpebral ptosis, which changed in intensity during the day. The investigation showed negative anti-acetylcholine receptor antibody, repetitive nerve stimulation and "Tensilon test", but the brain magnetic resonance image reveals DWS with hydrocephalus associated with calosal dysgenesis. The characteristic of disease, clinical manifestations and pathologic features, specially the clinical evaluation of ocular abnormalities in suspicion of DWS, including the MG in differential diagnosis are discussed.A síndrome de Dandy-Walker (DWS é uma rara malformação da fossa posterior que pode ocorrer associada com outras malformações cerebrais ou sistêmicas. As alterações oculares são raras e as manifestações clínicas, simulando miastenia gravis (MG, não foram descritas até o momento. Descrevemos uma mulher de 23 anos apresentando discreta limitação da movimentação ocular com progressiva ptose palpebral que mudava de intensidade durante o dia. A investigação mostrou negativos o anticorpo anti-receptor de acetilcolina, a estimulação nervosa repetitiva e o "teste do Tensilon", porém a ressonância magnética de crânio revelou DWS com hidrocefalia associada à disgenesia de corpo caloso. As características da doença, manifestações clínicas e patológicas, especialmente a avaliação clínica de anormalidade ocular na suspeita de DWS serão discutidas, incluindo a MG no diagnóstico diferencial.
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 ...
Boer, H.D. de; Egmond, J. van; Driessen, J.J.; Booij, L.H.D.J.
A neuromuscular blocking drug (NMBD) induced neuromuscular blockade (NMB) in patients with myasthenia gravis usually dissipates either spontaneously or by administration of neostigmine. We administered sugammadex to a patient with myasthenia gravis to reverse a rocuronium-induced profound NMB. NMBDs
Yu, S; Li, F; Chen, B; Lin, J; Yang, M; Fu, X; Li, J; Bu, B
To depict the long-term outcome of patients with myasthenia gravis after thymectomy in combination with immunotherapy, and the factors that may potentially affect the outcome. The 306 patients with myasthenia gravis who underwent extended thymectomy from January 1984 to December 2011 at Tongji Hospital were retrospectively evaluated. The patients consisted of 174 cases with thymoma and 132 cases without thymoma. Pharmaceutical treatment was tailored for each case during follow-up. Nine patients with thymomatous myasthenia gravis died during the perioperative period, and 297 patients were followed for 8.6 years. By their latest visits, 241 patients (81.1%) gained satisfactory efficacy, 24 cases died (8.1%), and 32 cases (10.8%) remained unchanged or deteriorated. Favorable factors for satisfactory efficacy included the presence of ocular myasthenia gravis before operation, no presence of thymoma, and lack of concomitant diseases. It is interesting to mention that, patients with non-thymomatous myasthenia gravis obtained significantly higher rates of complete stable remission and clinical remission than the patients with thymomatous myasthenia gravis. Extended thymectomy combined with immunotherapy is a preferred treatment with a satisfactory long-term remission rate. Patients with non-thymomatous myasthenia gravis have a much more promising prognosis than the patients with thymomatous myasthenia gravis. However, appropriate caution must be taken to discontinue pharmaceutical therapy as relapse remains a major concern after a patient who has already undergone thymectomy becomes symptom-free. © 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
Motobayashi, Mitsuo; Inaba, Yuji; Nishimura, Takafumi; Kobayashi, Norimoto; Nakazawa, Yozo; Koike, Kenichi
Myasthenia gravis is a B cell-mediated autoimmune disorder. The pathophysiology of childhood-onset ocular myasthenia gravis remains unclear. We investigated serum B cell-activating factor levels and other immunological parameters in child patients with ocular myasthenia gravis. Blood samples were obtained from 9 children with ocular myasthenia gravis and 20 age-matched controls. We assayed serum concentrations of B cell-activating factor, anti-acetylcholine receptor antibody titers, 7 types of cytokines (interleukins-2, -4, -6, -10, and -17A; interferon-γ; tumor necrosis factor-α) as well as the percentages of peripheral blood CD4+, CD8+, and CD19+ cells. Serum B cell-activating factor levels were significantly higher before immunosuppressive therapy in patients with childhood-onset ocular myasthenia gravis than in controls and decreased after immunosuppressive therapy. A significant positive correlation was observed between serum B cell-activating factor levels and anti-acetylcholine receptor antibody titers in patients with myasthenia gravis. Serum B cell-activating factor concentrations did not correlate with the percentages of CD4+, CD8+, and CD19+ cells or the CD4+/CD8+ ratio. No significant differences were observed in the levels of the 7 different types of cytokines examined, including interleukin-17A, between preimmunosuppressive therapy myasthenia gravis patients and controls. Circulating B cell-activating factor may play a key role in the pathophysiology of childhood-onset ocular myasthenia gravis. Copyright © 2015 Elsevier Inc. All rights reserved.
Idiaquez, J F; Gonzalez, S; Lasso-Penafiel, J; Barnett, C
Medication adherence is a public health problem and this has not been previously studied in myasthenia gravis patients. To determine if patients with myasthenia gravis are adherent to treatment and to describe the clinical factors of patients who are non-adherent to treatment. Cross-sectional study of patients with myasthenia gravis followed at Padre Hurtado Hospital, Santiago de Chile, who received their medication through the hospital and therefore were on the pharmacy's list. Patients' participation was voluntary and anonymous. Medication adherence was assessed with the Morisky-Green-Levine survey (4 items). Patients were assessed for myasthenia gravis severity with the Manual Muscle Test, and myasthenia gravis-related quality of life with the MG-QOL15. Finally, patients were screened for depression with the 12-Item General Health Questionnaire. 26 patients were enrolled and 15 (57.7%) were women. Only 10 (38.5%) of patients were adherent to treatment. Patients who were not adherent to medication had more weakness (p = 0.06), worse quality of life (p = 0.008), were taking a greater number of myasthenia gravis drugs (p = 0.003) and had a higher risk of depression (p = 0.03). In this cohort of myasthenia gravis patients, three out of five patients were not adherent to treatment. These patients tended to have more weakness, worse quality of life and higher risk of depression. Medication adherence should be assessed routinely in patients with myasthenia gravis.
Wirtz, P. W.; Sotodeh, M.; Nijnuis, M.; van Doorn, P. A.; van Engelen, B. G. M.; Hintzen, R. Q.; de Kort, P. L. M.; Kuks, J. B.; Twijnstra, A.; de Visser, M.; Visser, L. H.; Wokke, J. H.; Wintzen, A. R.; Verschuuren, J. J.
Background: Myasthenia gravis and the Lambert-Eaton myasthenic syndrome (LEMS) may have a similar distribution of muscle weakness. Deciding on a diagnosis of myasthenia gravis or LEMS on clinical grounds may therefore be difficult. Objective: To compare the localisation of initial muscle weakness
Niks, Erik H.; Kuks, Jan B. M.; Verschuuren, Jan J. G. M.
The epidemiology of myasthenia gravis subtypes and the frequency of antibodies to muscle-specific kinase (MuSK) was studied in patients with generalised myasthenia gravis without anti-acetylcholine receptor antibodies who had an onset of symptoms between 1990 and 2004 in a well-defined region in the
Erkan Turan, Kadriye; Kocabeyoglu, Sibel; Bekircan-Kurt, Can Ebru; Bezci, Figen; Erdem-Ozdamar, Sevim; Irkec, Murat
To evaluate ocular surface alterations and characteristics of corneal basal epithelium and subbasal nerves in patients with myasthenia gravis. Myasthenia gravis patients (n = 21) and healthy controls (n = 20) were enrolled. All participants underwent ocular surface testing in the following order: tear break-up time, lissamine green staining, Schirmer I test with anesthesia, and Ocular Surface Disease Index questionnaire. The Cochet-Bonnet esthesiometer was used to measure corneal sensitivity. Basal epithelial cells and subbasal nerves were evaluated using in vivo confocal microscopy. Myasthenia gravis patients had higher Ocular Surface Disease Index score (13.9 ± 15.0 vs 1.4 ± 2.2, p myasthenia gravis had lower basal epithelial cell density (3775.7 ± 938.1 vs 4983.1 ± 608.5, p myasthenia gravis and the number of corneal nerves (rho = -0.497, p = 0.022). Significant alterations of basal epithelial cells and subbasal nerves were demonstrated in myasthenia gravis patients although there was no difference of corneal sensitivity between myasthenia gravis patients and healthy controls. Thus, it should be borne in mind that myasthenia gravis patients deserve further evaluation with regard to ocular surface disease.
O'Sullivan, S S
We describe a patient with the diagnoses of acquired neuromyotonia, cutaneous lupus erythematosus and alopecia areata, occurring many years after a thymectomy for myasthenia gravis associated with a thymoma. We review the current literature on autoimmune conditions associated with myasthenia gravis and thymectomy. To our knowledge, this combination of multiple autoimmune conditions has not been reported previously.
Melvin Ramírez Bogantes
Full Text Available En el siguiente trabajo se presentan los principales resultados de investigación obtenidos en la fase de diseño de un algoritmo que permite la detección y el seguimiento de un objeto presente en una grabación de video. El algoritmo se diseñó e implementó en el ambiente de programación MatLab y los videos utilizados fueron facilitados por el Centro de Investigación Apícola Tropical (CINAT y corresponden a la presencia del ácaro Varroa Destructor en las celdas de abejas melíferas africanizadas. El principal resultado que se tiene es la creación de un programa capaz de detectar y registrar el movimiento del ácaro, lo cual es algo innovador y útil para los estudios del comportamiento de esta especie en las celdas de las abejas melíferas que realiza el CINAT.
Igor I. Bonnet, MD
Full Text Available Se presenta un caso clínico poco frecuente de feocromocitoma bilateral, en el cual los estudios de diagnóstico por imagen, tanto de información estructural como funcional, constituyeron una fuente fundamental para su detección y seguimiento.
Vanessa Regiane Resqueti
Full Text Available Este estudo objetivou determinar a confiabilidade do teste da caminhada de seis minutos (TC6M como um teste de capacidade funcional em pacientes com miastenia gravis generalizada (MG. Foram selecionados 11 pacientes com MG - 5 homens, 6 mulheres - com idade de 55±9 anos, avaliados inicialmente quanto à função fulmonar, que se submeteram a três TC6M em dias diferentes. Durante e/ou após cada teste foram medidas freqüência cardíaca e saturação de oxigênio (por oxímetro portátil, sensação de dispnéia (pela escala de Borg e distância percorrida. Nos três testes as distâncias percorridas foram 498 m, 517 m e 520 m (respectivamente 99%, 103% e 104% do valor predito. Em média, a freqüência cardíaca, dispnéia e saturação de oxigênio mostraram comportamento constante nos três testes. Foram encontradas alta confiabilidade relativa, com coeficiente de correlação interclasse maior que 0,90 entre os testes (TC6M1-TC6M2, 0,960; TC6M1-TC6M3, 0,945; e TC6M2-TC6M3, 0,970 e confiabilidade absoluta de 4%, 3,5% e 4,8%, com reprodutibilidade de 11%, 9,8% e 13,4%, respectivamente para o primeiro, segundo e terceiro testes. Os limites superiores e inferiores de concordância e o valor médio das médias das diferenças (bias calculados pelo teste de Bland-Altman mostraram-se clinicamente aceitáveis. Conclui-se que o TC6M se mostrou seguro, confiável e reprodutível, podendo ser aplicado para avaliação e seguimento da tolerância ao exercício em pacientes com MG generalizada.The purpose of this study was to assess the reliability of the six minutes walking test (6MWT as a functional capacity test for patients with generalized myasthenia gravis (MG. Eleven patients with generalized MG (5 men, six women, aged 55±9 years, were first assessed as to pulmonary function and then submitted to three 6MWT in different days. Heart rate and oxygen saturation were measured (by means of portable oxymeter during, and dyspnea (by the Borg scale and
Cobián Rodríguez MB
Introducción: El seguimiento farmacoterapéutico es un servicio que atiende las necesidades de los enfermos crónicos polimedicados pero invierte muchos recursos en un solo paciente. El objetivo del estudio fue conocer las variaciones en la dispensación de medicamentos derivadas del seguimiento farmacoterapéutico y determinar los recursos invertidos en el funcionamiento y sus costes asociados. Métodos: Se realizó el servicio de seguimiento farmacoterapéutico durante seis meses de una muestr...
Soyoral, Lokman; Goktas, Ugur; Cegin, Muhammed Bilal; Baydi, Volkan
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. Copyright © 2014 Sociedade Brasileira de Anestesiologia. Published by Elsevier Editora Ltda. All rights reserved.
Rivner, Michael H; Pasnoor, Mamatha; Dimachkie, Mazen M; Barohn, Richard J; Mei, Lin
Around 20% of patients with myasthenia gravis are acetylcholine receptor antibody negative; muscle-specific tyrosine kinase antibodies (MuSK) were identified as the cause of myasthenia gravis in 30% to 40% of these cases. Anti MuSK myasthenia gravis is associated with specific clinical phenotypes. One is a bulbar form with fewer ocular symptoms. Others show an isolated head drop or symptoms indistinguishable from acetylcholine receptor-positive myasthenia gravis. These patients usually respond well to immunosuppressive therapy, but not as well to cholinesterase inhibitors. Other antibodies associated with myasthenia gravis, including low-density lipoprotein receptor-related protein 4, are discussed. Copyright © 2018 Elsevier Inc. All rights reserved.
Javier Salazar Zárate; Blanca Hidalgo Ponce; Narcisa Salazar Alvarez; Byron Vaca Barahona
Esta investigación desarrolla y propone un método para la ejecución y se-guimiento de los proyectos de fin de curso de las materias de lenguajes de programación en carreras de Ingeniería Electrónica soportado tecnológica-mente por GitHub. El objetivo es obtener un método que permita mejorar la calidad de los proyectos. Para la validación del nuevo método se realiza un contraste entre dos grupos de estudiantes, el primer grupo utilizando el mé-todo tradicional de ejecución de proyectos y el se...
Varó Galvañ, Pedro José; Gómez Bernabeu, Ana; Suriá Martínez, Raquel; González-Pacanowski, Antonio; Rosser Limiñana, Ana; Ortuño, Manuel; Benavídez, Paula Gabriela; Brocal Fernández, Francisco; Blanco Bartolomé, Lucía; Ros Gilabert, José Manuel; Pacheco Mateo, María Rosario; García González, Encarnación; Sentana Gadea, Irene; Poveda Pérez, Jose Luís; Prado Govea, Raúl Hugo
El profesorado de la red participó durante el curso 2016/17 en un proyecto de coordinación y seguimiento de la titulación Máster Universitario en Prevención de Riesgos Laborales. En el curso 2016-17 la titulación Máster en Prevención de Riesgos Laborales desarrolla la quinta edición desde su implantación en el curso 2012-13. En el curso 2015-16 la titulación recibió informe favorable de renovación de acreditación emitido por la Agencia para la evaluación, acreditación y prospectiva del sistem...
Danay Hernández Díaz
Full Text Available Introducción: El seguimiento sistemático y personalizado de la ganancia de peso gestacional puede constituir una herramienta útil a nivel primario de salud para el binomio madre-hijo para su salud presente y futura. Método: Estudio observacional analítico para determinar si existen diferencias en la evolución ponderal en dos grupos según sistematicidad en intervención sobre estilos de vida y cultura alimentaria en 256 embarazadas del policlínico Chiqui Gómez en Santa Clara, Cuba desde octubre 2012 a septiembre 2014; las mismas fueron vistas en los tres trimestres de la gestación (190 o sólo en el primero y en el tercer trimestre (66, las que conformaron los dos grupos analizados de acuerdo por la sistematicidad en la asistencia a los encuentros presenciales (consultas. Se calcularon y compararon las ganancias de peso según grupo de pertenencia y estado nutricional pregestacional. Se usaron métodos de estadística descriptiva y de comparación de grupos por estado nutricional. Resultados: Las gestantes con mayor asistencia a las consultas mostraron durante el segundo trimestre un mayor ajuste a las ganancias de peso recomendadas, lo cual no pudo comprobarse estadísticamente en el tercer trimestre; no obstante la cuantía de ganancia de peso en las obesas y sobrepeso, en este trimestre, fue menor que en aquellas que no asistieron regularmente a las consultas. Conclusiones: La sistematicidad y personalización de acciones ejecutadas en embarazadas, se asoció a mejor adherencia a la ganancia de peso recomendadas en el segundo trimestre; así como con una disminución de la cuantía de ganancia ponderal, en aquellas pacientes con sobrepeso y obesidad durante el tercer trimestre.
Elkin Veslin Diaz
Full Text Available Este documento propone una aplicación con Differential Flatness para el problema de seguimiento de trayectorias en manipuladores robóticos. Para cada coordenada generalizada, se proponen sus trayectorias como una función en el tiempo donde deben encontrar las entradas correspondientes para garantizar el seguimiento. Se demuestra que la posición de cada coordenada generalizada del manipulador robótico y sus correspondientes derivadas son salidas planas que, en conjunto con un controlador PD pueden determinar, con algunas restricciones, los valores de fuerza para conseguir un movimiento en el manipulador con una mínima desviación a lo largo del trayecto, tanto en movimientos planos como en el espacio.
Renton, Alan E.; Pliner, Hannah A.; Provenzano, Carlo; Evoli, Amelia; Ricciardi, Roberta; Nalls, Michael A.; Marangi, Giuseppe; Abramzon, Yevgeniya; Arepalli, Sampath; Chong, Sean; Hernandez, Dena G.; Johnson, Janel O.; Bartoccioni, Emanuela; Scuderi, Flavia; Maestri, Michelangelo; Raphael Gibbs, J.; Errichiello, Edoardo; Chiò, Adriano; Restagno, Gabriella; Sabatelli, Mario; Macek, Mark; Scholz, Sonja W.; Corse, Andrea; Chaudhry, Vinay; Benatar, Michael; Barohn, Richard J.; McVey, April; Pasnoor, Mamatha; Dimachkie, Mazen M.; Rowin, Julie; Kissel, John; Freimer, Miriam; Kaminski, Henry J.; Sanders, Donald B.; Lipscomb, Bernadette; Massey, Janice M.; Chopra, Manisha; Howard, James F.; Koopman, Wilma J.; Nicolle, Michael W.; Pascuzzi, Robert M.; Pestronk, Alan; Wulf, Charlie; Florence, Julaine; Blackmore, Derrick; Soloway, Aimee; Siddiqi, Zaeem; Muppidi, Srikanth; Wolfe, Gil; Richman, David; Mezei, Michelle M.; Jiwa, Theresa; Oger, Joel; Drachman, Daniel B.; Traynor, Bryan J.
IMPORTANCE Myasthenia gravis is a chronic, autoimmune, neuromuscular disease characterized by fluctuating weakness of voluntary muscle groups. Although genetic factors are known to play a role in this neuroimmunological condition, the genetic etiology underlying myasthenia gravis is not well understood. OBJECTIVE To identify genetic variants that alter susceptibility to myasthenia gravis, we performed a genome-wide association study. DESIGN, SETTING, AND PARTICIPANTS DNA was obtained from 1032 white individuals from North America diagnosed as having acetylcholine receptor antibody–positive myasthenia gravis and 1998 race/ethnicity-matched control individuals from January 2010 to January 2011. These samples were genotyped on Illumina OmniExpress single-nucleotide polymorphism arrays. An independent cohort of 423 Italian cases and 467 Italian control individuals were used for replication. MAIN OUTCOMES AND MEASURES We calculated P values for association between 8114394 genotyped and imputed variants across the genome and risk for developing myasthenia gravis using logistic regression modeling. A threshold P value of 5.0 × 10−8 was set for genome-wide significance after Bonferroni correction for multiple testing. RESULTS In the over all case-control cohort, we identified association signals at CTLA4 (rs231770; P = 3.98 × 10−8; odds ratio, 1.37; 95% CI, 1.25–1.49), HLA-DQA1 (rs9271871; P = 1.08 × 10−8; odds ratio, 2.31; 95% CI, 2.02 – 2.60), and TNFRSF11A (rs4263037; P = 1.60 × 10−9; odds ratio, 1.41; 95% CI, 1.29–1.53). These findings replicated for CTLA4 and HLA-DQA1 in an independent cohort of Italian cases and control individuals. Further analysis revealed distinct, but overlapping, disease-associated loci for early- and late-onset forms of myasthenia gravis. In the late-onset cases, we identified 2 association peaks: one was located in TNFRSF11A (rs4263037; P = 1.32 × 10−12; odds ratio, 1.56; 95% CI, 1.44–1.68) and the other was detected
Full Text Available ObjectivesTo describe the clinical profile, clinical outcomes and factors that may affect the outcome of juvenile myasthenia gravis (JMG patients in southern China.MethodsWe reviewed information relating to JMG patients treated and evaluated at the First Affiliated Hospital, Sun Yat-sen University, between 1998 and 2015. The study involved 327 JMG patients who had been followed up for ≥1 year.ResultsOverall, 77.4% patients showed initial symptoms in the prepubertal period (<12 years. 306 patients showed only ocular symptoms at onset. By the final follow-up, 61 ocular myasthenia gravis (OMG patients (61/306, 19.9% had developed generalized myasthenia gravis (GMG. Anti-acetylcholine receptor antibodies (AChR-Ab titer was an independent risk factor for generalization. Eleven patients (3.4% experienced spontaneous remission, but four relapsed. Low-dose oral prednisone (0.25 mg/kg was administered when symptoms did not significantly improve after pyridostigmine treatment. Immunosuppressants were administered when prednisone was unsatisfactory. Optimal outcome was achieved in 59.6% of patients. Specifically, 60 patients (18.3% attained complete stable remission (CSR, 12 (3.7% attained pharmaceutical remission (PR, and 123 (37.6% attained minimal manifestation (MM. In total, 53 OMG patients (21.5% attained CSR, a significantly higher proportion than among the GMG patients (8.6%, P = 0.009. Moreover, 67.2% of patients with duration <2 years showed significant clinical improvement compared with 46.3% of those with duration >2 years (P < 0.001. Thymectomy did not exhibit definite efficacy for JMG patients.ConclusionThere was a low frequency of cases positive for AChR-Ab in the Chinese population. AChR-Ab titer was revealed as an independent risk factor for generalization. Low doses of prednisone could treat JMG effectively with few side effects.
Gil García MI; Benrimoj SI; Martínez-Martínez F; Cardero Rivas M; Gastelurrutia Garralda MA
En el contexto de un estudio de investigación sobre priorización de facilitadores para la implantación del Seguimiento Farmacoterapéutico en las farmacias comunitarias españolas, se realizó un grupo focal compuesto por miembros de Foro de Atención Farmacéutica en Farmacia Comunitaria. El objetivo era explorar sus opiniones sobre los facilitadores priorizados en dicho estudio que se habían definido como “Incentivos”, “Campañas externas”, “Experto en SFT” y “Profesionalidad del farmacéutico”. E...
Ojeda Zújar, José; Sánchez Rodríguez, Esperanza; Vallejo Villalta, Ismael; Fernández-Palacios Carmona, Arturo; Moreira Madueño, José Manuel
En el marco del Programa de seguimiento de la dinámica y calidad de las aguas litorales en Andalucía desarrollado por la Consejería de Medio Ambiente de la Junta de Andalucía se engloban una serie de experiencias a través de las que se pretenden testear las posibilidades de cuantificación de parámetros de calidad del agua utilizando datos de sensores remotos y tomas de muestras sobre el agua. Esta comunicación presenta una serie de reflexiones acerca de la posibilidad de cuantificar los parám...
Liu, Chanchan; Gui, Mengcui; Cao, Yayun; Lin, Jing; Li, Yue; Ji, Suqiong; Bu, Bitao
Myasthenia gravis tends to affect children in China. Oral pyridostigmine and prednisone could effectively improve the symptoms, but multiple side effects become a major concern after long-term oral prednisone. To avoid the long-term complications of prednisone therapy and to obtain more satisfactory improvement, we tested the efficacy and safety of tacrolimus in children with myasthenia gravis. Children with myasthenia gravis who had not achieved satisfactory improvement or who experienced severe side effects after prednisone therapy were recruited between January 2015 and December 2016 at Tongji Hospital. All the children were treated with tacrolimus 1 mg to 2 mg daily and the dose was adjusted on the basis of the clinical response and the serum concentration. The dosage of prednisone, the severity of symptoms, blood samples, the serum concentration of tacrolimus, and titers of antiacetylcholine receptor antibodies were evaluated every four weeks. Fourteen children were enrolled. One child withdrew two weeks after the enrollment. Thirteen children have completed the therapy for one year. At the end point, the dosage of prednisone was significantly decreased (P myasthenia gravis score, and myasthenia gravis-specific manual muscle testing and myasthenia gravis-activities of daily living scores were significantly improved (P myasthenia gravis. Randomized clinical trials are needed to confirm the observation. Copyright © 2017 Elsevier Inc. All rights reserved.
Brown, L.R.; Muhm, J.R.; Sheedy, P.F. II; Unni, K.K.; Bernatz, P.E.; Hermann, R.C. Jr.
In a 5 year study, 19 patients with myasthenia gravis were studied by computed tomography (CT) and underwent thymectomy. CT was accurate in detecting the nine true thymic masses but could not differentiate thymomas from nonthymomatous masses, including thymic cysts. No thymoma was found in a patient under 25 years of age. In one case, the 18 sec scanner could not differentiate a large gland from a thymoma. In eight cases, glands with histologic thymic hyperplasia and histologically normal thymus appeared to be similar and could not be differentiated by CT
Guertler, K.F.; Janzen, R.W.C.; Hagemann, J.; Otto, H.F.; Hamburg Univ.; Hamburg Univ.
Computed tomography of the mediastinum was performed in 45 patients with myasthenia gravis. Surgery was carried out in fourteen. Amongst these, there were four thymomas, one thymolipoma, eight thymic hyperplasias and one normal thymus gland. A further patient, who did not have surgery, probably also had a thymic tumour. The normal thymus and thymic hyperplasia cannot be distinguished on computed tomography. Differentiation of small thymomas from normal thymus is not always possible. Invasion by thymomas can only be appreciated with large tumours. (orig.) [de
Thorvinger, B.; Lyttkens, K.; Samuelsson, L.
Sixteen consecutive patients with myasthenia gravis were examined by computed tomography (CT) before thymectomy. Surgical and histologic findings were compared with those obtained at CT. The results, like those of most other recent studies, indicate that CT is a reliable method for identifying thymoma. In all three patients with thymoma, and in five out of eight patients with hyperplasia, the lesion was observed at CT, giving an overall accuracy of 80%. Although tymic hyperplasia is a basically histological diagnosis an enlarged gland may give a correct diagnosis of this abnormality. (orig.)
Thorvinger, B.; Lyttkens, K.; Samuelsson, L.
Sixteen consecutive patients with myasthenia gravis were examined by computed tomography (CT) before thymectomy. Surgical and histologic findings were compared with those obtained at CT. The results, like those of most other recent studies, indicate that CT is a reliable method for identifying thymoma. In all three patients with thymoma, and in five out of eight patients with hyperplasia, the lesion was observed at CT, giving an overall accuracy of 80%. Although tymic hyperplasia is a basically histological diagnosis an enlarged gland may give a correct diagnosis of this abnormality.
Brown, L.R.; Muhm, J.R.; Sheedy, P.F. II; Unni, K.K.; Bernatz, P.E.; Hermann, R.C. Jr.
In a 5 year study, 19 patients with myasthenia gravis were studied by computed tomography (CT) and underwent thymectomy. CT was accurate in detecting the nine true thymic masses but could not differentiate thymomas from nonthymomatous masses, including thymic cysts. No thymoma was found in a patient under 25 years of age. In one case, the 18 sec scanner could not differentiate a large gland from a thymoma. In eight cases, glands with histologic thymic hyperplasia and histologically normal thymus appeared to be similar and could not be differentiated by CT.
Nacu, Aliona; Andersen, Jintana Bunpan; Lisnic, Vitalie; Owe, Jone Furlund; Gilhus, Nils Erik
Abstract Myasthenia gravis (MG) is a rare autoimmune disease of skeletal muscle endplates. MG subgroup is relevant for comorbidity, but usually not accounted for. MG patients have an increased risk for complicating autoimmune diseases, most commonly autoimmune thyroid disease, systemic lupus erythematosus and rheumatoid arthritis. In this review, we present concomitant autoimmune disorders associated with the different MG subgroups, and show how this influences treatment and prognosis. Concomitant MG should always be considered in patients with an autoimmune disorder and developing new neuromuscular weakness, fatigue or respiratory failure. When a second autoimmune disorder is suspected, MG should be included as a differential diagnosis. PMID:25915571
Full Text Available IntroducciónLa disección aguda de la aorta tipo A (DAA-A es una emergencia que requiere cirugía inmediata, debido al mal pronóstico de su evolución natural. A pesar del avance en las técnicas quirúrgicas, el procedimiento aún tiene una morbimortalidad elevada.ObjetivosAnalizar la morbimortalidad hospitalaria y la sobrevida alejada de una serie consecutiva de pacientes operados por DAA-A.Material y métodosSe incluyeron 63 pacientes consecutivos (el 71,4% eran hombres en cuatro centros asistenciales de Buenos Aires desde julio de 1994 a mayo de 2007. El seguimiento se realizó en el 89% de los pacientes. La edad promedio fue de 63 ± 11,3 años. En 15 pacientes, el reemplazo se extendió hasta el hemiarco y en 5 se reemplazó el arco completo. La válvula aórtica se reemplazó en 12 pacientes.ResultadosDurante la estadía hospitalaria fallecieron 19 pacientes (30,1%: un caso durante la cirugía, 7 por complicaciones isquémicas o falla multiorgánica, 3 por complicaciones neurológicas, 5 por complicaciones cardíacas, 1 por hemorragia digestiva y 2 pacientes a consecuencia de múltiples complicaciones. Durante el seguimiento fallecieron 12 pacientes (32,4%: 8 casos de causa cardiovascular y 4 de causa no cardíaca. El análisis multivariado detectó que las variables asociadas con mayor mortalidad hospitalaria fueron el bajo volumen minuto y el tiempo de circulación extracorpórea (CEC prolongado, en tanto que las asociadas con mayor mortalidad alejada fueron la edad > 70 años y un tiempo menor de CEC. La sobrevida a 1, 3, 5 y 10 años fue del 89%, 79,5%, 73% y 58%, respectivamente.ConclusionesLos resultados del tratamiento quirúrgico de la DAA-A en nuestro medio pueden asimilarse a los obtenidos en series internacionales, lo que a su vez confirma la elevada morbimortalidad hospitalaria y alejada de esta entidad.REV ARGENT CARDIOL 2009;77:108-115.
Basaran-Akgul, Nese; Rasco, Barbara A.
Dielectric properties (the dielectric constant (ε′) and the dielectric loss factor (ε″)) and the penetration depth of raw eye of round beef Semitendinosus muscle, raw beef marinated in gravy, raw beef cooked in gravy, and gravy alone were determined as a function of the temperature (20–130 °C) and frequency (27–1,800 MHz). Both ε′ and ε″ values increased as the temperature increased at low frequencies (27 and 40 MHz). At high frequencies (915 and 1,800 MHz), ε′ showed a 50 % decrease while ε″...
Rahbek, Martin Amadeus; Mikkelsen, Erik Elgaard; Overgaard, Kristian
Introduction: It has not been established whether progressive resistance training (PRT) and aerobic training (AT) are feasible and efficient in myasthenia gravis (MG). Methods: Fifteen subjects with generalized MG (Myasthenia Gravis Foundation of America (MGFA) clinical classification II-IV) were...... randomly assigned to 20 training sessions during 8 weeks of either PRT or AT. Feasibility was evaluated based on adherence, drop-out rate, adverse events, and Quantitative Myasthenia Gravis (QMG) score. Results: Twelve subjects (MGFA II, n = 11; MGFA III, n=1) completed the intervention with a mean...
Full Text Available Muscle-specific tyrosine kinase- (MuSK- antibodies-positive Myasthenia Gravis accounts for about one third of Seronegative Myasthenia Gravis and is clinically characterized by early onset of prominent bulbar, neck, shoulder girdle, and respiratory weakness. The response to medical therapy is generally poor. Here we report a case of late-onset MuSK-antibodies-positive Myasthenia Gravis presenting with signs of cognitive impairment and parkinsonism in addition to bulbar involvement and external ophthalmoplegia. The pattern of involvement of both peripheral and central nervous system dysfunction might suggest a common pathogenic mechanism, involving impaired cholinergic transmission.
Full Text Available Abstract Background Myasthenia gravis, an uncommon autoimmune syndrome, is commonly associated with thymus abnormalities. Thymomatous myasthenia gravis is considered to have worst prognosis and thymectomy can reverse symptoms if precociously performed. Case report We describe a case of a patient who underwent mitral valve repair and was found to have an occasional thymomatous mass during the surgery. A total thymectomy was performed concomitantly to the mitral valve repair. Conclusion The diagnosis of thymomatous myasthenia gravis was confirmed postoperatively. Following the surgery this patient was strictly monitored and at 1-year follow-up a complete stable remission had been successfully achieved.
Cristian Bravo Roman
Full Text Available La gran mayoría de los proyectos de minería de datos que utilizan la metodología KDD en la vida real entregan solamente soluciones estáticas, que con el paso del tiempo pierden la capacidad de explicar los fenómenos para los que fueron construidos inicialmente. Presen-tamos un marco teórico-práctico que permite realizar un seguimiento cercano a los modelos para determinar el momento donde éstos deben ser actualizados, manteniendo un estricto control sobre la evolución de los mismos, las variables presentes en ellos y los cambios re-levantes que pueden ocurrir en la población desde que fueron inicialmente diseñados. Los tests estadísticos incluyen tests clásicos como las pruebas de Kolmogorov-Smirnov o la prue-ba de Chi-Cuadrado para medir los cambios en las medias de las variables en los modelos, más un test novedoso diseñado en base a la distribución de los coeficientes en los modelos y la desviación estándar observada de las variables, que permite medir cuándo la población ha cambiado más allá de los intervalos de confianza definidos por los parámetros iniciales. La metodología fue puesta a prueba utilizando las bases de datos reales de dos proyectos de Credit Scoring a microempresarios, realizados entre los años 2007 y 2008 con muy buenos resultados. Most data mining projects in real life applications give as a result only static solutions which, in time, lose their inherent capacity to explain the phenomena they were originally built for. We introduce an theoretical-practical framework that allows to closely follow up logistic regression models to determine the moment when they must be updated, maintaining an strict control over their evolution, the variables in them and relevant changes that can occur in the popula-tion since they were originally designed. The statistical test presented include classical tests such as Kolmogorov-Smirnov and Chi-Squared statistic to measure changes in means of the variables present
NRE102/2: Resoluci??n del Rectorado de la Universidad de Granada por la que se aprueba la programaci??n del Plan de Control Interno, auditorias y otras actividades a desarrollar por la Oficina de Control Interno durante el ejercicio 2016.
Universidad de Granada
Resoluci??n de la Rectora de la Universidad de Granada, de 28 de enero de 2016, por la que se aprueba la programaci??n del Plan de Control Interno, auditorias y otras actividades a desarrollar por la Oficina de Control Interno durante el ejercicio 2016. Se incluye la Memoria de Actividades y un seguimiento del Plan Anueal de Control Interno.
Ana Laura Colle Kauling
Full Text Available JUSTIFICATIVA E OBJETIVOS: A Miastenia gravis (MG é uma doença neurológica autoimune que afeta a porção pós-sináptica da junção neuromuscular. Trata-se de um desafio ao anestesiologista, pela diversidade das manifestações da doença e pela possibilidade de complicações ventilatórias no pós-operatório. O objetivo deste trabalho é demonstrar a importância da monitoração adequada ao bloqueio neuromuscular (BNM em virtude das múltiplas formas de apresentação da MG. CONTEÚDO: Neste artigo serão descritos dois casos de pacientes com MG - um que apresentou a forma clássica de sensibilidade ao bloqueador neuromuscular (BNM e outro com resposta semelhante à de um paciente normal. A revisão da literatura será restrita às características da doença e a descrição de sua fisiopatologia estará voltada às reações aos BNM. CONCLUSÕES: Como conclusão, sugere-se que, em decorrência das múltiplas formas de apresentação e de tratamento da MG, é fundamental o uso de monitores da transmissão neuromuscular quando se usa BNM.
Marcos C. Sandmann
Full Text Available Entre 1982 e 1988 24 mulheres e 6 homens com miastenia gravis foram submetidos a protocolo diagnóstico e terapêutico prospectivo e estandardizado. A idade variou de 10 a 74 anos (34±16, média±desvio padrão. Três com forma ocular foram tratados com piridostigmina. Quatro com forma generalizada, de idade avançada, receberam corticosteróide e/ou azatioprina. Vinte e três com a forma generalizada foram submetidos a timectomia seguida de prednisona e/ou azatioprina. Um paciente faleceu após timectomia complexa de timoma invasivo. Qutro faleceu logo após a admissão, em crise miastênica/colinérgica. Dois outros tiveram complicações menores da timectomia. De 19 pacientes seguidos por 1-60 (média 24 meses, 11 (58% têm fraqueza residual mas levam vida normal e 8 (42% estão em remissão completa, um sem medicação.
Full Text Available Renato Mantegazza, Silvia Bonanno, Giorgia Camera, Carlo AntozziDepartment of Neuromuscular Diseases and Neuroimmunology, Fondazione Istituto Neurologico Carlo Besta, Milan, ItalyAbstract: Myasthenia gravis (MG is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future.Keywords: myasthenia gravis, therapy, immunosuppression, thymectomy, plasmapheresis
Pestronk, A.; Drachman, D.B.; Teoh, R.; Adams, R.N.
A therapeutic strategy was designed to eliminate the humoral immune response to acetylcholine receptor (AChR) in ongoing experimental autoimmune myasthenia gravis (EAMG). Rats with EAMG were treated with a protocol consisting of three components: (1) A single high dose of cyclophosphamide (200 mg/kg) was used to produce a rapid and sustained fall in the anti-AChR antibody levels by preferential destruction of antibody-producing B-lymphocytes. ''Memory'' lymphocytes were not eliminated by cyclophosphamide. (2) Irradiation (600 rads) was used to eliminate the ''memory'' cells. It eliminated the anamnestic response to a challenge with the antigen AChR. (3) Bone marrow transplantation was used to repopulate the hematopoietic system after the otherwise lethal dose of cyclophosphamide. We used bone marrow from syngeneic rats with active EAMG to simulate an autologous transplant. Rats with EAMG treated with this combined protocol showed a prompt and sustained fall in the anti-AChR antibody levels and had no anamnestic response to a challenge with AChR. Thus, an affected animal's own marrow could be stored and used later for repopulation after cyclophosphamide-irradiation treatment. This treatment eliminates the animal's ongoing immune responses and reconstitutes the immune system in its original state. The success of this approach suggests that, if their safety could be established, similar ''curative'' strategies might be developed for the treatment of patients with severe antibody-mediated autoimmune disorders, such as myasthenia gravis
de Silva, S.; Blum, J.E.; McIntosh, K.R.; Order, S.; Drachman, D.B.
Total lymphoid irradiation (TLI) has been reported to be effective in the immunosuppressive treatment of certain human and experimental autoimmune disorders. We have investigated the effects of TLI in Lewis rats with experimental autoimmune myasthenia gravis (EAMG) produced by immunization with purified torpedo acetylcholine receptor (AChR). The radiation is given in 17 divided fractions of 200 rad each, and nonlymphoid tissues are protected by lead shielding. This technique suppresses the immune system, while minimizing side effects, and permits the repopulation of the immune system by the patient's own bone marrow cells. Our results show that TLI treatment completely prevented the primary antibody response to immunization with torpedo AChR, it rapidly abolished the ongoing antibody response in established EAMG, and it suppressed the secondary (anamnestic) response to a boost of AChR. No EAMG animals died during TLI treatment, compared with six control animals that died of EAMG. TLI produces powerful and prompt immunosuppression and may eventually prove useful in the treatment of refractory human myasthenia gravis
Leon-Sarmiento, Fidias E; Leon-Ariza, Juan S; Prada, Diddier; Leon-Ariza, Daniel S; Rizzo-Sierra, Carlos V
Myasthenia gravis is a paradigmatic muscle disorder characterized by abnormal fatigue and muscle weakness that worsens with activities and improves with rest. Clinical and research studies done on nicotinic acetylcholine receptors have advanced our knowledge of the muscle involvement in myasthenia. Current views still state that sensory deficits are not "features of myasthenia gravis". This article discusses the gap that exists on sensory neural transmission in myasthenia that has remained after >300years of research in this neurological disorder. We outline the neurobiological characteristics of sensory and motor synapses, reinterpret the nanocholinergic commonalities that exist in both sensory and motor pathways, discuss the clinical findings on altered sensory pathways in myasthenia, and propose a novel way to score anomalies resulting from multineuronal inability associated sensory troubles due to eugenic nanocholinergic instability and autoimmunity. This medicine-based evidence could serve as a template to further identify novel targets for studying new medications that may offer a better therapeutic benefit in both sensory and motor dysfunction for patients. Importantly, this review may help to re-orient current practices in myasthenia. Copyright © 2016 Elsevier B.V. All rights reserved.
de Silva, S.; Blum, J.E.; McIntosh, K.R.; Order, S.; Drachman, D.B.
Total lymphoid irradiation (TLI) has been reported to be effective in the immunosuppressive treatment of certain human and experimental autoimmune disorders. We have investigated the effects of TLI in Lewis rats with experimental autoimmune myasthenia gravis (EAMG) produced by immunization with purified torpedo acetylcholine receptor (AChR). The radiation is given in 17 divided fractions of 200 rad each, and nonlymphoid tissues are protected by lead shielding. This technique suppresses the immune system, while minimizing side effects, and permits the repopulation of the immune system by the patient's own bone marrow cells. Our results show that TLI treatment completely prevented the primary antibody response to immunization with torpedo AChR, it rapidly abolished the ongoing antibody response in established EAMG, and it suppressed the secondary (anamnestic) response to a boost of AChR. No EAMG animals died during TLI treatment, compared with six control animals that died of EAMG. TLI produces powerful and prompt immunosuppression and may eventually prove useful in the treatment of refractory human myasthenia gravis.
Ralli, Massimo; Altissimi, Giancarlo; Di Stadio, A; Mazzei, Filippo; Turchetta, Rosaria; Cianfrone, Giancarlo
There is increasing evidence of a connection between hearing function and myasthenia gravis (MG). Studies of the pathophysiological basis of this relationship suggest that acetylcholine receptors (AChRs) on outer hair cells (OHCs) play a central role. In patients with MG, autoantibodies against AChRs induce a progressive loss of AChRs on OHCs, decreasing their electromotility. The stapedial reflex decay test can be altered in MG patients, and can be used as an additional tool for diagnosis and monitoring. Transient evoked and distortion product otoacoustic emissions are the main diagnostic tool for monitoring OHC functionality in MG patients, and can be used to record subclinical hearing alterations before the onset of clinically evident hearing loss. Understanding the association between MG and hearing dysfunction requires a multidisciplinary approach. Otolaryngologists should take this relationship into account when approaching patients with a diagnosis of myasthenia gravis and "in patients with MG" with ण128;in MG patients, and the progress of hearing alterations should always be monitored in patients with MG.
Paola María Miranda Morales
Full Text Available Resumen La investigación realizada por la Escuela Latinoamericana de Cooperación y Desarrollo de la Universidad de San Buenaventura, en asocio con el Instituto de Estudios Ambientales de la Universidad Nacional de Colombia, titulada “Impacto de la Cooperación Internacional en la Gestión Ambiental: el caso colombiano” (Miranda, et al., 2010, arrojó unos resultados desalentadores en cuanto al cumplimiento de los compromisos establecidos en la Declaración de París (2005 en relación con la Ayuda Oficial al Desarrollo (AOD canalizada por Colombia en el período comprendido entre los años 1993 y 2008. El análisis realizado al proceso de financiación de la gestión ambiental con recursos de Cooperación Internacional para el Desarrollo (CID; que abarcó los recursos, las instituciones públicas del Sistema Nacional Ambiental (SINA, los donantes y al Gobierno Nacional como líder del proceso; permitió verificar el incumplimiento de una serie de criterios definidos a partir de los cinco principios básicos de la eficacia de la AOD establecidos por la Comunidad Internacional, y evidenció que de las tres etapas del proceso: planeación, implementación y control y seguimiento, siendo esta última la más crítica de todas.AbstractThe present paper presents and approach un the debate regardong the relevance of control and tracking of resources and the results obtained on the effectiveness of International Development Cooperation (IDC. We discuss the public and private sectors, as well as civil organizations and donors. Using an international view point and the current Colombian Legislation, our analysis allows us to identify and define the latent risks of inadequate management of resources of IDC in Colombia. In particular, we point out some weakness of financial, legislative and technical nature. With the analysis presented we are able to propose several improvement in financing process involving the IDC.
Carlos A Romero Díaz
Full Text Available Los divertículos faringoesofágicos o de Zenker, son los más frecuentes de los divertículos por pulsión, constituidos por la protución de la mucosa de la faringe a través de un defecto de la pared posterior de ella, entre los músculos constrictor inferior de la faringe y el cricofaríngeo. Se realizó un estudio de 14 pacientes intervenidos quirúrgicamente por esta afección con seguimiento entre 2 y 14 años, divididos en 2 series, una de 5 pacientes a los cuales se les realizó diverticulopexia a uno y a los otros diverticulectomía, y otra serie de 9 pacientes en los que se efectuó diverticulectomía con cricomiotomía. La halitosis (100 %, disfagia (85,7 % y regurgitación (57,1 % fueron los síntomas que mayormente se hallaron. Se corroboró el diagnóstico en todos los casos con un estudio baritado de esófago, estómago y duodeno. Los resultados a largo plazo fueron altamente satisfactorios en 11 pacientes, regulares en 2 y malos (recidiva en 2. El tratamiento de elección fue la diverticulectomía con cricomiotomíaThe pharyngo-oesophageal or Zenker´s diverticula are the most frequent pulsion diverticula produced by a protrusion of the mucous membrane of the pharynx through a defect of its posterior wall between the inferior constrictor musclessof the pharynx and the cricopharyngoeal muscle. A study of 14 patients who underwent surgery due to this affection and were followed-up from 2 to 14 years was conducted. They were divided into 2 groups: one of 5 patients among whom one underwent diverticulopexy and the diverticulectomy with cricomyotomy. Halitosis (100 %, dysphagia (85.7 % and regurgitation (57.1 % were the most commonly found symptoms. The diagnosis was confirmed in every case through a barium study of the esophagus, the stomach and the duodenum. The results on the long term were highly satisfactory in ll patients, fair in 2 and bad (relapse in 2. The election treatment was diverticulectomy with cricomyotomy
Kisabay, Aysin; Sari, Ummu Serpil; Boyaci, Recep; Batum, Melike; Yilmaz, Hikmet; Selcuki, Deniz
Myasthenia gravis is a disease of neuromuscular junction due to auto-immune destruction of the acetylcholine receptors. Behçet's disease, on the other hand, is a multisystemic vascular-inflammatory disease. Both conditions are not common in the general population although their association has not been reported in the literature. We wanted to present our patient who developed clinical course of myasthenia gravis following discontinuation of medications due to complications of corticosteroid for Behçet's disease. It was observed that clinical findings of myasthenia gravis recovered following restarting steroid treatment and he did not experience attacks of both conditions. Although Myasthenia gravis and Behçet's disease are distinct entities clinically as well as in terms of pathogenesis, they share common physiopathological features and their treatment is based on their common features. Copyright © 2016 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.
Miarzynska, M.; Szlezak, L.; Fibak, J.; Wolski, M.; Lis-Podrzycka, E.; Miarzynski, K. [Szpital im. F. Raszei, Poznan (Poland)
The rare case of malignant tumor of thymus - Thymoma malignum was described. The initial diagnosis was difficult, because of the irregular symptoms of myasthenia gravis. The diagnostic difficulties, treatment and clinical features of this neoplasm were also discussed. (author)
Alam, Md. Shahid
Full Text Available Myasthenia gravis is an autoimmune disorder affecting the neuromuscular junction. Ocular myasthenia gravis presents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy or congenital or aponeurotic ptosis. Juvenile ocular myasthenia gravis in very young children is difficult to diagnose and can be easily labeled as a case of congenital ptosis, the more so when the condition is bilateral. We present a case of a two-year-old child who presented with bilateral ptosis and was diagnosed as a case of simple congenital ptosis elsewhere with the advice to undergo tarsofrontalis sling surgery. The child was diagnosed with juvenile myasthenia gravis on thorough history, examination, and systemic evaluation and was started on anti-myasthenic treatment.
Francisca Santisteban Aguilera
Full Text Available Se presenta el caso de un paciente atendido en el Hospital Provincial “Saturnino Lora” y el Centro de Retinosis Pigmentaria de Santiago de Cuba con características audiológicas, propias de una hipoacusia neurosensorial moderada, afecciones oftalmológicas, cansancio generalizado, disfagia, ptosis palpebral bilateral asimétrica con predominio en el lado izquierdo, afectación del oblicuo, del recto interno y ausencia del reflejo nauseoso, además de ideas delirantes y alucinaciones. Las alteraciones clínicas presentes en este enfermo hacen sospechar la asociación de un síndrome de Usher, con miastenia gravis y una esquizofrenia.The case of a patient attended at "Saturnino Lora" Provincial Hospital and at the Pigmentary Retinosis Center in Santiago de Cuba with audiology characteristics, moderate neurosensorial hypoacusia, ophthalmologic affections, generalized fatigue, dysphagia, asymmetric bilateral palpebral ptosis with prevalence in the left side, affection of the oblique muscle and of the internal rectum muscle and absence of the gag reflex, besides delusion and hallucinations is presented. The clinical changes in this patient make the suspect of an association of the Usher`s syndrome, with miastenia gravis and a schizophrenia.
Marleny Valencia Arredondo
Full Text Available En Colombia el cáncer cérvico-uterino (Cacu es una de las principales causas de muerte. Anualmente se diagnostican 7.000 casos y se registran 3.300 muertes, el 50% por diagnóstico tardío y falta de acceso a tratamientos especializados. Objetivo: describir el seguimiento a mujeres con resultado citológico positivo en una ESE de Medellín, 2011-2012. Metodología: estudio descriptivo transversal en 277 mujeres con resultado citológico positivo para lesiones sugestivas de alto grado y/o alteraciones glandulares. Se empleó muestreo estratificado y sistemático. Se utilizaron fuentes de información primaria y secundaria. La sistematización de datos se realizó en el programa SPSS, y se calcularon medidas de frecuencia, tendencia central y dispersión. Se empleó análisis descriptivo uni-bivariado para variables sociodemográficas, clínicas y relacionadas con la atención recibida. Resultados: el 10,7% de mujeres con lesiones de alto grado o invasoras no culminaron el tratamiento. La oportunidad total del seguimiento fue del 52,3% y las fases con mayores tiempos de espera para la atención fueron las citas para diagnóstico y tratamiento. Conclusión: A pesar de los esfuerzos para mejorar la cobertura del cribado y búsqueda activa de pacientes con citología positiva evidenciada en el estudio aún se presentan obstáculos en la atención de pacientes para un diagnóstico y tratamiento oportuno, lo que lleva en varios casos al avance de la enfermedad e incluso la muerte.
Fujimoto, Masafumi; Terasaki, Shuhei; Nishi, Masaaki; Yamamoto, Tatsuo
Several previous studies using univariate analysis have suggested that the pre-anaesthetic train-of-four (TOF) ratio, concentration of anti-acetylcholine receptor (AChR) antibodies and the presence of preoperative generalised muscular involvement are determinants of an increased response to neuromuscular blocking agents (NMBAs) in patients with myasthenia gravis. However, the determinants of the response of patients with myasthenia gravis to rocuronium, which is expected to be used more frequently since the advent of sugammadex, have not been studied. To clarify whether previously suggested determinants of the response to other intermediate-acting NMBAs would also affect the response to rocuronium and to reveal the determinants of the increased response to rocuronium in individual patients with myasthenia gravis using multivariate analysis. Case control study. Kumamoto University Hospital, November 2010 to September 2013. Thirty-eight patients with myasthenia gravis having surgery using a total intravenous anaesthetic technique were investigated. After induction of general anaesthesia, the 95% effective dose (ED95) of rocuronium was calculated using cumulative dose-finding methods. Neuromuscular function was monitored by acceleromyographic assessment of TOF responses of the adductor pollicis muscle to ulnar nerve stimulation. Patients were then divided into the increased response (ED95 rocuronium injection (baseline TOF ratio), concentration of anti-AChR antibodies, Osserman classification and treatment for myasthenia gravis in the two groups were compared. Stepwise logistic regression identified baseline TOF ratio and age of onset of myasthenia gravis as determinants of the increased response to rocuronium in patients with myasthenia gravis [odds ratios (95% confidence interval) of 0.87 (0.77 to 0.98; P = 0.02) and 0.92 (0.86 to 0.99; P = 0.03), respectively]. Multivariate analysis identified baseline TOF ratio and age of disease onset as determinants of
Suárez Vélez, María Fernanda
El propósito del proyecto es desarrollar una aplicación basada en el sistema operativo Android que ofrezca ayuda en el seguimiento de delfines en el mar, mediante el cálculo de la distancia donde aparece el animal. Esto se consigue haciendo uso de la Realidad Aumentada y con el empleo del acelerómetro, la brújula, la cámara y el GPS de la tablet, junto con su altura sobre la superficie del mar y el ángulo de inclinación del dispositivo, que es usado para calcular la distancia ...
Pardo Sánchez, Abelardo
Proyecto de Innovación Docente de la asignatura de Arquitectura de Ordenadores En el presente documento se detalla una forma uso de las tecnologías de la información y la comunicación (en adelante TIC) para incrementar el seguimiento de las sesiones experimentales en la impartición de la asignatura de Arquitectura de Ordenadores de primer curso, segundo cuatrimestre de la titulación de Ingeniería Técnica de Telecomunicación, Especialidad Telemática. Mediante un sistema automático de proces...
Toro Galván, Jaime
Estas líneas sirven para resumir lo que en páginas posteriores será el desarrollo completo del proyecto final de carrera. Como indica el título “Seguimiento de obra de un edificio industrial entre medianeras en Barcelona” el objetivo es reflejar los procesos de ejecución, control de costes, planificación y control de calidad, así como los diferentes procesos necesarios para el control de una obra. En una primera parte, veremos de forma general la metodología de control y seguimien...
Qi, Guoyan; Liu, Peng; Gu, Shanshan; Yang, Hongxia; Dong, Huimin; Xue, Yinping
Our study is aimed to explore the underlying genetic basis of myasthenia gravis. We collected a Chinese pedigree with myasthenia gravis, and whole-exome sequencing was performed on the two affected siblings and their parents. The candidate pathogenic gene was identified by bioinformatics filtering, which was further verified by Sanger sequencing. The homozygous mutation c.G40A (p.V14M) in interferon gamma receptor 1was identified. Moreover, the mutation was also detected in 3 cases of 44 sporadic myasthenia gravis patients. The p.V14M substitution in interferon gamma receptor 1 may affect the signal peptide function and the translocation on cell membrane, which could disrupt the binding of the ligand of interferon gamma and antibody production, contributing to myasthenia gravis susceptibility. We discovered that a rare variant c.G40A in interferon gamma receptor 1 potentially contributes to the myasthenia gravis pathogenesis. Further functional studies are needed to confirm the effect of the interferon gamma receptor 1 on the myasthenia gravis phenotype.
Sund, K.K.; Skeie, G.O.; Gilhus, N.E.; Aarli, J.A.; Varhaug, J.E. [Haukeland Sykehus, Bergen (Norway)
The authors have compared clinical, immunological and radiological data in 20 patients with myasthenia gravis and thymoma and in 21 patients with myasthenia gravis and thymic atrophy. The median age at onset was 54 years in the thymoma group and 63 years in the thymic atrophy group. The severity of the disease was similar in the two groups, and there was no significant difference in the concentration of acetylcholine receptor antibodies. CA antibodies were demonstrated in 17/20 thymoma patients and in 6/21 with thymic atrophy, while 19/20 thymoma patients had antibodies to titin, compared with 9/21 among those with thymic atrophy. The diagnosis and treatment of patients with myasthenia gravis is based upon an evaluation of clinical, immunological and radiological data. 28 refs., 2 tabs.
Tajima, Shogo; Yanagiya, Masahiro; Sato, Masaaki; Nakajima, Jun; Fukayama, Masashi
Among human neoplasms, thymomas are well known for their association with paraneoplastic autoimmune diseases such as myasthenia gravis. However, regarding rare metaplastic thymoma, only one case of an association with myasthenia gravis has been reported. Here, we present the second case of a 44-year-old woman with metaplastic thymoma associated with myasthenia gravis. In metaplastic thymoma, intratumoral terminal deoxynucleotidyl transferase-positive T-cells (immature T-cells) are generally scarce, while they were abundant in the present case. We believe that these immature T-cells could be related to the occurrence of myasthenia gravis.
Full Text Available Myasthaenia gravis (MG is an autoimmune neuromuscular disorder which is twice as common among women, often presenting in the second and third decades of life. Typically, the first trimester of pregnancy and first month postpartum are considered high-risk periods for MG exacerbations. During pregnancy, treatment for MG is usually individualised, thus improving its management. Plasma exchange and immunoglobulin therapies can be safely used to treat severe manifestations of the disease or myasthaenic crises. However, thymectomies are not recommended because of the delayed beneficial effects and possible risks associated with the surgery. Assisted vaginal delivery—either vacuum-assisted or with forceps—may be required during labour, although a Caesarean section under epidural anaesthesia should be reserved only for standard obstetric indications. Myasthaenic women should not be discouraged from attempting to conceive, provided that they seek comprehensive counselling and ensure that the disease is under good control before the start of the pregnancy.
Sangüesa Gómez, Clara; Flores Robles, Bryan Josué; Méndez Perles, Clara; Barbadillo, Carmen; Godoy, Hildegarda; Andréu, José Luis
The association of dermatomyositis with myasthenia gravis (MG) is uncommon, having been reported so far in only 26 cases. We report the case of a 69 year-old man diagnosed with MG two years ago and currently treated with piridostigmyne. The patient developed acute proximal weakness, shoulder pain and elevated creatine-kinase (CK). He also developed generalized facial erythema and Gottron's papules. Laboratory tests showed positive antinuclear and anti-Mi2 antibodies. Further analysis confirmed CK levels above 1000 U/l. The clinical management of the patient and the therapeutic implications derived from the coexistence of both entities are discusssed. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.
Jonsson, Dagur Ingi; Pirskanen, Ritva; Piehl, Fredrik
Muscle fatigue associated with myasthenia gravis is caused by autoantibodies interfering with neuromuscular transmission. Immunomodulating treatment is widely used in moderate to severe myasthenia, although the use of newer biological drugs except rituximab is rare. We describe the effect of tocilizumab, a blocker of interleukin-6 signalling, in two female myasthenia patients with high titres of serum acetylcholine receptor antibodies and insufficient response to rituximab. The first patient had been treated with high dose immunoglobulins regularly for several years and the second patient had been treated both with different oral immune suppressants and immunoglobulins before testing a low dose of rituximab without significant clinical effect. Subsequent treatment with tocilizumab resulted in clinical improvement within a few months. The first patient was switched back to rituximab, which resulted in worsening until tocilizumab was restarted. Tocilizumab can be a therapeutic option in cases not responding to rituximab. Copyright © 2017 Elsevier B.V. All rights reserved.
Mantegazza, Renato; Cordiglieri, Chiara; Consonni, Alessandra; Baggi, Fulvio
Myasthenia gravis (MG) is a chronic autoimmune disease caused by the immune attack of the neuromuscular junction. Antibodies directed against the acetylcholine receptor (AChR) induce receptor degradation, complement cascade activation, and postsynaptic membrane destruction, resulting in functional reduction in AChR availability. Besides anti-AChR antibodies, other autoantibodies are known to play pathogenic roles in MG. The experimental autoimmune MG (EAMG) models have been of great help over the years in understanding the pathophysiological role of specific autoantibodies and T helper lymphocytes and in suggesting new therapies for prevention and modulation of the ongoing disease. EAMG can be induced in mice and rats of susceptible strains that show clinical symptoms mimicking the human disease. EAMG models are helpful for studying both the muscle and the immune compartments to evaluate new treatment perspectives. In this review, we concentrate on recent findings on EAMG models, focusing on their utility and limitations. PMID:27019601
Adam J. Lake
Full Text Available Myasthenia gravis (MG is a rare autoimmune disease that leads to progressive muscle weakness and is common during female reproductive years. The myasthenic mother and her newborn must be observed carefully, as complications during all stages of pregnancy and the puerperium may arise suddenly. Preeclampsia is a common obstetrical condition for which magnesium sulfate is used for seizure prophylaxis. However, magnesium sulfate is strongly contraindicated in MG as it impairs already slowed nerve-muscle connections. Similarly, many first-line antihypertensive medications, including calcium channels blockers and β-blockers, may lead to MG exacerbation. This case describes the effective obstetrical management of a patient with MG who developed severe preeclampsia. The effective use of levetiracetam and various antihypertensive medications including intravenous labetalol is described. A review of the ten reported cases of MG complicated by preeclampsia is examined to aggregate observations of clinical care, with focus on delivery methods, anticonvulsants, and antihypertensive medications.
The development of validated assessment tools for evaluating disease status and response to interventions in patients with myasthenia gravis (MG) has been driven by clinical studies of emerging MG therapies. However, only a small proportion of MG-focused neurology practices have adopted these assessment tools for routine clinical use. This article reviews the suitability of 5 assessment instruments for incorporation into clinical practice, which should be driven by their ability to contribute to improved patient outcomes, and to be implemented within practice personnel and resource constraints. It is recommended that assessments based on both physician-evaluated and patient-reported outcomes be selected, to adequately evaluate both point-in-time symptom load and functional impact of MG symptoms over time. Provider resource allocation and reimbursement issues may be the most significant roadblocks to successful ongoing use of these tools; to that end, the addition of regular assessments to MG standards of care is recommended.
Luo, Jie; Lindstrom, Jon
Myasthenia gravis (MG) is an organ-specific autoimmune disease characterized by muscle fatigability. In most cases, it is mediated by autoantibodies targeting muscle nicotinic acetylcholine receptors (AChRs) at the neuromuscular junction. Experimental autoimmune myasthenia gravis (EAMG) is an animal model for MG, which is usually induced by immunization with AChR purified from fish electric organ. Pathological autoantibodies to AChRs are directed at the extracellular surface, especially the main immunogenic region (MIR). Current treatments for MG can help many but not all patients. Antigen-specific immunosuppressive therapy for MG that specifically suppresses the autoimmune response without affecting the entire immune system and avoids side effects of general immunosuppression is currently unavailable. Early attempts at antigen-specific immunosuppression for EAMG using AChR extracellular domain sequences that form epitopes for pathological autoantibodies risked provoking autoimmunity rather than suppressing it. We discovered a novel approach to specific immunosuppression of EAMG with a therapeutic vaccine consisting of bacterially-expressed human AChR cytoplasmic domains, which has the potential to specifically suppress MG without danger of causing exacerbation. This approach prevents development of chronic EAMG when initiated immediately after the acute phase of EAMG, and rapidly reverses established chronic EAMG when started during the chronic phase of EAMG. Successfully treated rats exhibited long-term resistance to re-induction of EAMG. In this review we also discuss the current understanding of the mechanisms by which the therapy works. Vaccination with AChR cytoplasmic domains in adjuvant is promising as a safe, antigen-specific, potent, effective, rapidly acting, and long lasting approach to therapy of MG. Copyright © 2015 Elsevier Inc. All rights reserved.
Pestronk, A.; Drachman, D.B.; Teoh, R.; Adams, R.N.
A therapeutic strategy was designed to eliminate the humoral immune response to acetylcholine receptor (AChR) in ongoing experimental autoimmune myasthenia gravis (EAMG). Rats with EAMG were treated with a protocol consisting of three components: (1) A single high dose of cyclophosphamide (200 mg/kg) was used to produce a rapid and sustained fall in the anti-AChR antibody levels by preferential destruction of antibody-producing B-lymphocytes. ''Memory'' lymphocytes were not eliminated by cyclophosphamide. (2) Irradiation (600 rads) was used to eliminate the ''memory'' cells. It eliminated the anamnestic response to a challenge with the antigen AChR. (3) Bone marrow transplantation was used to repopulate the hematopoietic system after the otherwise lethal dose of cyclophosphamide. We used bone marrow from syngeneic rats with active EAMG to simulate an autologous transplant. Rats with EAMG treated with this combined protocol showed a prompt and sustained fall in the anti-AChR antibody levels and had no anamnestic response to a challenge with AChR. Thus, an affected animal's own marrow could be stored and used later for repopulation after cyclophosphamide-irradiation treatment. This treatment eliminates the animal's ongoing immune responses and reconstitutes the immune system in its original state. The success of this approach suggests that, if their safety could be established, similar ''curative'' strategies might be developed for the treatment of patients with severe antibody-mediated autoimmune disorders, such as myasthenia gravis.
Estudio retrospectivo de la aplicación de la amplificación génica cualitativa en muestras biológicas para el seguimiento de la toxoplasmosis en pacientes pediátricos receptores de trasplante de células progenitoras hematopoyéticas
Mónica G Nigro
Full Text Available La toxoplasmosis es una infección oportunista causada por el parásito Toxoplasma gondii; su infección es grave y de difícil diagnóstico en pacientes que reciben un trasplante alogénico de células progenitoras hematopoyéticas (TCPH. En el Hospital de Pediatría S.A.M.I.C. "Profesor Dr. Juan P. Garrahan"� se realizó la vigilancia postrasplante de 12 pacientes receptores de TCPH mediante la técnica de PCR cualitativa. La necesidad de seguimiento de estos pacientes fue definida por el antecedente de serología positiva para toxoplasmosis en el donante o receptor y ante la imposibilidad de iniciar el uso profiláctico de trimetoprima-sulfametoxazol a causa de la condición hematológica. Dos pacientes presentaron signos de enfermedad por T. gondii con resultado de PCR positivo y recibieron tratamiento con pirimetamina-clindamicina. En otros dos, la toxoplasmosis fue causa de muerte y hallazgo de autopsia, con resultado de PCR negativo. Cuatro pacientes recibieron tratamiento contra toxoplasmosis por la detección de una PCR positiva, sin manifestaciones clínicas. En los cuatro pacientes restantes no se detectaron signos de enfermedad por toxoplasmosis, con resultados de PCR negativos durante el seguimiento. La técnica de PCR cualitativa demostró ser útil para detectar la reactivación de la toxoplasmosis en receptores de TCPH, pero tiene limitaciones para el seguimiento y la toma de decisiones clínicas en pacientes con PCR positiva que persiste en el tiempo y manifestaciones de toxicidad por el tratamiento.
Full Text Available Resumen: Este documento muestra en detalle el proceso de simulación y evaluación multiobjetivo de las propuestas al Concurso en Ingeniería de Control 2012, Control autónomo del seguimiento de trayectorias de un vehículo cuatrirrotor, organizado por el Comité Español de Automática (CEA. El concurso busca ofrecer una herramienta de trabajo adicional que pueda servir para reforzar la educación en control y orientar a los alumnos de ingeniería sobre el tipo de problemas que un ingeniero de control debe enfrentar. Copyright c 2012 CEA. Publicado por Elsevier España, S.L. Todos los derechos reservados. Abstract: In this document, the simulation and multi-objective evaluation of the competition on Control Enginering 2012, Autonomous trajectory control of a quadricopter vehicle is shown and explained. This competition is organized by the Comité Español de Automática (CEA. This benchmark is presented as an educational platform to reinforce control education as well as to show the participants the kind of problems that a control engineer could face. Palabras clave: Educación en control, Control de helicopteros, Identificación de procesos, Diseño de sistemas de control, Decisión multiobjetivo, Keywords: Control education, Helicopter control, Control system design, Process identification, Multiobjective decision
Gavilla González, Bárbara; Díaz Cabrera, Leticia
En el siguiente trabajo se realizó el seguimiento de un niño desde la etapa de recién nacido hasta el año de edad por malnutrición por defecto, de causa materna por psicosis puerperal. En este caso, la madre rechazaba a su hijo, negándose a alimentarlo, privando al bebé de algo tan importante en esta etapa de la vida como la lactancia materna exclusiva, requiriendo de alimentación complementaria en edad temprana para lograr un aumento de peso adecuado y un desarrollo psicomotor que se igualar...
Gavilla González, Bárbara; Díaz Cabrera, Leticia
En el siguiente trabajo se realizó el seguimiento de un niño desde la etapa de recién nacido hasta el año de edad, por malnutrición por defecto, de causa materna por psicosis puerperal. En este caso, la madre rechazaba a su hijo, negándose a alimentarlo, privando al bebé de algo tan importante en esta etapa de la vida como la lactancia materna exclusiva, requiriendo de alimentación complementaria en edad temprana para lograr un aumento de peso adecuado y un desarrollo psicomotor que se iguala...
Bagheri, Reza; Boonstani, Reza; Sadrizadeh, Ali; Salehi, Maryam; Afghani, Reza; Rahnama, Ali; Azmounfar, Vahab; Hakimian, Saeed; Baradaran Firoozabadi, Mohammad
Thymectomy considered as a standard procedure in treatment of all the steps of myasthenia gravis. Video-assisted thoracoscopic surgery (VATS) thymectomy is one of the minimally invasive procedures that because of the short duration of hospitalization, less postoperative pain, and scar after surgery, nowadays it is replaced the traditional methods of surgery for patients with myasthenia gravis, but there are still differences and concerns. The aim of this study was to compare outcomes of two different techniques of surgery, VATS versus transsternal (TS) in the treatment of myasthenia gravis. In this pilot study, 42 patients with myasthenia gravis and without a thymus tumor were evaluated based on Myasthenia Gravis Foundation of America classification and drug consumption. Then, they randomly underwent two different techniques of surgery: VATS versus TS. Patients were evaluated based on preoperative and postoperative variables. Duration of intensive care unit stay and hospitalization has been reduced in patients who underwent VATS thymectomy operation technique. In addition, duration of surgical procedure has been reduced significantly in these patients. These patients have less blood loss during surgery compared with TS group. The Myasthenia Gravis Foundation of America postoperative status in VATS thymectomy revealed that the number of patients with complete stable remission was higher and number of persons remained unchanged was lower in this group. VATS thymectomy is a safe and appropriate approach comparing with traditional methods such as TS thymectomy for patients with myasthenia gravis. This method has better results after surgery and can be used as a minimally invasive alternative method instead of TS thymectomy.
Almeida, Carlos; Coutinho, Ester; Moreira, Daniela; Santos, Ernestina; Aguiar, José
Myasthenia gravis is an autoimmune neuromuscular disease, usually affecting women in the second and third decades. The course is unpredictable during pregnancy and puerperium. Myasthenia gravis can cause major interference in labour and partum and exacerbations of the disease frequently occur. The aim of this series of cases is to analyse retrospectively the anaesthetic management of myasthenia gravis patients and complications during the peripartum period. Retrospective, single centre study from clinical files of female myasthenia gravis patients who delivered between 1985 and 2007 at Hospital de Santo António, Porto, Portugal. Seventeen myasthenia gravis patients delivered between 1985 and 2007 in Hospital Santo António. Two women were not included in the study as they had a spontaneous abortion in the first trimester. Four patients presented exacerbations of the disease during pregnancy, no exacerbation occurred in eight patients and three patients presented their first symptoms of myasthenia gravis during pregnancy (without diagnosis at time of delivery). Concerning the eight patients without exacerbations of the disease during pregnancy, pregnancy was brought to term in 87.5% of the cases; five women were submitted to nonurgent caesarean section (62.5%); and epidural block was performed in six patients (75%). No complications related to anaesthesia occurred in the peripartum period. Concerning the four patients with exacerbations of the disease, pregnancy was brought to term in three cases (75%); three women were submitted to nonurgent caesarean section (75%); and epidural block was performed in three patients (75%). One patient underwent an uncomplicated thymectomy under general anaesthesia during pregnancy and, in the postpartum period, there was a myasthenic crisis in another patient. Concerning the three patients without a myasthenia gravis diagnosis at partum, one woman already being followed for presenting muscular weakness had a vaginal delivery under
Du, Ailian; Huang, Shiqian; Zhao, Xiaonan; Feng, Kuan; Zhang, Shuangyan; Huang, Jiefang; Miao, Xiang; Baggi, Fulvio; Ostrom, Rennolds S; Zhang, Yanyun; Chen, Xiangjun; Xu, Congfeng
Myasthenia gravis is an autoimmune disorder of the neuromuscular junction manifested as fatigable muscle weakness, which is typically caused by pathogenic autoantibodies against postsynaptic CHRN/AChR (cholinergic receptor nicotinic) in the endplate of skeletal muscle. Our previous studies have identified CA3 (carbonic anhydrase 3) as a specific protein insufficient in skeletal muscle from myasthenia gravis patients. In this study, we investigated the underlying mechanism of how CA3 insufficiency might contribute to myasthenia gravis. Using an experimental autoimmune myasthenia gravis animal model and the skeletal muscle cell C2C12, we find that inhibition of CAR3 (the mouse homolog of CA3) promotes CHRN internalization via a lipid raft-mediated pathway, leading to accelerated degradation of postsynaptic CHRN. Activation of CAR3 reduces CHRN degradation by suppressing receptor endocytosis. CAR3 exerts this effect by suppressing chaperone-assisted selective autophagy via interaction with BAG3 (BCL2-associated athanogene 3) and by dampening endoplasmic reticulum stress. Collectively, our study illustrates that skeletal muscle cell CAR3 is critical for CHRN homeostasis in the neuromuscular junction, and its deficiency leads to accelerated degradation of CHRN and development of myasthenia gravis, potentially revealing a novel therapeutic approach for this disorder.
Yang, D H; Su, Z Q; Chen, Y; Chen, Z B; Ding, Z N; Weng, Y Y; Li, J; Li, X; Tong, Q L; Han, Y X; Zhang, X
To assess the predictive value of the albumin to globulin ratio (AGR) in evaluation of disease severity and prognosis in myasthenia gravis patients. A total of 135 myasthenia gravis (MG) patients were enrolled between February 2009 and March 2015. The AGR was detected on the first day of hospitalization and ranked from lowest to highest, and the patients were divided into three equal tertiles according to the AGR values, which were T1 (AGR 1.53). The Kaplan-Meier curve was used to evaluate the prognostic value of AGR. Cox model analysis was used to evaluate the relevant factors. Multivariate Logistic regression analysis was used to find the predictors of myasthenia crisis during hospitalization. The median length of hospital stay for each tertile was: for the T1 21 days (15-35.5), T2 18 days (14-27.5), and T3 16 days (12-22.5) (Pmyasthenia gravis. At the multivariate Cox regression analysis, the AGR (Pmyasthenia gravis patients. Respectively, the hazard ratio (HR) were 4.655 (95% CI: 2.355-9.202) and 0.596 (95% CI: 0.492-0.723). Multivariate Logistic regression analysis showed the AGR (Pmyasthenia crisis. The AGR may represent a simple, potentially useful predictive biomarker for evaluating the disease severity and prognosis of patients with myasthenia gravis.
Conclusiones: La reparación valvular mitral se ha mostrado como una técnica segura y eficaz. Un equipo multidisciplinario permite la correcta identificación de pacientes candidatos y la aplicación de las técnicas quirúrgicas adecuadas. A la espera de un mayor seguimiento, los resultados son satisfactorios.
Esteban Chapapría, Vicent; Aguilar, José; Serra Peris, José; Medina, Josep R.
Se describen los métodos de ejecución de trabajos topográficos y batimétricos de seguimiento de playas, esenciales para la comprensión y control de los procesos litorales. Se plantea el problema de la medición de profundidades y de otras variables descriptivas de la fenomenología oceánica y las fuentes de error dependiendo de las técnicas y métodos empleados. Se comparan los errores de medida típicos de los sistemas de medición de profundidades habituales señalando los niveles de error ...
José M. Santos
Full Text Available El objetivo de este estudio fue describir la etiología, presentación clínica, métodos diagnósticos, tratamiento, evolución y pronóstico alejado de 35 pacientes con pericarditis constrictiva crónica que fueron evaluados prospectivamente de acuerdo a un protocolo de enfermedad pericárdica que se aplica en nuestra Institución. En 24 (68% la pericarditis constrictiva fue idiopática y 11 (32% presentaron etiología definida, realizándose pericardiectomía en 34 (97%. Hubo 4 (12% muertes intrahospitalarias, todas con etiología definida. La mediana de seguimiento fue de 5.6 años (percentilos 25-75: 2.4-7.4 años. La supervivencia de acuerdo al método de Kaplan-Meier fue de 97% al año (IC 95% 80 - 99%, 83% a los 5 años (IC 95% 65 - 93%, 78% a los 7 años (IC 95% 60 - 90%, y 69% a los 10 años (IC 95% - 50% - 84%. En conclusión, la pericarditis constrictiva crónica es una enfermedad generalmente idiopática, de diagnóstico tardío, cuyo tratamiento es la pericardiectomía. Los pacientes con etiología específica presentaron mayor mortalidad intrahospitalaria. En el seguimiento a 10 años 2/3 de los pacientes están vivos y con mejoría de los síntomas.
Conclusiones: El desajuste paciente-prótesis es un hallaz-go frecuente en el paciente joven intervenido de recambio valvular aórtico por estenosis grave. Además, su influencia a los 30 días de seguimiento parece relevante.
Danikowski, K M; Jayaraman, S; Prabhakar, B S
Multiple sclerosis (MS) is a chronic debilitating disease of the central nervous system primarily mediated by T lymphocytes with specificity to neuronal antigens in genetically susceptible individuals. On the other hand, myasthenia gravis (MG) primarily involves destruction of the neuromuscular junction by antibodies specific to the acetylcholine receptor. Both autoimmune diseases are thought to result from loss of self-tolerance, which allows for the development and function of autoreactive lymphocytes. Although the mechanisms underlying compromised self-tolerance in these and other autoimmune diseases have not been fully elucidated, one possibility is numerical, functional, and/or migratory deficits in T regulatory cells (Tregs). Tregs are thought to play a critical role in the maintenance of peripheral immune tolerance. It is believed that Tregs function by suppressing the effector CD4+ T cell subsets that mediate autoimmune responses. Dysregulation of suppressive and migratory markers on Tregs have been linked to the pathogenesis of both MS and MG. For example, genetic abnormalities have been found in Treg suppressive markers CTLA-4 and CD25, while others have shown a decreased expression of FoxP3 and IL-10. Furthermore, elevated levels of pro-inflammatory cytokines such as IL-6, IL-17, and IFN-γ secreted by T effectors have been noted in MS and MG patients. This review provides several strategies of treatment which have been shown to be effective or are proposed as potential therapies to restore the function of various Treg subsets including Tr1, iTr35, nTregs, and iTregs. Strategies focusing on enhancing the Treg function find importance in cytokines TGF-β, IDO, interleukins 10, 27, and 35, and ligands Jagged-1 and OX40L. Likewise, strategies which affect Treg migration involve chemokines CCL17 and CXCL11. In pre-clinical animal models of experimental autoimmune encephalomyelitis (EAE) and experimental autoimmune myasthenia gravis (EAMG), several strategies
Impacto de un servicio de seguimiento farmacoterapéutico implementado a pacientes con diabetes mellitus tipo 2 Impact of a pharmaceutical follow-up service implemented in type 2 diabetes mellitus patients
Yaily Lazo Roblejo
Full Text Available Se evaluó el impacto de un servicio de seguimiento farmacoterapéutico implementado a 30 pacientes con diabetes mellitus tipo 2, dispensarizados en la Farmacia Principal Municipal Santiago de Cuba, según la metodología Dáder, en el período comprendido desde enero del 2009 hasta enero de 2010. El índice de impacto de la estabilidad clínica de los pacientes resultó moderado, mientras que en las intervenciones farmacéuticas aceptadas y el nivel de satisfacción de los pacientes, los índices de impacto obtenidos fueron elevados, por lo que el impacto global del servicio de seguimiento farmacoterapéutico implementado para la atención de estos fue considerado alto.The impact of a pharmacotherapy follow-up service was assessed in 30 patients dispensarized in municipal main drugstore of Santiago de Cuba according to Dáder's methodology from January, 2009 to January, 2010. The level of the clinical stability impact of patients was qualified as moderate, whereas in the accepted pharmaceutical interventions and at level of patient's satisfaction, the rates of impact achieved were high thus; the global above mentioned service repercussion was also qualified as high.
Raul Lopes Ruiz Jr
Full Text Available INTRODUÇÃO: Diversas variações da timectomia podem ser realizadas, dentre elas a transesternal ampliada. A literatura sugere que, quanto mais extenso o procedimento para ressecção da glândula e tecidos do mediastino anterior, melhores os resultados e prognóstico. OBJETIVO: Avaliar retrospectivamente a resposta à timectomia ampliada em portadores de Miastenia gravis. MÉTODO: Foram avaliados 46 portadores de Miastenia gravis, submetidos à plasmaferese pré-operatória e à timectomia ampliada, entre agosto de 1992 e janeiro de 2003, divididos em três grupos, segundo o tempo decorrido desde o início dos sintomas: menor que 12 meses, 13 a 24 meses e maior que 25 meses. RESULTADOS: Trinta e um pacientes eram do sexo feminino e 15 do masculino. A média de idade foi de 30 anos. O tempo médio de evolução da doença foi de 26,3 meses. O acompanhamento ambulatorial pós-operatório foi em média de 26,6 meses. Quanto ao grau de resposta à timectomia, 89% dos pacientes tiveram boa resposta, sendo que 50% apresentaram remissão completa. Ocorreu um óbito nesta série. O exame anatomopatológico demonstrou que a hiperplasia tímica foi o achado mais freqüente. Apenas 3 pacientes (6,5% apresentaram timomas benignos. Em 5 pacientes (10,8% encontramos tecido tímico extraglandular: na gordura peritímica em 2 deles, na gordura pericárdica em 1, junto ao nervo frênico esquerdo em outro e na janela aorto-pulmonar em outro. CONCLUSÃO: A timectomia ampliada para tratamento da Miastenia gravis mostrou-se segura, eficiente, e apresentou alta porcentagem de remissão completa. Houve a detecção de tecido tímico extraglandular em alguns pacientes. Tão logo seja feito o diagnóstico, está indicada como terapêutica associada à plasmaferese pré-operatória e à medicamentosa, independentemente da idade, patologia tímica, e início dos sintomas.BACKGROUND: Extended thymectomy is one of several types of thymectomy. Literature suggests that the
Kaplan, I.; Blakely, B.T.; Pavlath, G.K.; Travis, M.; Blau, H.M.
Antibodies to the acetylcholine receptor (AChR), which are diagnostic of the human autoimmune disease myasthenia gravis, block AChR function and increase the rate of AChR degradation leading to impaired neuromuscular transmission. Steroids are frequently used to alleviate symptoms of muscle fatigue and weakness in patients with myasthenia gravis because of their well-documented immunosuppressive effects. The authors show here that the steroid dexamethasone significantly increases total surface AChRs on cultured human muscle exposed to myasthenia gravis sera. The results suggest that the clinical improvement observed in myasthenic patients treated with steroids is due not only to an effect on the immune system but also a direct effect on muscle. They propose that the identification and development of pharmacologic agents that augment receptors and other proteins that are reduced by human genetic or autoimmune disease will have broad therapeutic applications
Sund, K.K.; Skeie, G.O.; Gilhus, N.E.; Aarli, J.A.; Varhaug, J.E.
The authors have compared clinical, immunological and radiological data in 20 patients with myasthenia gravis and thymoma and in 21 patients with myasthenia gravis and thymic atrophy. The median age at onset was 54 years in the thymoma group and 63 years in the thymic atrophy group. The severity of the disease was similar in the two groups, and there was no significant difference in the concentration of acetylcholine receptor antibodies. CA antibodies were demonstrated in 17/20 thymoma patients and in 6/21 with thymic atrophy, while 19/20 thymoma patients had antibodies to titin, compared with 9/21 among those with thymic atrophy. The diagnosis and treatment of patients with myasthenia gravis is based upon an evaluation of clinical, immunological and radiological data. 28 refs., 2 tabs
Full Text Available Intestinal pseudo-obstruction is considered to be one of the most frequent gastrointestinal manifestations of myasthenia gravis, accompanied by the presence of neoplasia of the thymus gland in the vast majority of the cases presented in the international literature. Despite the fact that myasthenia gravis has been implicated to be the cause of recurrent episodes of intestinal pseudo-obstruction, adhesive ileus has never been reported to complicate this – in any sense rare – condition. We present a unique case of a patient with myasthenia gravis, free of thymus neoplasia, who was submitted to emergency surgery due to the presence of extended adhesive ileus as a complication of chronic intestinal functional obstruction.
Kaplan, I.; Blakely, B.T.; Pavlath, G.K.; Travis, M.; Blau, H.M. (Stanford Univ. School of Medicine, CA (USA))
Antibodies to the acetylcholine receptor (AChR), which are diagnostic of the human autoimmune disease myasthenia gravis, block AChR function and increase the rate of AChR degradation leading to impaired neuromuscular transmission. Steroids are frequently used to alleviate symptoms of muscle fatigue and weakness in patients with myasthenia gravis because of their well-documented immunosuppressive effects. The authors show here that the steroid dexamethasone significantly increases total surface AChRs on cultured human muscle exposed to myasthenia gravis sera. The results suggest that the clinical improvement observed in myasthenic patients treated with steroids is due not only to an effect on the immune system but also a direct effect on muscle. They propose that the identification and development of pharmacologic agents that augment receptors and other proteins that are reduced by human genetic or autoimmune disease will have broad therapeutic applications.
Gratton, Sean M; Herro, Angela M; Feuer, William J; Lam, Byron L
Myasthenia gravis is an autoimmune disease of the neuromuscular junction, commonly affecting the ocular muscles. Cigarette smoking has been shown to influence many autoimmune diseases, including multiple sclerosis and rheumatoid arthritis, but its effect on myasthenia gravis has not been well studied. We sought to determine whether cigarette smoking influenced disease-related symptoms in ocular myasthenia gravis (OMG). We performed a prospective, clinic-based cross-sectional study in a single academic neuro-ophthalmology practice. All patients diagnosed with OMG between November 2006 and April 2014 were included. A prospective telephone survey was administered to determine smoking status and myasthenia gravis-related symptom severity. The main outcome measure was the myasthenia gravis-specific activities of daily living (MG-ADL) score, a well-validated marker of symptoms and quality of life in myasthenia gravis. Forty-four patients were included in the analysis. Comparison of MG-ADL ocular subscores between current smokers (3.4 ± 2.6), former smokers (1.8 ± 2.1), and never smokers (1.1 ± 1.5) revealed a statistically significant relationship (P = 0.031) where current smokers had the highest MG-ADL ocular subscores and never smokers the lowest. Comparison of MG-ADL total scores revealed the same relationship (current 5.6 ± 4.5, former 2.9 ± 3.1, never 1.4 ± 2.5, P = 0.003). There were borderline significant correlations of pack years with MG-ADL ocular subscore (r = 0.27, P = 0.074) and MG-ADL total score (r = 0.30, P = 0.051). Our findings indicate an association between cigarette smoking and symptom severity in OMG. This association suggests that smoking cessation in OMG patients may lead to improved symptom-related quality of life.
Baggi, Fulvio; Mantegazza, Renato; Antozzi, Carlo; Sanders, Donald
Clinical registries may facilitate research on myasthenia gravis (MG) in several ways: as a source of demographic, clinical, biological, and immunological data on large numbers of patients with this rare disease; as a source of referrals for clinical trials; and by allowing rapid identification of MG patients with specific features. Physician-derived registries have the added advantage of incorporating diagnostic and treatment data that may allow comparison of outcomes from different therapeutic approaches, which can be supplemented with patient self-reported data. We report the demographic analysis of MG patients in two large physician-derived registries, the Duke MG Patient Registry, at the Duke University Medical Center, and the INNCB MG Registry, at the Istituto Neurologico Carlo Besta, as a preliminary study to assess the consistency of the two data sets. These registries share a common structure, with an inner core of common data elements (CDE) that facilitate data analysis. The CDEs are concordant with the MG-specific CDEs developed under the National Institute of Neurological Disorders and Stroke Common Data Elements Project. © 2012 New York Academy of Sciences.
Kang, Ki Mun; Choi, Ihl Bohng; Kim, In Ah
Myasthenia Gravis (MG) is relatively rare occuring as one of important autoimmune disease to affect neuromuscular junction. This study was clinically to evaluate total body irradiation (TBI) against two patients including 33-year and 39-year females for chronic MG with thymoma who hospitalized in the St. Mary's Hospital, Catholic University since 1994 as well as who showed no response by thymectomy, immunotherapy and hormonal therapy. TBI designed by the dose of 150-180 cGy consisting of 10 cGy per fraction, three times a week, for 5-6 weeks using linear accelerator of 6 MV. During the treatment of TBI, they did complain acute side effect such as vomiting and also appear improved physical condition from 4-6 weeks after TBI. Through the follow-up period of 18 or 42 months after TBI, they did not have any symptomatic recurrence. Consequently, the results suggest that TBI can be used as an alternative tool for the patients concurrently for MG with thymoma who had been refractory to various conventional therapies like thymectomy, immunotherapy and hormonal therapy
Tanimura, Shigeo; Banba, Jiro; Masaki, Mikio; Irimoto, Masahiro; Matsushita, Akira
Comparison of radiographical findings of the thymus between pneumomediastinography and computed tomography were studied in 35 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 25 without thymoma confirmed at the operations. Pneumomediastinography was very useful to discern whether the thymoma was invasive or noninvasive, but not contributory to know whether the thymus was composed of folicular lymphoid hyperplasia or normal thyimic tissues. Computed tomography was also useful to dertermine the localization and the invasiveness of the thymoma, but not helpful to know whether the thymus was of follicular lymphoid hyperplasia or normal tissues. However, the finding of ''reticular pattern''-many small nodules scattered resicularly in the thymus-in computed tomography could be regarded as a sign suggesting follicular lymphoid hyperplasia of the thymus. Therfore, both pneumomediastinography and computed tomography were very useful in the diagnosis of the localization and the invasiveness of the thymoma but not for the diagnosis of follicular lymphoid hyperplasia of the thymus. Neverethless the finding of ''reticular pattern'' computred tomography was helpful in the diagnosis of follicular lymphoid hyperplasia. (author)
Full Text Available Myasthenia Gravis (MG is an autoimmune disorder, which may lead to paralysis and even death if not treated on time. One of its primary symptoms is severe muscular weakness, initially arising in the eye muscles. Testing the mobility of the eyeball can help in early detection of MG. In this study, software was designed to analyze the ability of the eye muscles to focus in various directions, thus estimating the MG risk. Progressive weakness in gazing at the directions prompted by the software can reveal abnormal fatigue of the eye muscles, which is an alert sign for MG. To assess the user’s ability to keep gazing at a specified direction, a fuzzy algorithm was applied to images of the user’s eyes to determine the position of the iris in relation to the sclera. The results of the tests performed on 18 healthy volunteers and 18 volunteers in early stages of MG confirmed the validity of the suggested software.
Mantegazza, Renato; Bonanno, Silvia; Camera, Giorgia; Antozzi, Carlo
Myasthenia gravis (MG) is an autoimmmune disease in which autoantibodies to different antigens of the neuromuscular junction cause the typical weakness and fatigability. Treatment includes anticholinesterase drugs, immunosuppression, immunomodulation, and thymectomy. The autoimmune response is maintained under control by corticosteroids frequently associated with immunosuppressive drugs, with improvement in the majority of patients. In case of acute exacerbations with bulbar symptoms or repeated relapses, modulation of autoantibody activity by plasmapheresis or intravenous immunoglobulins provides rapid improvement. Recently, techniques removing only circulating immunoglobulins have been developed for the chronic management of treatment-resistant patients. The rationale for thymectomy relies on the central role of the thymus. Despite the lack of controlled studies, thymectomy is recommended as an option to improve the clinical outcome or promote complete remission. New videothoracoscopic techniques have been developed to offer the maximal surgical approach with the minimal invasiveness and hence patient tolerability. The use of biological drugs such as anti-CD20 antibodies is still limited but promising. Studies performed in the animal model of MG demonstrated that several more selective or antigen-specific approaches, ranging from mucosal tolerization to inhibition of complement activity or cellular therapy, might be feasible. Investigation of the transfer of these therapeutic approaches to the human disease will be the challenge for the future. PMID:21552317
Tanimura, Shigeo; Sakaguchi, Kozo; Tomoyasu, Hiroshi; Banba, Jiro; Masaki, Mikio; Kurosaki, Atsuko; Matsushita, Hisashi [Toranomon Hospital, Tokyo (Japan)
Comparison of preoperative CT diagnosis and histopathological diagnosis for the thymus were studied in 39 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 29 without thymoma confirmed at the operation. CT diagnosis was 11 thymomas, 18 thymic hyperplasias and 10 normal thymuses. Eleven thymomas revealed histopathologically 9 thymomas, one follicular lymphoid hyperplasia (FLH) and one involved thymus. Out of 18 thymic hyperplasias 15 cases were FLH and 3 involved thymus. There were 5 involved thymuses, 4 FLHs and one thymoma in the 10 normal thymuses on CT. The finding of `reticular pattern`, many small nodules scattered reticularly in the thymus, in computed tomography could be regarded as a sign suggesting FLH of the thymus. The accuracy of this finding of CT was 83% for FLH. The finding of thymoma on CT revealed 82% of the accuracy. Therefore, CT was very useful in the diagnosis of the localization of the thymoma but not for the diagnosis of FLH of the thymus. Nevertheless the finding of `reticular pattern` on CT was helpful in the diagnosis of FLH. (author).
Braga, António Costa; Pinto, Clara; Santos, Ernestina; Braga, Jorge
We evaluated the clinical course during pregnancy and neonatal outcomes of a cohort of Portuguese women with myasthenia gravis (MG). Retrospective study. Twenty-five patients with 30 pregnancies were included. Mean maternal age was 32.4 ± 4.1 years. Miscarriage rate was 6.7%, with delivery of 28 newborns. Deterioration in MG during pregnancy occurred in 43.3%, and 46.4% occurred at postpartum. Eighty percent were medicated with pyridostigmine, 43.3% with corticosteroids, and 40% with intravenous immunoglobulin. There were no maternal or neonatal deaths. Mean gestational time at delivery was 38.2 weeks. No cases of fetal growth restriction, preeclampsia, preterm delivery, or fetal demise were observed. Global cesarean rate was 64.3%. Two newborns developed transient neonatal myasthenia. A high rate of clinical worsening of MG in the mother was observed in this retrospective study, which highlights the importance of a multidisciplinary approach for avoiding maternal adverse outcomes. Muscle Nerve 54: 715-720, 2016. © 2016 Wiley Periodicals, Inc.
Full Text Available Myasthenia gravis (MG is caused by antibodies that react mainly with the acetylcholine receptor on the postsynaptic site of the neuromuscular junction. A wide range of clinical presentations and associated features allow MG to be classified into subtypes based on autoantibody status. Striational antibodies, which react with epitopes on the muscle proteins titin, ryanodine receptor (RyR, and Kv1.4, are frequently found in MG patients with late-onset and thymoma. Antititin and anti-RyR antibodies are determined by enzyme-linked immunosorbent assay or immunoblot. More recently, a method for the detection of anti-Kv1.4 autoantibodies has become available, involving 12–15% of all MG patients. The presence of striational antibodies is associated with more severe disease in all MG subgroups. Anti-Kv1.4 antibody is a useful marker for the potential development of lethal autoimmune myocarditis and response to calcineurin inhibitors. Detection of striational antibodies provides more specific and useful clinical information in MG patients.
Tanimura, Shigeo; Sakaguchi, Kozo; Tomoyasu, Hiroshi; Banba, Jiro; Masaki, Mikio; Kurosaki, Atsuko; Matsushita, Hisashi
Comparison of preoperative CT diagnosis and histopathological diagnosis for the thymus were studied in 39 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 29 without thymoma confirmed at the operation. CT diagnosis was 11 thymomas, 18 thymic hyperplasias and 10 normal thymuses. Eleven thymomas revealed histopathologically 9 thymomas, one follicular lymphoid hyperplasia (FLH) and one involved thymus. Out of 18 thymic hyperplasias 15 cases were FLH and 3 involved thymus. There were 5 involved thymuses, 4 FLHs and one thymoma in the 10 normal thymuses on CT. The finding of 'reticular pattern', many small nodules scattered reticularly in the thymus, in computed tomography could be regarded as a sign suggesting FLH of the thymus. The accuracy of this finding of CT was 83% for FLH. The finding of thymoma on CT revealed 82% of the accuracy. Therefore, CT was very useful in the diagnosis of the localization of the thymoma but not for the diagnosis of FLH of the thymus. Nevertheless the finding of 'reticular pattern' on CT was helpful in the diagnosis of FLH. (author)
Oger, Joel; Frykman, Hans
This review describes the state of the art for the use of laboratory testing in myasthenia gravis. The review brings a detailed description of the different clinical forms of auto-immune myasthenia and of the Lambert Eaton Myasthenic Syndrome (LEMS). They stress the differences between the different forms of acquired (auto-immune) myasthenia. Then they present a summary of the different antibodies found in the disease. They insist on the advantage of the RIPA assay to measure antibodies to the acetylcholine receptor. They stress the different types of contribution of each of these antibodies to the clinical diagnosis. They also describe the methods to measure each of the specific antibodies that have recently permitted to split the diagnosis: Abs to omega-conotoxin receptor in Lambert Eaton Myasthenic Syndrome (LEMS), abs to the acetylcholine receptor (AchR) in MG, Abs to muscle specific tyrosine kinase (MuSK) in Ab negative MG, and Abs to low molecular weight receptor related low-density lipo protein-4 (LRP-4). They also broach over the striated antibodies, less frequent and clinically less useful such as anti-titin, -ryanodine, -agrin and -rapsyn. This represent a 360° view of the field as presented in Toronto in October 2014. Copyright © 2015. Published by Elsevier B.V.
Liu Fugeng; Wei Jiahu; Pan Jishu; Zhou Cheng; Chen Qihang; Yu Jingying; Wu Guogeng; Xu Xianhao
Objective: To evaluate the CT findings of lymphofollicular thymic hyperplasia in adult myasthenia gravis (MG). Methods: The CT findings of thymus area of 134 adult patients with lymphofollicular thymic hyperplasia in MG were reviewed, all of them with surgically and histologically proven diagnosis, and compared with the CT findings of 165 normal subjects. Results: In the group of patient, CT showed enlargement of thymus in 31 patients, 5 patients had nodule or mass ( 3 cm) and 9 patients (6.7%) had normal size thymus with soft-tissue density, it can considered with thymic hyperplasia. The spotty or streak shadow showed in other patients, though it could not be certain diagnosed as thymic hyperplasia, but could not be except it. The thymus area tissue complete replacement by fatty density were not found in patient group. The CT findings of patients had marked difference when compared with group of normal subjects (P<0.01), except the spotty or streak shadows. Conclusion: CT scan is an important method in diagnosing thymic lymphofollicular hyperplasia of MG in adult. (authors)
Kang, Ki Mun; Choi, Ihl Bohng; Kim, In Ah [College of Medicine, Catholic Univ., Seoul (Korea, Republic of)
Myasthenia Gravis (MG) is relatively rare occuring as one of important autoimmune disease to affect neuromuscular junction. This study was clinically to evaluate total body irradiation (TBI) against two patients including 33-year and 39-year females for chronic MG with thymoma who hospitalized in the St. Mary's Hospital, Catholic University since 1994 as well as who showed no response by thymectomy, immunotherapy and hormonal therapy. TBI designed by the dose of 150-180 cGy consisting of 10 cGy per fraction, three times a week, for 5-6 weeks using linear accelerator of 6 MV. During the treatment of TBI, they did complain acute side effect such as vomiting and also appear improved physical condition from 4-6 weeks after TBI. Through the follow-up period of 18 or 42 months after TBI, they did not have any symptomatic recurrence. Consequently, the results suggest that TBI can be used as an alternative tool for the patients concurrently for MG with thymoma who had been refractory to various conventional therapies like thymectomy, immunotherapy and hormonal therapy.
Morita Maria da Penha A.
Full Text Available Myasthenia gravis (MG in childhood is rare comprising 10 to 20 % of all myasthenic patients. We studied 18 patients with MG whose first symptoms started from 1 to 12 years of age, followed at the Department of Neurology of the UNIFESP-EPM, from January 1983 to August 1997. There were 10 girls and 8 boys (1.2:1. Eleven patients (61% presented moderate or severe generalized disease and 4 (22% had at least one myasthenic crisis. EMG with supramaximal repetitive nerve stimulation was diagnostic in 8 (47% out of 17 patients, and chest CT was normal in 14 patients. Seropositivity to acetylcholine receptor antibodies was found in 81.6% (9 out of 11 tested and the levels had no relation to clinical severity. Nine out of 16 patients (56% worsened with pyridostigmine alone and were treated with prednisone. Four out of those nine continued worsening despite steroids and were subjected to thymectomy (all showed thymic lymphoid follicular hyperplasia. Three patients (75% improved markedly after thymectomy and one (25% worsened, eventually getting better with intravenous immunoglobulin and oral azathioprine. MG treatment, using all resources available, has to be individualized for each child.
Peeler, Crandall E; De Lott, Lindsey B; Nagia, Lina; Lemos, Joao; Eggenberger, Eric R; Cornblath, Wayne T
The sensitivity of acetylcholine receptor (AChR) antibody testing is thought to be lower in ocular myasthenia gravis (OMG) compared with generalized disease, although estimates in small-scale studies vary. There is little information in the literature about the implications of AChR antibody levels and progression from OMG to generalized myasthenia gravis. To test the hypothesis that serum AChR antibody testing is more sensitive in OMG than previously reported and to examine the association between AChR antibody levels and progression from OMG to generalized myasthenia gravis. A retrospective, observational cohort study was conducted of 223 patients (mean [SD] age, 59.2 [16.4] years; 139 [62.3%] male) diagnosed with OMG between July 1, 1986, and May 31, 2013, at 2 large, academic medical centers. Baseline characteristics, OMG symptoms, results of AChR antibody testing, and progression time to generalized myasthenia gravis (if this occurred) were recorded for each patient. Multiple logistic regression was used to measure the association between all clinical variables and antibody result. Kaplan-Meier survival analysis was performed to examine time to generalization. Among the 223 participants, AChR antibody testing results were positive in 158 participants (70.9%). In an adjusted model, increased age at diagnosis (odds ratio [OR], 1.03; 95% CI, 1.01-1.04; P = .007) and progression to generalized myasthenia gravis (OR, 2.92; 95% CI, 1.18-7.26; P = .02) were significantly associated with positive antibody test results. Women were less likely to have a positive antibody test result (OR, 0.36; 95% CI, 0.19-0.68; P = .002). Patients who developed symptoms of generalized myasthenia gravis had a significantly higher mean (SD) antibody level than those who did not develop symptoms of generalized myasthenia gravis (12.7 [16.5] nmol/L vs 4.2 [7.9] nmol/L; P = .002). We demonstrate a higher sensitivity of AChR antibody testing than previously reported in the
Glathe, S.; Neufang, K.F.R.; Haupt, F.W.
Radiologic imaging in myasthenia gravis is used for the evaluation of pathologic changes of the thymus gland. Computed tomography can demonstrate tumors of the anterior mediastinum in nearly 90% and is therefore superior to conventional radiography.Because of the variety of size and shape of the normal thymus gland, differentiation between normal thymus, follicular hyperplasia and thymoma is rarely possible especially in younger patients. In elderly patients with myasthenia gravis and involution of the thymus gland tumors of the thymus are reliably detected by computed tomography, whereas the ability of computed tomography to predict the histological diagnosis is poor even with intravenous administration of contrast media. (orig.) [de
Linda L Kusner
Full Text Available The mechanisms that underlie the development and maintenance of autoimmunity in myasthenia gravis are poorly understood. In this investigation, we evaluate the role of survivin, a member of the inhibitor of apoptosis protein family, in humans and in two animal models. We identified survivin expression in cells with B lymphocyte and plasma cells markers, and in the thymuses of patients with myasthenia gravis. A portion of survivin-expressing cells specifically bound a peptide derived from the alpha subunit of acetylcholine receptor indicating that they recognize the peptide. Thymuses of patients with myasthenia gravis had large numbers of survivin-positive cells with fewer cells in the thymuses of corticosteroid-treated patients. Application of a survivin vaccination strategy in mouse and rat models of myasthenia gravis demonstrated improved motor assessment, a reduction in acetylcholine receptor specific autoantibodies, and a retention of acetylcholine receptor at the neuromuscular junction, associated with marked reduction of survivin-expressing circulating CD20+ cells. These data strongly suggest that survivin expression in cells with lymphocyte and plasma cell markers occurs in patients with myasthenia gravis and in two animal models of myasthenia gravis. Survivin expression may be part of a mechanism that inhibits the apoptosis of autoreactive B cells in myasthenia gravis and other autoimmune disorders.
José Lamartine de Assis
Full Text Available Foram estudados os resultados do tratamento da miastenia grave em 12 pacientes, sendo 10 com a forma generalizada severa, um com a forma generalizada moderada e um com miastenia acentuada associada a polimiosite. Sete pacientes foram previamente timectomizados e somente um apresentava timoma. Todos estavam em uso de doses elevadas de drogas anticolinesterásicas com resposta terapêutica pobre ou nula. Um paciente foi tratado com dexametaxona, outro com dexametazona seguida de prednisona e os restantes com prednisona, sempre em doses altas inicialmente (100 mg por dia ou em dias alternados e com redução gradativa ulterior até doses de manutenção. Os corticosteróides foram administrados sempre pela via oral, por períodos que variaram de duas semanas até dois anos e meio. Em conclusão, 75% de 12 pacientes com miastenia grave generalizada foram influenciados favoravelmente com este método terapêutico. Deste grupo apenas um apresentava timoma e não respondeu a qualquer tratamento. Outro paciente apresentava polimiosite associada e teve excelente resposta terapêutica. Dois miastênicos sem timoma tiveram a sintomatologia exacerbada durante o tratamento. Nenhum efeito colateral importante foi observado, mesmo nos pacientes tratados a longo prazo.The results of treatment of myasthenia gravis in 12 patients are reported. There were 10 cases with severe generalized form and two cases with moderate generalized form, one of which associated with polymyositis. Seven patients had prior thymectomy and one of them had a thymoma. All the patients were receiving anticholinesterase drugs with poor response or without any response. One patient received a short, intensive course of dexametazone, and other patient used prednisone after a more prolonged dexametazone course. The remaining patients received prednisone, always beginning with high (100 mg alternate day oral single-doses. This therapy has been maintained for a period thought to be sufficient
Marta L. Tamayo F.
Full Text Available
Introducción: Aunque ciertamente la Fenilcetonuria (PKU no sea un desorden metabólico común en Colombia, médicos generales, pediatras y genetistas deben estar alerta de su presencia y, deben propender por un diagnóstico completo que permita iniciar un manejo nutricional adecuado temprano. De manera que no sólo es importante el diagnóstico, sino la asesoría genética apropiada, el manejo y seguimiento. Sólo de esta manera se logran minimizar las secuelas y en especial, el daño neurológico propio de esta alteración metabólica, dado que es una de las pocas patologías genéticas en la que es posible ofrecer algún tipo de manejo que evite daños devastadores y ofrezca así un mejor pronóstico. Es igualmente importante estar atentos a los procesos del sistema de salud colombiano, que impone trabas y demoras en los manejos y tratamientos de estas enfermedades poco frecuentes, pues son causa de baja adherencia, suspensión de tratamientos y empeoramiento clínico de nuestros pacientes, con deterioro irreversible muy negativo en estas familias afectadas vulnerables e indefensas.
Objetivo: En este trabajo se realizó seguimiento durante un año y medio de evolución bajo manejo nutricional a una familia colombiana con diagnóstico clínico, bioquímico y molecular de Fenilcetonuria (PKU.
Materiales y Métodos: A una familia colombiana con diagnóstico de Fenilcetonuria, con 4 individuos afectados, se le hizo el seguimiento clínico de su evolución durante un año y medio, mientras recibían el tratamiento consistente en el manejo nutricional adecuado para su enfermedad de base, la ingesta de preparados de fórmulas comerciales adecuadas y seguimiento de pruebas bioquímicas y de evolución clínica con o sin tratamiento.
Resultados: Se presentan los resultados de la evolución clínica bajo tratamiento con manejo nutricional adecuado durante un a
Soetje, G.; Brinkmann, G. (Kiel Univ. (Germany, F.R.). Abt. Radiologie); Striepling, E.; Engemann, R. (Kiel Univ. (Germany, F.R.). Abt. Allgemeine Chirurgie)
A 40-year old woman with an invasive thymoma and myasthenia gravis is described in this article. Chest-x-ray, CT and MRI of the mediastinum could not offer definite results on tumour malignancy. Radical surgical removal was the consequence. This revealed a tumour infiltration of the pleura and pericardium; hence an adjuvant irradiation must have been performed. Mestinon-treatment was afterwards gradually reduced. (orig.).
Sherifa A Hamed
Full Text Available Aim: During the past decade, there has been an increasing interest in the evaluation of cognitive function in myasthenia gravis (MG, neuromuscular transmission disorder caused by acetylcholine receptor auto-antibodies. However, the results of previous studies on cognition and MG are inconsistent and controversial. This study aimed to evaluate cognition in patients with mild/moderate grades of MG. Methods: This study included 20 patients with MG with a mean age of 28.45 ± 8.89 years and duration of illness of 3.52 ± 1.15 years. Cognition was tested using a sensitive battery of psychometric testing (Mini-mental State Examination [MMSE], Stanford-Binet Intelligence Scale 4 th edition [SBIS] and Wechsler Memory Scale-Revised [WMS-R] and by recording P300 component of event-related potentials (ERPs, a neurophysiological analog for cognitive function. Results: Compared with healthy subjects (n = 20, patients had lower total scores of cognitive testing (MMSE, SBIS and WMS-R (P = 0.001, higher Beck Depression Inventory 2 nd edition scores (P = 0.0001 and prolonged latencies (P = 0.01 and reduced amplitudes (P = 0.001 of P300 component of ERPs. Correlations were identified between total scores of cognitive testing and age (r = -0.470, P = 0.010, duration of illness (r = -0.788, P = 0.001 and depression scores (r = -0.323, P = 0.045. Using linear regression analysis and after controlling for age and depression scores, a significant correlation was identified between total scores of cognitive testing and duration of illness (β = -0.305, P = 0.045. Conclusion: Patients with mild/moderate MG may have cognitive dysfunction. This is important to determine prognosis and managing patients.
Avidan, Nili; Le Panse, Rozen; Harbo, Hanne F
OBJECTIVE: To identify novel genetic loci that predispose to early-onset myasthenia gravis (EOMG) applying a two-stage association study, exploration, and replication strategy. METHODS: Thirty-four loci and one confirmation loci, human leukocyte antigen (HLA)-DRA, were selected as candidate genes...
Basaran-Akgul, Nese; Rasco, Barbara A
Dielectric properties (the dielectric constant (ε') and the dielectric loss factor (ε″)) and the penetration depth of raw eye of round beef Semitendinosus muscle, raw beef marinated in gravy, raw beef cooked in gravy, and gravy alone were determined as a function of the temperature (20-130 °C) and frequency (27-1,800 MHz). Both ε' and ε″ values increased as the temperature increased at low frequencies (27 and 40 MHz). At high frequencies (915 and 1,800 MHz), ε' showed a 50 % decrease while ε″ increased nearly three fold with increasing temperature in the range from 20 to 130 °C. ε' increased gradually while ε″ increased five fold when the temperature increased from 20 to 130 °C. Both ε' and ε″ of all samples decreased with increase in frequency. Marinating the beef in gravy dramatically increased the ε″ values, particularly at the lower frequencies. Power penetration depth of all samples decreased with increase temperature and frequency. These results are expected to provide useful data for modeling dielectric heating processes of marinated muscle food.
Fujii, Tateo; Kyoui, Daisuke; Takahashi, Hajime; Kuda, Takashi; Kimura, Bon; Washizu, Yukio; Emoto, Eiji; Hiramoto, Tadahiro
Kusaya is a salted, dried fish product traditionally produced on the Izu Islands in Japan. Fish are added to kusaya gravy repeatedly and intermittently, and used over several hundred years, which makes unique microbiota and unique flavors. In this study, we performed a metagenomic analysis to compare the composition of the microbiota of kusaya gravy between different islands. Twenty samples obtained from a total of 13 manufacturers on three islands (Hachijojima, Niijima, and Oshima Islands) were analyzed. The statistical analysis revealed that the microbiota in kusaya gravy maintain a stable composition regardless of the production steps, and that the microbiota are characteristic to the particular islands. The bacterial taxa common to all of the samples were not necessarily the dominant ones. On the other hand, the genera Halanaerobium and Tissierella were found to be characteristic to the microbiota of one or two islands. Because these genera are known to be present in the natural environment, it is likely that the bacterial strains peculiar to an island had colonized kusaya gravy for many years. The results of this study revealed an influence of geographical conditions on the microbiota in fermented food. Copyright © 2016 Elsevier B.V. All rights reserved.
Hansen, Julie S; Danielsen, Ditte H; Somnier, Finn E
INTRODUCTION: In previous studies of myasthenia gravis (MG), increased mortality has been reported. The aim of this study was to estimate mortality in patients with acetylcholine receptor antibody-positive (AChR-Ab-seropositive) MG in a nationwide population-based, long-term follow-up study...
Possibilities of manifestation of an additional neutron vector boson, the existence of which is predicted by the 7-dimensional theory of gravy-electro-weak interactions, are analyzed. A particular case of muon neutrino scattering on a muon is considered. In this case additional neutral current manifests both at high and at relatively low energies of particle collisions
Citirak, Gülsenay; Cejvanovic, Sanja; Andersen, Henning
INTRODUCTION: The aim of this observational, cross-sectional study was to quantify the potential presence of muscle weakness among patients with generalized myasthenia gravis (gMG). The influence of gender, treatment intensity and disease duration on muscle strength and disease progression was also...
Vinge, Lotte; Jakobsen, Johannes; Pedersen, Asger Roer
INTRODUCTION: In patients with myasthenia gravis (MG), muscle strength is expected to decrease gradually during the day due to physical activities. METHODS: Isometric muscle strength at the shoulder, knee, and ankle was determined in 10 MG patients (MGFA class II-IV) who were receiving usual...
Myasthenia gravis (MG) is an immunological disorder characterized by damaged acetylcholine receptors (AchR) due to antibody, which not only blocks the receptor site but also causes degenerations of the receptors.. It is closely associated with disorders of similar pathogenesis, such as pernicious anaemia, thyrotoxicosis, ...
Saidi, Trust; Sivarasu, Sudesh; Douglas, Tania S
Pharmacologic treatment of Myasthenia Gravis presents challenges due to poor tolerability in some patients. Conventional ptosis crutches have limitations such as interference with blinking which causes ocular surface drying, and frequent irritation of the eyes. To address this problem, a modular and adjustable ptosis crutch for elevating the upper eyelid in Myasthenia Gravis patients has been proposed as a non-surgical and low-cost solution. Areas covered: This paper reviews the literature on the challenges in the treatment of Myasthenia Gravis globally and focuses on a modular and adjustable ptosis crutch that has been developed by the Medical Device Laboratory at the University of Cape Town. Expert commentary: The new medical device has potential as a simple, effective and unobtrusive solution to elevate the drooping upper eyelid(s) above the visual axis without the need for medication and surgery. Access to the technology is provided through an open source platform which makes it available globally. Open access provides opportunities for further open innovation to address the current limitations of the device, ultimately for the benefit not only of people suffering from Myasthenia Gravis but also of those with ptosis from other aetiologies.
Leonardi, Matilde; Raggi, Alberto; Antozzi, Carlo; Confalonieri, Paolo; Maggi, Lorenzo; Cornelio, Ferdinando; Mantegazza, Renato
The objective of this study is to describe functional profiles of patients with myasthenia gravis (MG), and the relationships among symptoms, activities and environmental factors (EF), by using WHO's International Classification of Functioning Disability and Health (ICF). Patients were consecutively enrolled at the Besta Institute of Milan, Italy.…
McGurgan, Iain J
Increased rates of NMSC (nonmelanoma skin cancer) have recently been reported in people with MG (myasthenia gravis) receiving azathioprine treatment. Guidelines on azathioprine for patients with dermatological and gastrointestinal disorders stress the importance of NMSC risk awareness and prevention. The aim of this study is to assess whether MG patients are being informed of this risk.
Horigome, H; Nomura, T; Saso, K; Joh, T; Ohara, H; Akita, S; Sobue, S; Mizuno, Y; Kato, Y; Itoh, M
We present a case that suggests a relationship between primary biliary cirrhosis and myasthenia gravis. A 43-year-old Japanese woman was admitted to the Nagoya City University Medical School, First Department of Internal Medicine with abnormal liver function in August 1991. She had had ptosis of the right eye since 1990. She had not been treated for liver disease. Ptosis of the right eye and hepatomegaly were present. Serum laboratory examinations revealed elevated biliary enzymes and IgM levels; tests were positive for antimitochondrial antibody and antiacetylcholine antibody. Liver histology revealed chronic non-suppurative destructive cholangitis and led to a diagnosis of primary biliary cirrhosis. The tensilon test was positive. Electromyography with repetitive motor nerve stimulation revealed a neuromuscular junction defect; i.e., the primary characteristic of myasthenia gravis. The patient was diagnosed with myasthenia gravis. Although the development of myasthenia gravis has previously been reported in patients with primary biliary cirrhosis during D-penicillamine administration, this is a very rare case of the coexistence of both diseases before such treatment.
José Daniel Pabón-Caicedo
Full Text Available Como fase inicial de un estudio de los aspectos regionales del cambio climático se presenta un análisis preliminar de las series de registros de temperatura medias anuales para diferentes regiones y altitudes en territorio colombiano, con el propósito de seleccionar o conformar series de referencia para hacer el seguimiento del calentamiento global y para el estudio del cambio climático en general.
Conclusiones: El seguimiento de las recomendaciones de las guías clínicas para la prevención de la enfermedad cerebrovascular en atención primaria es mejorable, especialmente en los más jóvenes. Son necesarios cambios organizativos e intervención más activa de los profesionales, así como estrategias para combatir la inercia terapéutica.
Cobián Rodríguez MB
Full Text Available Introducción: El seguimiento farmacoterapéutico es un servicio que atiende las necesidades de los enfermos crónicos polimedicados pero invierte muchos recursos en un solo paciente. El objetivo del estudio fue conocer las variaciones en la dispensación de medicamentos derivadas del seguimiento farmacoterapéutico y determinar los recursos invertidos en el funcionamiento y sus costes asociados. Métodos: Se realizó el servicio de seguimiento farmacoterapéutico durante seis meses de una muestra de 20 pacientes seleccionados en función del riesgo de presentar RNM utilizando el método Dáder. Se registraron los recursos materiales, de información y humanos utilizados y se calculó su coste. Resultados: El número de medicamentos dispensado a los pacientes del programa disminuyó un 8,4 %. El coste del servicio de SFT de un paciente medio fue de 87 euros en los seis meses de estudio, siendo mayor en los pacientes que presentaban un mayor riesgo de presentar RNM Discusión: El servicio de SFT disminuye los ingresos de la farmacia al disminuir la dispensación de medicamentos y supone un coste para la farmacia relacionado fundamentalmente con el tiempo de trabajo de los farmacéuticos. Desde el punto de vista del pagador, el ahorro en medicamentos pagaría la mayor parte de este servicio.
Hussain, N.; Ahmed, S.W.; Ahmed, T.; Hafeez, A.B.; Baloch, R.; Ali, S.
Objective: To determine the outcome of thymectomy in patients with myasthenia gravis and safety of median sternotomy approach. Methodology: An observational descriptive study was conducted in the department of thoracic surgery JPMC from February 2005 to January 2009. Twenty two patients having persistent generalized or ocular myasthenia gravis referred to our department by neurologists and general physicians, partially or not responding to medical treatment with or without thymoma, were included in the study. Those who were not fit for anaesthesia were excluded. Preoperatively 2 to 3 sessions of plasmaphaeresis were done and each patient was given anti myasthenia gravis treatment. Clinical staging was done by Modified Osserman classification. Median sternotomy approach was used. Outcome was assessed on the basis of remission of disease in different Osserman groups. All patients were followed for a minimum of 6 months. Results: Out of 22 patients, 16 (72.7%) were females and 6 (27.2%) males. Mean age at presentation was 35.2 +- 14.5 years. Mean duration of symptoms was 1.5 +- 1.2 years. A total of 4 (18.1%) patients with myasthenia gravis had thymoma and histopathology of 18 (81.9%) patients showed thymic hyperplasia. Remission was seen in most grades of Osserman. The best response was seen in Grade I where all patients achieved remission. Most patients in Grade II A and II B were benefited. The only patient in Grade III had no improvement of symptoms. No patient in Grade IV underwent thymectomy. Overall 86.3 % had a positive outcome on basis of remission and improvement. Conclusion: Thymectomy by median sternotomy is safe and effective with more favourable outcomes for patients of myasthenia gravis not responding to medical treatment. (author)
Koopman, Wilma J; LeBlanc, Nicole; Fowler, Sue; Nicolle, Michael W; Hulley, Denise
Myasthenia gravis significantly impacts quality of life. However, the relationship between hope, coping, and quality of life (QOL)in myasthenia patients has not been studied (Kulkantrakorn & Jarungkiatkul, 2009; Raggi et al., 2010). The aim of this study was to explore the relationship between hope, coping, and quality of life in adults with myasthenia gravis. Subjects with MG (n = 100) completed six questionnaires, including a demographic profile, the Myasthenia Gravis Activities of Daily Living Scale (MG-ADL), Herth Hope Index (HHI), Jalowiec Coping Scale (JCS), Myasthenia Gravis Quality-of-Life Scale (MG-QOL15), and Short Form Health Survey (SF-36v2). Mean hope scores indicated a high level of hope. An optimistic coping style was the most common and effective coping strategy identified by subjects. Positive thinking and humour were also frequently used strategies. Participants identified quality of life as good tolerability, above general population mental well-being, and below general populationphysical well-being. Participants who identified good quality of life had low scores on the MG-QOL15 scale and high scores on the SF36v2. Hope and independence for activities of daily living were found to correlate with improved quality of life and mental well-being(p life in the relationship between hope and coping. Hope and coping were not important factors for well-being or quality of life. Nurses caring for adults with myasthenia gravis should use interventions that continue to support hope, quality of life, and coping throughout the unpredictable and chronic course of MG.
Torres-Vega, Estefanía; Mancheño, Nuria; Cebrián-Silla, Arantxa; Herranz-Pérez, Vicente; Chumillas, María J; Moris, Germán; Joubert, Bastien; Honnorat, Jérôme; Sevilla, Teresa; Vílchez, Juan J; Dalmau, Josep; Graus, Francesc; García-Verdugo, José Manuel; Bataller, Luis
To identify cell-surface antibodies in patients with neuromyotonia and to describe the main clinical implications. Sera of 3 patients with thymoma-associated neuromyotonia and myasthenia gravis were used to immunoprecipitate and characterize neuronal cell-surface antigens using reported techniques. The clinical significance of antibodies against precipitated proteins was assessed with sera of 98 patients (neuromyotonia 46, myasthenia gravis 52, thymoma 42; 33 of them with overlapping syndromes) and 219 controls (other neurologic diseases, cancer, and healthy volunteers). Immunoprecipitation studies identified 3 targets, including the Netrin-1 receptors DCC (deleted in colorectal carcinoma) and UNC5A (uncoordinated-5A) as well as Caspr2 (contactin-associated protein-like 2). Cell-based assays with these antigens showed that among the indicated patients, 9 had antibodies against Netrin-1 receptors (7 with additional Caspr2 antibodies) and 5 had isolated Caspr2 antibodies. Only one of the 219 controls had isolated Caspr2 antibodies with relapsing myelitis episodes. Among patients with neuromyotonia and/or myasthenia gravis, the presence of Netrin-1 receptor or Caspr2 antibodies predicted thymoma ( p myasthenia gravis, and neuromyotonia, often with Morvan syndrome ( p = 0.009). Expression of DCC, UNC5A, and Caspr2 proteins was demonstrated in paraffin-embedded thymoma samples (3) and normal thymus. Antibodies against Netrin-1 receptors (DCC and UNC5a) and Caspr2 often coexist and associate with thymoma in patients with neuromyotonia and myasthenia gravis. This study provides Class III evidence that antibodies against Netrin-1 receptors can identify patients with thymoma (sensitivity 21.4%, specificity 100%). © 2017 American Academy of Neurology.
Sudulagunta, Sreenivasa Rao; Sepehrar, Mona; Sodalagunta, Mahesh Babu; Settikere Nataraju, Aravinda; Bangalore Raja, Shiva Kumar; Sathyanarayana, Deepak; Gummadi, Siddharth; Burra, Hemanth Kumar
Introduction: Myasthenia gravis (MG) is an antibody mediated autoimmune neuromuscular disorder characterized by fatigable muscle weakness. A proportion of myasthenia gravis patients are classified as refractory due to non responsiveness to conventional treatment. This retrospective study was done to evaluate clinical profile, epidemiological, laboratory, and features of patients with MG and mode of management using rituximab and complications. Methods: Data of myasthenia gravis patients admitted or presented to outpatient department (previous medical records) with MG between January 2008 and January 2016 were included. A total of 512 patients fulfilled the clinical and diagnostic criteria of myasthenia gravis of which 76 patients met the diagnostic certainty for refractory myasthenia gravis and were evaluated. Results: Out of 76 refractory MG patients, 53 (69.73%) patients fulfilled all the three defined criteria. The median age of onset of the refractory MG group was 36 years with a range of 27–53 years. In our study 25 patients (32.89%) belonged to the age group of 21–30 years. Anti-MuSK antibodies were positive in 8 non-refractory MG patients (2.06%) and 36 refractory MG patients (47.36%). Mean HbA1C was found to be 8.6±2.33. The dose of administered prednisone decreased by a mean of 59.7% (p=3.3x10–8) to 94.6% (p=2.2x10–14) after the third cycle of rituximab treatment. Conclusion: The refractory MG patients are most commonly female with an early age of onset, anti-MuSK antibodies, and thymomas. Refractory MG patients have higher prevalence and poor control (HbA1C >8%) of diabetes mellitus and dyslipidemia probably due to increased steroid usage. Rituximab is very efficient in treatment of refractory MG with adverse effects being low. PMID:27790079
Mineo, Tommaso Claudio; Tamburrini, Alessandro; Schillaci, Orazio; Ambrogi, Vincenzo
Patients with thymoma and without clinical or electromyographical myasthenic signs may occasionally develop myasthenia several years after thymectomy. Hereby, we investigated the predictors and the evolution of this peculiar disease. We performed a retrospective analysis in 104 consecutive patients who underwent thymectomy between 1987 and 2013 for thymoma without clinical or electromyographic signs of myasthenia gravis. Predictors of post-thymectomy onset of myasthenia gravis were investigated with univariate time-to-disease analysis. Evolution of myasthenia was analyzed with time-to-regression analysis. Eight patients developed late myasthenia gravis after a median period of 33 months from thymectomy. No significant correlation was found for age, gender, Masaoka's stage, and World Health Organization histology. Only high preoperative serum acetylcholine-receptor antibodies titer (>0.3 nmol/L) was significantly associated with post-thymectomy myasthenia gravis at univariate time-to-disease (P = 0.003) analysis. Positron emission tomography was always performed in high-titer patients, and increased metabolic activity was detected in 4 of these patients. Surgical treatment through redo-sternotomy or video-assisted thoracoscopy was performed in these last cases with a remission in all patients after 12, 24, 32 and 48 months, respectively. No patient under medical treatment has yet developed a complete remission. In our study the presence of preoperative high-level serum acetylcholine receptor antibodies was the only factor significantly associated with the development of post-thymectomy myasthenia gravis. The persistence of residual islet of ectopic thymic tissue was one of the causes of the onset of myasthenia and its surgical removal was successful. Copyright © 2018 Elsevier Inc. All rights reserved.
Fukuhara, Mitsuro; Higuchi, Mitsunori; Owada, Yuki; Inoue, Takuya; Watanabe, Yuzuru; Yamaura, Takumi; Muto, Satoshi; Hasegawa, Takeo; Suzuki, Hiroyuki
Microscopic thymomas, defined as epithelial proliferations smaller than 1 mm in diameter, characteristically occur in patients with myasthenia gravis without macroscopic thymic epithelial tumors. However, some clinical and pathological aspects of this entity are still unclear. This retrospective study includes five consecutive patients who had undergone extended thymectomy for myasthenia gravis at our institution from April 2007 to March 2016 and in whom microscopic thymomas were diagnosed by histopathological examination of the resected specimens. During the same period, we performed 32 extended transsternal thymothymectomies/thymectomies in patients with myasthenia gravis, including the above five cases. We here review 18 cases of microscopic thymoma, including our five cases and 13 previously reported cases. The incidence of previously undiagnosed microscopic thymoma in patients undergoing thymectomy for myasthenia gravis in our institution is 15.2%. Serum preoperative anti-acetylcholine receptor antibody (anti-AchR Ab) titers were abnormally high in all of our five cases h (74.4±53.3 nmol/L) and decreased significantly after surgery (11.7±13.5 nmol/L, P=0.037). We divided our cases into the following three groups: microscopic thymoma group (Group M), thymoma group (Group T) and non-thymic tumor group (Group N). The mean preoperative anti-AchR Ab titers of these groups were 74.4, 26.5, and 368 nmol/L, respectively. All these values decreased postoperatively. The mean anti-AchR Ab titer was significantly higher in Group M than in Group T (P=0.034). All five cases in Group M were found by post-operative pathological examination to have multifocal type A thymomas. Microscopic thymomas tend to be multifocal type A thymomas. Anti-AchR Ab titers decreased significantly in all groups. It is very important to both perform complete extended thymectomies in patients with myasthenia gravis and pathological examination of thin slices of thymic tissue to maximize detection
Howard, James F; Utsugisawa, Kimiaki; Benatar, Michael
BACKGROUND: Complement is likely to have a role in refractory generalised myasthenia gravis, but no approved therapies specifically target this system. Results from a phase 2 study suggested that eculizumab, a terminal complement inhibitor, produced clinically meaningful improvements in patients...... with anti-acetylcholine receptor antibody-positive refractory generalised myasthenia gravis. We further assessed the efficacy and safety of eculizumab in this patient population in a phase 3 trial. METHODS: We did a phase 3, randomised, double-blind, placebo-controlled, multicentre study (REGAIN) in 76...... hospitals and specialised clinics in 17 countries across North America, Latin America, Europe, and Asia. Eligible patients were aged at least 18 years, with a Myasthenia Gravis-Activities of Daily Living (MG-ADL) score of 6 or more, Myasthenia Gravis Foundation of America (MGFA) class II-IV disease...
Shirai, Takushi; Sano, Tasuku; Kamijo, Fuminao; Saito, Nana; Miyake, Tomomi; Kodaira, Minori; Katoh, Nagaaki; Nishie, Kenichi; Okuyama, Ryuhei; Uhara, Hisashi
We reported an 81-year-old woman with metastatic melanoma, in whom myasthenia gravis and rhabdomyolysis developed after nivolumab monotherapy. The first symptom of myasthenia gravis was dyspnea. Ultrasonography detected hypokinesis of the bilateral diaphragm suggesting myasthenia gravis, although there was no abnormal finding of the lungs in computed tomography images. Acetylcholine receptor binding antibodies were low-titer positive in the preserved serum before administration of nivolumab, strongly suggesting that the myasthenia gravis was a nivolumab-related immune adverse event. Despite the remarkable clinical benefits of immune checkpoint inhibitors for patients with advanced melanoma, it is important to recognize unexpected immune-related adverse events. © The Author 2015. Published by Oxford University Press. All rights reserved. For Permissions, please email: firstname.lastname@example.org.
Yusnita Hamzah*; Ng Kim Yee
This research was carried out to study the physical and chemical characteristics of pumpkin Javanese noodle gravy prepared in powder form. The finished product is categorized as an instant product as it only needs to be rehydrated with warm water (60°C) before consumption. Five gravy formulations were developed with the incorporation of two types of flour (tapioca and corn flours) as the thickening agent. Physical characteristics including colour and swelling properties of the final products ...
Baggi, Fulvio; Andreetta, Francesca; Maggi, Lorenzo; Confalonieri, Paolo; Morandi, Lucia; Salerno, Franco; Bernasconi, Pia; Montomoli, Cristina; Barberis, Massimo; Mantegazza, Renato; Antozzi, Carlo
Patients with myasthenia gravis (MG) are subgrouped as acetylcholine receptor (AChR)-positive, muscle-specific kinase (MuSK)-positive, and AChR/MuSK-negative MG (or double negative [DN]) on the basis of autoantibody assay. We investigated the relationships between autoantibody specificity, main clinical features, and outcome of the disease, in particular the occurrence of complete stable remission (CSR), by means of a retrospective study on a cohort of 677 Italian patients with MG. A total of 517 (76%) patients with AChR-positive MG, 55 (8%) patients with MuSK-positive MG, and 105 (16%) patients with DN MG were included in the study. Kaplan-Meier and Cox proportional hazard regression analyses were used to evaluate associations between baseline characteristics, antibody specificity, and CSR. Clinical stage at onset and at maximal worsening was more severe for MuSK-positive patients: bulbar impairment at maximal worsening was found in 83.6% of MuSK-positive patients compared with 58.6% of AChR-positive patients and 43.8% of DN patients (p CSR was observed in 3.6% of MuSK-positive patients compared with 22.2% of AChR-positive and 21.9% of DN patients. In the whole MG cohort, onset before age 40 (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.27-3.02, p = 0.002) and ocular and generalized clinical stages at maximal worsening were associated with CSR (ocular, HR = 8.05, 95% CI 1.88-34.53, p = 0.005; generalized, HR = 3.71, 95% CI 1.16-11.90, p = 0.023; bulbar, HR = 3.16, 95% CI 1.00-10.05, p = 0.051). MuSK antibodies identify a clinically distinguishable, more severe form of MG since the disease onset, with a lower occurrence of CSR. These features should be considered by the clinician in the management of this particular form of MG.
Full Text Available Resumen: El problema del seguimiento de trayectoria en robots manipuladores ha sido abordado aplicando una gran variedad de controladores, desde estructuras sencillas basadas en PD hasta otras más complejas basadas en controladores adaptativos y robustos. Estas últimas técnicas presentan inconvenientes como la presunción de ciertas cotas en los términos de la ecuación dinámica del robot o la no inclusión de las ligaduras del sistema en el algoritmo de control. En el presente trabajo se hace una revisión de las técnicas clásicas de control de manipuladores y se introduce un conjunto de técnicas novedosas de control robusto y de control predictivo, con las que se evitan los problemas comentados. En particular se describe un controlador con una acción robusta autoadaptativa, necesaria para evitar los errores en la cancelación de términos no lineales de la dinámica del robot. Este esquema es mejorado mediante técnicas predictivas que permiten la inclusión de las ligaduras de movimiento del robot en el algoritmo de control. Se incluyen resultados reales y en simulación en un robot PUMA-560 de Unimation que prueban la bondad de dichos controladores. Palabras clave: Manipuladores robot, Control Robusto, Control Adaptativo, Control Autoadaptativo, Control Predictivo, Implementación, Tareas en tiempo real
Thaiane Soares Montandon
Full Text Available En los últimos años el crecimiento de la población y la acción del hombre se volvieron muchos ecosistemas, incrementando significativamente la cantidad de áreas degradadas. En vista de la necesidad de recuperar estas áreas, el gobierno brasileño determina la realización de zonas de recuperación de Proyectos Degradadas (Prads, incluyendo el monitoreo y evaluación de la zona. Este artículo se basa en dos formas de monitoreo, la SER propuesto (2004, que cuenta con directrices de recuperación universales, 9 atributos de las áreas restauradas y 3 estrategias de evaluación, aunque tiene indicadores no RAD ;. Y la propuesta MELO et al. (2010, que presenta un Proyecto Matriz de Evaluación de la Restauración, con 7 indicadores RAD. Los indicadores de sostenibilidad para estas áreas se definieron a través del Método Delphi. La aplicación de la matriz de indicadores de sostenibilidad es una herramienta importante para la planificación, seguimiento y evaluación de las áreas degradadas y el uso del método Delphi para construir la matriz asegura un trabajo de monitoreo y evaluación más eficiente y completa. DOI: http://dx.doi.org/10.12957/sustinere.2015.17326
Full Text Available La mSalud es una herramienta práctica, útil y disponible para la comunicación unidireccional o bidireccional entre profesionales de la salud y el paciente, cuyo uso es especialmente promisorio en países como Chile, con una amplia y creciente cobertura de telefonía móvil muy bien aceptada por la población. Nuestro objetivo es mostrar el proceso de diseño de un modelo de comunicación y seguimiento móvil, destinado a facilitar la comunicación entre profesionales de los centros atención primaria en salud y sus usuarios, para lograr el diagnóstico oportuno e inicio del tratamiento de la diabetes mellitus de tipo 2 (DM2. Este modelo se caracteriza por utilizar el teléfono móvil como herramienta de comunicación, ser un método unidireccional (desde los centros de salud hacia los usuarios, estar integrado con la atención presencial que se entrega en los centros de salud, utilizar diferentes estrategias de comunicación (vía voz y escrita, y funcionar integrado en un software diseñado en código abierto. El sistema incluyó la implementación de comunicación personalizada, comunicación automatizada de voz y comunicación automatizada escrita a través de servicio de mensaje corto de voz. Se describen estas estrategias y los componentes del sistema. Entre las lecciones aprendidas, se destaca el aporte de lograr implementar la innovación tecnológica COSMOS (sistemas operadores modulados consolidados en línea, por su sigla en inglés, para apoyar el proceso de cuidado de la salud de las personas con sospecha de DM2 en centros de atención primaria de salud. Para este logro, resulta imprescindible el trabajo conjunto con los equipos en terreno.
Ricci A, Paolo; Lema C, Rodrigo; Solà D, Vicente; Wash F, Alex; Pardo S, Jack
Antecedentes: La neuralgia del nervio pudendo pocas veces es sospechada y menos diagnosticada. Por esta razón, las pacientes que la padecen, consultan múltiples veces antes de llegar a un diagnóstico definitivo. Objetivo: Revisar la seguridad y eficacia de la infiltración de nervio pudendo, en el tratamiento del dolor en pacientes con neuralgia del nervio pudendo. Método: Seguimiento prospectivo de cinco pacientes ingresadas bajo el diagnóstico de síndrome de atrapamiento del nervio pudendo. ...
John G. Skedros
Full Text Available This report describes a patient who had a series of daily interscalene nerve blocks to treat pain following a shoulder manipulation for postsurgical stiffness. She experienced acute respiratory compromise that persisted for many weeks. All typical and unusual causes of these symptoms were ruled out. Her treating pulmonologist theorized that the ipsilateral carotid body had been injured. However, it was subsequently determined that the constellation of symptoms and their prolonged duration were best explained by a poor stress response from Addison's disease coupled with exacerbation of early onset myasthenia gravis. This patient's case is not a typical reaction to interscalene nerve blocks, and thus preoperative testing would not be recommended for myasthenia gravis or Addison's disease without underlying suspicion. We describe this report to inform physicians to consider a workup for these diagnoses if a typical workup rules out all usual causes of complications from an interscalene block.
Skedros, John G; Kiser, Casey J; Mendenhall, Shaun D
This report describes a patient who had a series of daily interscalene nerve blocks to treat pain following a shoulder manipulation for postsurgical stiffness. She experienced acute respiratory compromise that persisted for many weeks. All typical and unusual causes of these symptoms were ruled out. Her treating pulmonologist theorized that the ipsilateral carotid body had been injured. However, it was subsequently determined that the constellation of symptoms and their prolonged duration were best explained by a poor stress response from Addison's disease coupled with exacerbation of early onset myasthenia gravis. This patient's case is not a typical reaction to interscalene nerve blocks, and thus preoperative testing would not be recommended for myasthenia gravis or Addison's disease without underlying suspicion. We describe this report to inform physicians to consider a workup for these diagnoses if a typical workup rules out all usual causes of complications from an interscalene block.
Werneck, L C
A study of 17 muscle biopsies from patients with myasthenia gravis was done, using freshfrozen section and histochemistry tecnics. It was found 15 abnormal muscle biopsies. The most common abnormality were small dark angular fibers, excess of lipids droplets outside the muscle membrane, changes in fiber size and type II fiber atrophy. These findings suggested denervation in 11 biopsies, type II fiber atrophy in 7, linfocyte infiltration in 4, fiber necrosis with fagocitosis in 1 and 2 were normal. Was noted a direct correlation between the disease duration and the severity of the histological abnormality. Two patients had tymoma. Congenital myasthenia gravis, rheumatoid arthritis, intersticial hypertrophic neuritis, Hashimoto tireoiditis and concomitance of myasthenic syndrome was found once in different patients.
Full Text Available Myasthenia gravis (MG patients visiting outpatient clinics frequently complain of headache. However, there have been few reports on the relation between chronic headache and myasthenia gravis (MG. We aimed to investigate whether MG symptoms affect the development or worsening of chronic headache. Among the 184 MG patients who were followed at the MG clinics, tension-type headache was observed in 71 (38.6% patients and 9 (4.9% complained of migraine. Twenty-five (13.6% complained that headache appeared or was exacerbated after the MG onset. The investigation into differences in the clinical characteristics of the MG patients showed that women tended to suffer from MG-associated headache more often than men. Logistic regression analyses revealed that female gender and mild ocular symptoms were independently predictive of headache associated with MG. Our results suggest that treatment of chronic headache should be required to improve the quality of life in MG patients.
Gao, Feng; Zhao, Xue; Zhang, Jing; Cui, Xinzheng; Zhang, Yingna; Li, Qianru; Liu, Pingping; Fang, Hua; Du, Ying; Zhang, Qingyong; Yang, Junhong; Zhang, Yunke
Myasthenia gravis (MG) occurs globally, and many studies have indicated that there are regional differences in epidemiology, biomarkers, and prognosis of MG. A total of 478 patients with MG who visited hospitals in the Henan Province between January 2010 and February 2014 were included. Age, gender, age at onset, serum antibody, thymus pathology, treatment information, and Myasthenia Gravis Foundation of America (MGFA) classification were assessed. Compared with previous reports from other areas, we found some differences in MG patients from Henan. The proportion of childhood MG in our study was lower than other reports of Oriental patients with MG. There seems to be an association between onset age, muscle involvement, and acetylcholine receptor antibody levels. Thymectomy improved the symptoms in 78.5% of thymectomy-treated patients. These differences are likely related to geographical, environmental, and ethnic differences. Understanding these differences will help us to define more specific treatment. © 2016 Wiley Periodicals, Inc.
Christlbauer, Monika; Schieberle, Peter
By application of the aroma extract dilution analysis (AEDA) on an aroma distillate isolated from a freshly prepared, stewed beef/vegetable gravy, 52 odor-active compounds were detected in the flavor dilution (FD) factor range of 4-4096. On the basis of high FD factors in combination with the results of the identification experiments, 3-(methylthio)propanal (cooked potato), 3-mercapto-2-methylpentan-1-ol (gravy-like), (E,E)-2,4-decadienal (deep-fried, fatty), 3-hydroxy-4,5-dimethyl-2(5H)-furanone (lovage-like), vanillin (vanilla-like), (E,E)-2,4-nonadienal (deep-fried), and (E)-2-undecenal (metallic) are suggested as key contributors to the aroma of the gravy. To get an insight into the role of the vegetables as sources of gravy odorants, a beef gravy was prepared without vegetables. The AEDA results revealed that, in particular, onions and leek are important sources of gravy aroma compounds, adding particularly the very potent, gravy-like smelling 3-mercapto-2-methylpentan-1-ol to the overall aroma profile. Further compounds that were clearly derived from the vegetables and, thus, are important modifiers of the overall aroma were 4-vinyl-2-methoxyphenol, (E)-beta-damascenone, beta-ionone, 2-isopropyl-3-methoxypyrazine, and 2-(sec-butyl)-3-methoxypyrazine. Interestingly, none of the key odorants detected in the gravy can be assumed to be formed from a reaction between beef and vegetable constituents. A comparison of the odorants in the beef/vegetable gravy with a gravy prepared according to the same procedure, but substituting beef by pork meat, indicated that most of the aroma compounds were identical-although different in FD factors-but the tallowy smelling 12-methyltridecanal was detected as key odorant only in the beef/vegetable gravy.
Pérez Guerra, Alfonso
Full Text Available All of the follow-up process is laid down with precision in a clear and impeccable report This makes it possible to prove that in the Process of Execution of Works, everything planned in the Safety Study and Developed in the subsequent Safety Plan is covered.
En un trabajo claro e impecable se plasma con precisión todo el proceso de seguimiento y control que permite acreditar que, en el Proceso de Ejecución de Obra, se cubre todo lo planeado en el Estudio de Seguridad y, a su vez, se ha desarrollado en el posterior Plan de Seguridad.
Soetje, G.; Brinkmann, G.; Striepling, E.; Engemann, R.
A 40-year old woman with an invasive thymoma and myasthenia gravis is described in this article. Chest-x-ray, CT and MRI of the mediastinum could not offer definite results on tumour malignancy. Radical surgical removal was the consequence. This revealed a tumour infiltration of the pleura and pericardium; hence an adjuvant irradiation must have been performed. Mestinon-treatment was afterwards gradually reduced. (orig.) [de
La miastènia gravis és una malaltia autoimmune i crónica, caracteritzada per una gran debilitat muscular. Un assaig clínic realitzat per la Unitat de Miastènia de l'Hospital General Universitari Vall d'Hebron durant cinc anys acaba de demostrar els beneficis d'incloure un fàrmac, el tacrolimus, per guarir aquesta malaltia.
Tomulescu, Victor; Sgarbura, Olivia; Stanescu, Codrut; Valciu, Crisanda; Campeanu, Ana; Herlea, Vlad; Popescu, Irinel
The aim of this study was to analyze the 10-year results of thoracoscopic unilateral extended thymectomy (TUET) performed in nontumoral myasthenia gravis according to the Myasthenia Gravis Foundation of America recommendations. Thoracoscopic unilateral extended thymectomy has the benefits of a minimally invasive approach. Previous data have shown promising midterm results but long-term results were lacking. Two hundred forty patients with nontumoral myasthenia gravis who underwent surgery between 1999 and 2009 were eligible for the study. The mean follow-up was of 67 months (range: 12-125), 134 patients completed follow-up assessments more than 60 months after TUET. There were 39 males (16.3%) and 201 females (83.7%), with an age range from 8 to 60 years. The mean preoperative disease duration was 21.5 months. All patients underwent preoperative steroid therapy. Anticholinesterase drugs were required for 123 patients (51.3%), and immunosuppressive drugs were required for 87 (36.3%) patients. The pathologic findings were as follows: normal thymus in 13 patients (5.5%), involuted thymus in 65 patients (27%), and hyperplastic thymus in 162 patients (67.5%). The average weight of the thymus was 110 ± 45 g. Ectopic thymic tissue was found in 147 patients (61.3%). There was no mortality, and morbidity consisted of 12 patients (5%). Complete stable remission was achieved in 61% of the patients, and the cumulative probability of achieving complete stable remission was 0.88 at 10 years. With zero mortality, low morbidity, and comparable long-term results to open surgery, TUET can be regarded as the best treatment option for patients undergoing surgery for myasthenia gravis.
Full Text Available BACKGROUND Von Hippel-Lindau disease (VHL is a rare autosomal dominant inherited disorder characterized by an increased risk of tumours in a number of locations (eyes, brain, adrenal gland, pancreas, liver, kidneys, or other areas of the body. It is caused by germline mutation in the VHL gene. The VHL gene is a tumour suppressor gene that has been identified on the short arm of chromosome 3. CASE REPORT We report a case of a 60 year-old female with the clinical diagnosis of VHL type 1 (cerebellar haemangioblastoma, pancreatic cysts with subsequent steatorrhoea, and bilateral renal carcinoma who developed weakness and fatigability of skeletal muscles, left lid ptosis, snarling expression and nasal timbre speech. Acetylcholine receptor antibodies were negative in serum, while the electrodiagnostic test demonstrated an alteration of neuromuscolar junction which was consistent with the diagnosis of myasthenia gravis. Contrast-enhanced TC scan of the anterior mediastinum was performed, which excluded thymus enlargement. VHL gene evaluation in this patient identified a new mutation (c279delC9 and polymorphism c291C>G. At present the patient still suffers from ataxia and dysmetria due to cerebellar involvement in VHL, while fatigue and lid ptosis improved after the treatment with oral pyridostigmine 60 mg tid. DISCUSSION AND CONCLUSIONS To our knowledge this is the first report of a case of VHL associated with myasthenia gravis without thymoma. A case of VHL associated with a form of myasthenia gravis related to thymoma has been recently reported. In our case the absence of acetylcholine receptor antibodies may suggest a genetic origin also for the myasthenia gravis.
Park, Mi-Jeong; Kim, Yun-A; Lee, Shin-Seok; Kim, Byeong-Chae; Kim, Myeong-Kyu; Cho, Ki-Hyun
We report two cases of systemic lupus erythematosus (SLE) in myasthenia gravis (MG) patients who had undergone thymectomy. SLE developed in the patients 3 months or 13 yr after thymectomy, and polyarthritis was the main clinical manifestation of SLE. Both patients fulfilled at least four of the revised criteria for the classification of SLE. In this report, we describe two postthymectomy lupus patients and perform a comparative review of previous cases.
Full Text Available This research was carried out to study the physical and chemical characteristics of pumpkin Javanese noodle gravy prepared in powder form. The finished product is categorized as an instant product as it only needs to be rehydrated with warm water (60°C before consumption. Five gravy formulations were developed with the incorporation of two types of flour (tapioca and corn flours as the thickening agent. Physical characteristics including colour and swelling properties of the final products were evaluated. For chemical analysis, ash, moisture, protein, crude fiber, fat and carbohydrate compositions were also determined. For physical analysis, all formulations showed similar colour appearance of the gravy powder including lightness (L, redness (a* and yellowness (b*. Pertaining swelling properties of the dried gravy, the water absorption index (WAI values were low (2.0–3.0g/g, while the water solubility index (WSI were moderate (33.0–50.0% when evaluated at both hot (85°C and cold (25°C water. In termn of chemical compositions, carbohydrate and fat content increased and decreased, respectively with the addition of tapioca and corn flours. The moisture content of the instant gravy increased with the increased of corn flour addition. There were differences in fiber and ash content in all formulations, but no specific trend was observed. Finally, protein content remained the same in all formulations. This current finding provided information of the gravy powder produced. Further analysis on the characteristics of the rehydrated pumpkin Javanese noodle gravy will provide a more complete picture of the finished product.
José Manuel Cerqueiro González
Full Text Available OBJETIVO: Determinar la repercusión de una consulta monográfica para pacientes con insuficiencia cardíaca crónica (ICC y comorbilidad asocia-da, dirigida por internistas y enfermería cualificada, sobre la calidad asis-tencial y los reingresos y la influencia en los ingresos de estos pacientes. Así como la influencia en los ingresos globales del hospital por IC.MATERIAL Y MÉTODOS: Estudio de cohortes retro y prospectivo de 169 pacientes con ICC, incluidos en dos consultas monográficas similares, pertenecientes a dos hospitales de diferente ámbito asistencial (comarcal y provincial. Se evalúan porcentaje de pacientes que recibe fármacos de 1º línea, la adherencia al seguimiento y se compara los ingresos por ICC realizados el año previo y posterior al seguimiento. Para conocer el impacto de estas consultas, en base a los datos de los centros, se analiza la evolución en el nº de ingresos totales y estancias medias por ICC (GRDs 127, 544 durante los años 2001-2009. Coincide el ecuador de este pe-riodo con la puesta en marcha de estas consultas.RESULTADOS: Los pacientes sometidos al seguimiento recibieron fárma-cos de 1º línea en un porcentaje elevado (97.8% para IECAS/ARA-II,y 88% para BB, con una adherencia al seguimiento muy alta (90% y con una reducción de los reingresos de hasta un 69%, con respecto al año previo. Desde los datos facilitados por ambos hospitales, se observa una disminución de la estancia hospitalaria global del servicio de Medicina interna por ICC de 3.5 días, un aumento de pacientes atendidos en Medi-cina interna y una disminución de los ingreso por ICC. CONCLUSIONES: El seguimiento por internistas y enfermería de los pacien-tes con ICC y comorbilidad, basado en una atención continuada e integral, en la información y en la disponibilidad, es un buen método no solo para disminuir reingresos hospitalarios , sino que además puede influir en la me-jora de las cifras globales de la hospitalización por
Diaz, Andres; Black, Edward; Dunning, Joel
A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was if thymectomy in non-thymomatous myasthenia gravis was of any benefit? Overall, 137 papers were found using the reported search, of which 16 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results of these papers are tabulated. The outcome variables were similar in all of the papers, including complete stable remission (CSR), pharmacological remission, age at presentation, gender, duration of symptoms, preoperative classification (Oosterhius, Osserman or myasthenia gravis Foundation of America (MGFA)), thymic pathology, preoperative medications (steroids, immunosuppressants), mortality and morbidity. We conclude that evidence-based reviews have shown that relative rates of thymectomy patients compared with non-thymectomy patients attaining outcome indicate that the former group of patients is more likely to achieve medication-free remission, become asymptomatic and clinically improve (54%, P benefit rate (BR), P benefits has not been established due to factors such as the confounding differences between myasthenia gravis patients receiving and not receiving thymectomy, the non-randomized nature of class II studies and the lack of Class I evidence to support its use. There is currently a randomized trial ongoing looking at thymectomy by sternotomy vs controls and the results are eagerly awaited.
Howard, James F; Freimer, Miriam; O'Brien, Fanny; Wang, Jing Jing; Collins, Stephen R; Kissel, John T
A phase 2 study of eculizumab for treating myasthenia gravis (MG) used the quantitative myasthenia gravis score (QMG) and myasthenia gravis activities of daily living profile (MG-ADL) to evaluate baseline disease severity and treatment response. Correlations were then analyzed between these assessments. Patients were given eculizumab or placebo during the first 16-week treatment period of the crossover study, with treatment assignments reversed for the second treatment period following a 5-week washout. QMG and MG-ADL scores at baseline and endpoint of each treatment period generated correlation coefficients for baseline status and treatment response during eculizumab therapy. Correlation strength between QMG and MG-ADL scores was higher for treatment response (R = 0.726; 95% confidence interval, 0.264-0.907; P = 0.0036) than for assessing baseline disease status (R = 0.552; 95% confidence interval, -0.022-0.839; P = 0.0495). MG-ADL may be more sensitive for assessing treatment response than point-in-time disease status. Muscle Nerve 56: 328-330, 2017. © 2016 Wiley Periodicals, Inc.
Giraldo, Lilliana María; Duque, Camilo; Uribe, Carlos Santiago; Hernández, Olga Helena
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. 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. 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.
Lee, Ha Neul; Kang, Hoon-Chul; Lee, Joon Soo; Kim, Heung Dong; Shin, Ha Young; Kim, Seung Min; Sunwoo, Il Nam; Lee, Young-Mock
Juvenile myasthenia gravis presents before 18 years of age with different characteristics according to racial background and pubertal development. The authors aimed to determine the clinical characteristics of children and adolescents of Korean ethnicity with myasthenia gravis, and evaluate the presentation and clinical outcomes according to the sex and onset age of the patients. The authors recruited 88 Korean juvenile myasthenia gravis patients between September 2005 and August 2015. Worse clinical severity from presentation, more aggressive treatment strategies, and worse final treatment outcomes were noted in girls with postpubertal onset than in the other patients. The symptoms were milder (pure ocular presentation in 96.6% [85/88]) and the disease course was more benign (94.3% [83/88]) in this study than in the literature. The homogenous racial background might have contributed to these results. These findings highlight the influence of pubertal development and the need for timely and appropriate active treatment, including thymectomy, to improve prognosis. © The Author(s) 2016.
Rahbek, Martin Amadeus; Mikkelsen, Erik Elgaard; Overgaard, Kristian; Vinge, Lotte; Andersen, Henning; Dalgas, Ulrik
It has not been established whether progressive resistance training (PRT) and aerobic training (AT) are feasible and efficient in myasthenia gravis (MG). Fifteen subjects with generalized MG (Myasthenia Gravis Foundation of America (MGFA) clinical classification II-IV) were randomly assigned to 20 training sessions during 8 weeks of either PRT or AT. Feasibility was evaluated based on adherence, drop-out rate, adverse events, and Quantitative Myasthenia Gravis (QMG) score. Twelve subjects (MGFA II, n = 11; MGFA III, n=1) completed the intervention with a mean adherence of 95 % ± 8. One dropout (PRT) could potentially be related to PRT. Both groups reported adverse events, including bulbar symptoms (n = 2) and increased fatigue (n = 3), but no change in QMG score was observed in either group. The PRT group showed increases in maximal strength and functional capacity. Eight weeks of moderate to high intensity AT and PRT were feasible for most patients with mild MG. Maximal strength and functional capacity increased in the PRT group. Muscle Nerve 56: 700-709, 2017. © 2017 Wiley Periodicals, Inc.
Jung Jin Lee
Full Text Available Aim. To estimate the clinical significance of anti-acetylcholine receptor antibody (anti-AChR-Ab levels in suspected ocular myasthenia gravis. Methods. In total, 144 patients complaining of fluctuating diplopia and ptosis were evaluated for serum levels of anti-acetylcholine receptor antibody and their medical charts were retrospectively reviewed. Subjects were classified into three groups: variable diplopia only, ptosis only, and both variable diplopia and ptosis. We investigated serum anti-AChR-Ab titer levels and performed thyroid autoantibody tests. Results. Patients’ chief complaints were diplopia (N=103, ptosis (N=12, and their concurrence (N=29. Abnormal anti-AChR-Ab was observed in 21 of 144 patients (14.1%. Between the three groups, mean age, number of seropositive patients, and mean anti-AChR-Ab level were not significantly different (P=0.224, 0.073, and 0.062, resp.. Overall, 27.5% of patients had abnormal thyroid autoantibodies. Conclusion. The sensitivity of anti-AChR-Ab was 14.1% in suspected ocular myasthenia gravis and seropositivity in myasthenia gravis patients showed a high correlation with the presence of thyroid autoantibodies.
Ali Hassan, FCPS
Full Text Available Although rare, the co-occurrence of myasthenia gravis and preeclampsia during pregnancy is responsible for considerable maternal and foetal morbidity and mortality. Both careful selection of medications and a multidisciplinary approach are required for treating such cases. This study presents a case report of a patient with a known history of generalized myasthenia gravis who presented with preeclampsia at 33 weeks' gestation. Subsequently, the patient developed recurrent seizures that necessitated the use of multiple medications, including phenytoin, valproic acid, levetiracetam, and propofol. Magnesium sulphate was not administered due to its blocking effect on calcium channels at the neuromuscular junction. The patient underwent a caesarean section under spinal anaesthesia and gave birth to a baby with intrauterine growth restriction (IUGR. Blood pressure control was achieved with the administration of methyldopa and parenteral hydralazine, an increased dose of pyridostigmine, and intravenous immunoglobulin therapy. The status of the patient's myasthenia gravis remained stable. This case serves to highlight the conflicts in the management of these two disorders and suggests strategies to resolve these conflicts in clinical management.
Full Text Available La gestión de las áreas protegidas en el actual contexto de cambio global requiere de aproximaciones que permitan caracterizar y seguir el funcionamiento de los ecosistemas. La teledetección ofrece herramientas para ello, ya que proporciona índices que informan reiteradamente y para grandes porciones de territorio, de los intercambios de materia y energía que tienen lugar entre la biota y la superficie terrestre. Considerando este principio, y la cada vez mayor disponibilidad de información satelital de calidad y facilidad de uso, desarrollamos junto con el Organismo Autónomo de Parques Nacionales de España (OAPN, un sistema de seguimiento que complementa a otras iniciativas desarrolladas por este organismo para informar del estado de conservación de los parques nacionales. El sistema, denominado REMOTE, se basa en el empleo de los índices de vegetación espectrales (IVs de uso más extendido (EVI -índice de vegetación mejorado- y NDVI -índice de vegetación normalizado-, derivados de la serie temporal de imágenes satelitales del sensor MODIS-Terra. El sistema permite avanzar en la identificación de las condiciones de referencia para la comprensión y predicción de las respuestas de los ecosistemas frente a perturbaciones e intervenciones, y de los cambios direccionales que éstos están experimentando (tendencias. Así mismo ayuda a la detección de anomalías recientes que alertan de cambios bruscos en el funcionamiento de los ecosistemas. REMOTE se caracteriza porque usa como indicadores tres atributos relacionados con las ganancias de carbono anuales (productividad primaria neta por parte del dosel vegetal, su estacionalidad y fenología. Además, está diseñado y programado con software libre y gratuito, lo que permitirá modificar o implementar mejoras en él fácilmente. Su implementación tiene como objetivo informar a los tomadores de decisiones y gestores de la Red de Parques Nacionales de España de la salud y estado
MarcosRaúl Martín Ruiz
Full Text Available Algunos aspectos del Programa Cubano de Prevención de Anemia Falciforme fueron analizados en Ciudad de La Habana durante 1992. Todas las gestantes detectadas con hemoglobinas anormales fueron citadas por telegrama para explicarles el significado del hallazgo. El 62,2 % acudió con prontitud a la citación. El 21,6 % vino más tardíamente y se requirieron otros mecanismos de comunicación. El 16,2 % no acudió. Todas las gestantes que asistieron, aceptaron recibir asesoramiento genético y se hizo el seguimiento de los casos. El 47,6 % concluyó el estudio del cónyuge en tiempo útil para optar por diagnóstico prenatal. Los resultados muestran la necesidad de reducir la edad gestacional en que se determina el riesgo de la pareja. Ello sería posible mediante el pesquisaje en la primera consulta prenatal, métodos más eficientes en comunicar con las gestantes positivas, y más agilidad en la realización de los análisis y en informar los resultados.Some aspects of the Cuban Programme for Prevention of Sickle Cell Anemia were analyze in Havana City during 1992. All pregnants detected with abnormal hemoglobins received an appointment by telegram to get an explanation about the significance of findings; 62,2 % attended promptly to the appointment; 21,6 % came later, making necessary the use of other mechanisms of communication with pregnants; and 16,2 % never attended. All pregnants who went to the appointment accepted genetic counseling and were followed up. Only 47,6 % concluded testing of partner early enough for consideration of prenatal diagnosis. The results showed the need to reduce gestational age in order to determine the risk of the couple. This is possible by screaning pregnants at the first prenatal visit, by using more efficient methods for contacting with positive women, and by doing the test and informing the results faster.
Zhu, Kai; Li, Jiaoxing; Huang, Xin; Xu, Wei; Liu, Weibin; Chen, Jiaxin; Chen, Pei; Feng, Huiyu
Ocular myasthenia gravis, an autoimmune disease, is characterized by extraocular muscle weakness. Myasthenia gravis is closely associated with the functional status of the thymus gland. The efficacy of thymectomy for non-thymomatous ocular myasthenia gravis remains controversial. Here, we present the first systematic review and meta-analysis of studies assessing the outcome of thymectomy in patients with non-thymomatous ocular myasthenia gravis and found that the pooled rate of complete stable remission was 0.5074 with considerable heterogeneity. Furthermore, subgroup analysis showed that the efficacy of thymectomy differed according to geographical location. Furthermore, thymectomy outcomes are better in children than they are in adults. Thymectomy clearly represents an effective treatment for patients with non-thymomatous ocular myasthenia gravis. However, more multicenter, randomized, controlled clinical trials are now required to confirm these conclusions.
Ferrer, Yiezenia Rosario
Las evaluaciones de impacto ambiental surgen con el fin de minimizar los efectos que la actividad humana produce sobre el entorno y corregirlos en la medida de lo posible. Moa (Holguín, Cuba), es una ciudad industrial en la cual la actividad minera ha causado grave daños sobre el entorno, por lo que existe en la actualidad una degradación ambiental importante en el área. Por lo que se hace necesaria la realización de estudios ambientales económicamente sustentables, que propicien una adecuada...
CANDELA ANDREU, NATIVIDAD
[es] El espectáculo consiste en el disparo o lanzamiento sucesivo de una serie de artificios pirotécnicos, de suelo y aéreos. Las características más significativas del material pirotécnico serán aportadas por la Empresa. En los Planes se reflejará: el día y la hora del acto y del montaje preparatorio. Los dispositivos previstos para el lanzamiento de los artificios pirotécnicos deberán quedar fijados, de tal forma que se imposibilite su movimiento por efecto del disparo, manteniendo en todo...
Salomé Muray Cases
Conclusión: La ecografía en el seguimiento de la maduración aporta ventajas frente al seguimiento clínico. Con nuestro abordaje del AV en prediálisis conseguimos que el 80% de nuestros pacientes inicien hemodiálisis mediante una FAV distal.
María de los Ángeles Peña Machado
Full Text Available Con el objetivo de caracterizar la reactogenicidad de la vacuna Antidiftérica-Antitetánica- Antipertusis (DTP cubana, desarrollada por el Instituto Finlay, se realizó un estudio utilizando dos métodos o fuentes de información diferentes y paralelos. La primera se obtuvo de un ensayo clínico (EC Fase IV, abierto, no controlado, en el que se incluyeron 57 lactantes. La segunda fuente de información se obtuvo de los reportes de los eventos temporalmente asociados a la vacunación (ETAV, recepcionados por la Unidad Coordinadora Nacional de Farmacovigilancia (UCNFv que incluyen a 86 lactantes que fueron atendidos por sospechas de ETAV. En el EC se aplicaron 167 dosis, los eventos adversos esperados e inesperados se observaron en un escaso número de lactantes, fueron mayoritariamente ligeros y autolimitados en el tiempo y su frecuencia de aparición se redujo en la 2da y 3ra dosis. El dolor fue el evento local más frecuente y la fiebre fue el evento general que más se presentó. No se reportaron vómitos y la anorexia, la somnolencia, y el llanto persistente aparecieron en un número limitado de sujetos vacunados.Se presentaron 12 eventos no esperados, solo en 2 de ellos se consideró que existía relación causal con la vacunación. Se reportó un evento adverso grave (niña hospitalizada por síndrome febril prolongado postvacunación, que se estudió y demostró que fue causado por una sepsis urinaria persistente provocada por una malformación vesico-ureteral. Los 86 reportes recepcionados por la UCNFv incluían un total de 141 ETAV, dentro de los síntomas locales, el eritema alcanzó un 9,30% y la induración un 5,81%, la fiebre fue un síntoma frecuente aunque no se especifica la temperatura corporal alcanzada. Aunque existen varias diferencias con relación a las condiciones en que se realiza la vigilancia de eventos adversos en un EC o en la práctica clínica habitual, los resultados aquí analizados nos confirman que la
Mohammad Etezad Razavi
Full Text Available Introduction: The aim of this study was to evaluate the efficacy of acupuncture therapy on relieving ocular symptoms in resistant Ocular Myasthenia Gravis (OMG. Case: In this pilot study, three patients with OMG were offered ten sessions of acupuncture therapy, twice weekly, while they received their routine medical treatment. Their symptoms included ptosis and diplopia. Subjective impression of changes was assessed based on Ocular-quantitative Myasthenia Gravis (QMG scoring before and after treatment sessions. Conclusion:All three patients enrolled in this study showed resolution of diplopia. Their subjective impression of symptoms was improved. However, the score of ptosis did not improve considerably. Acupuncture is another choice for reducing ocular symptoms besides conventional treatment in resistant Ocular Myasthenia Gravis (OMG. However, future randomized controlled trial studies are needed to approve this issue.
Igor I Bonnet
Full Text Available Se presenta un caso clínico poco frecuente de feocromocitoma bilateral, en el cual los estudios de diagnóstico por imagen, tanto de información estructural como funcional, constituyeron una fuente fundamental para su detección y seguimiento.We report a rare case of bilateral pheochromocytoma, in which imaging studies, both of structural and functional information, were a major source for its detection and follow-up.
M. P. Guillén-Paredes
éuticas en función de la clínica, localización y lesión asociada a la intususcepción, así como, su seguimiento y complicaciones. Pacientes y métodos: pacientes ingresados en el HGU Morales Meseguer (Murcia desde enero de 1995 hasta enero 2009, con diagnóstico de invaginación intestinal. Se recogieron datos demográficos, clínicos, exploraciones complementarias, diagnóstico presuntivo, tratamiento, seguimiento y complicaciones. Resultados: 14 pacientes (edad media 41,9 años, rango: 17-77, 7 varones y 7 mujeres, que debutaron principalmente con dolor abdominal. La exploración más fiable en el diagnóstico fue la tomografía computerizada, TC (8 diagnósticos, de 10 exploraciones. El diagnóstico preoperatorio se obtuvo en 12 casos, encontrando, invaginaciones ileocólicas en 8 casos (las más frecuentes, entéricas en 5 casos y colocólicas en 2, teniendo en cuenta que son 14 los pacientes y 15 las lesiones debido a la coexistencia de 2 invaginaciones en un mismo sujeto. La etiología de las intususcepciones es idiopática o secundaria a una lesión que hace de cabeza de invaginación. Según la naturaleza de dichas lesiones la causa de intususcepciones entéricas fue benigna en 3 casos y maligna en 2. De las ileocólicas, se repartieron equitativamente (4 benignas y 4 malignas; y de las colocólicas, sus lesiones fueron benignas (2 casos. Se realizó tratamiento conservador en 4 pacientes y quirúrgico en 10 (7 urgente. Con 5 hemicolectomías derechas, 3 resecciones de intestino delgado, 2 hemicolectomías izquierdas y una resección ileocecal. Las complicaciones quirúrgicas: 3 menores y 1 mayor (de etiología maligna y consecuente exitus. En los pacientes con manejo conservador desapareció la lesión entre 3 días y 6 semanas. Se siguieron durante 28,25 meses de media (rango 5-72 meses. Conclusiones: para diagnosticar las intususcepciones es importante una adecuada técnica de imagen, recomendablemente TC. Abogamos por un tratamiento conservador en aquellos
Feijoo Calles, Daniela; Moreno Royo, Lucrecia; Salar Ibáñez, Luis
La enfermedad pulmonar obstructiva crónica (EPOC) es una patología caracterizada por una reducción persistente del flujo de aire. Esta enfermedad pulmonar es progresiva y potencialmente mortal. Suele causar disnea y las exacerbaciones y comorbilidades asociadas hacen que el pronóstico sea peor. Se presenta un caso de seguimiento farmacoterapéutico realizado siguiendo la metodología del Foro de Atención Farmacéutica en Farmacia Comunitaria en el Aula de Práctica Farmacéutica “Paco Martínez...
Carlos A Martínez Blanco
Full Text Available Se reporta un estudio de cohorte analítico para evaluar factores pronósticos en 34 pacientes con timoma y miastenia gravis, a los cuales se les realizó timectomía, entre enero de 1984 y diciembre de 1998, 22 femeninos y 12 masculinos, con un promedio de edad de 49,7 años. De acuerdo con la clasificación clínica de Masaoka, 14 pertenecieron al estadio I, 2 al II, 12 al III y 6 al IV. Histológicamente 17 fueron mixtos, 9 corticales, 5 medulares y 3 carcinomas tímicos bien diferenciados. Según la clasificación de Osserman 5 enfermos se encontraban en el grupo IIa, 23 en el IIb y 6 en el IV. La vía de acceso fue la esternotomía media. Los pacientes con tumores invasivos recibieron radioterapia y poliquimioterapia adyuvante. El índice de remisión de los síntomas miasténicos al año fue de 53,1%. Hubo 3 fallecidos en el período posoperatorio y 7 tardíamente. El timoma cortical y, en general, los tumores invasivos fueron los de peores resultados. Los factores que más se relacionaron con el pronóstico por su influencia sobre el intervalo libre de enfermedad y la supervivencia a largo plazo, fueron el estadio clínico del tumor, el tipo hístico y el tipo de miasteniaThis paper presents an analytical cohort study to evaluate prognostic factors in 34 patients with thymona and myasthemia gravis whom thymectomy from January 1984 to December 1998. Twenty two were females and 12 males aged 49,7 years of age as average. According to Masaoka´s clinical classification, 14 patients were at stage I; two at stage II, 12 at stage III and 6 at stage IV. From the histological viewpoint, 17 were mixed thymona, 9 cortical thymonas, 5 medullar thymonas and 3 well-differentiated thymic carcinomas. According to Osswerman´s classification, 5 patients were included in group IIa, 23 in group IIb and 6 in group IV. The access pathway was median sternotomy. Remission index of myasthemic symptoms after a year was 53.1%. there were 3 deaths in the
Beuchat, L R; Clavero, M R; Jaquette, C B
The presence of psychrotrophic enterotoxigenic Bacillus cereus in ready-to-serve meats and meat products that have not been subjected to sterilization treatment is a public health concern. A study was undertaken to determine the survival, growth, and diarrheal enterotoxin production characteristics of four strains of psychrotrophic B. cereus in brain heart infusion (BHI) broth and beef gravy as affected by temperature and supplementation with nisin. A portion of unheated vegetative cells from 24-h BHI broth cultures was sensitive to nisin as evidenced by an inability to form colonies on BHI agar containing 10 micrograms of nisin/ml. Heat-stressed cells exhibited increased sensitivity to nisin. At concentrations as low as 1 microgram/ml, nisin was lethal to B. cereus, the effect being more pronounced in BHI broth than in beef gravy. The inhibitory effect of nisin (1 microgram/ml) was greater on vegetative cells than on spores inoculated into beef gravy and was more pronounced at 8 degrees C than at 15 degrees C. Nisin, at a concentration of 5 or 50 micrograms/ml, inhibited growth in gravy inoculated with vegetative cells and stored at 8 or 15 degrees C, respectively, for 14 days. Growth of vegetative cells and spores of B. cereus after an initial period of inhibition is attributed to loss of activity of nisin. One of two test strains produced diarrheal enterotoxin in gravy stored at 8 or 15 degrees C within 9 or 3 days, respectively. Enterotoxin production was inhibited in gravy supplemented with 1 microgram of nisin/ml and stored at 8 degrees C for 14 days; 5 micrograms of nisin/ml was required for inhibition at 15 degrees C. Enterotoxin was not detected in gravy in which less than 5.85 log10 CFU of B. cereus/ml had grown. Results indicate that as little as 1 microgram of nisin/ml may be effective in inhibiting or retarding growth of and diarrheal enterotoxin production by vegetative cells and spores of psychrotrophic B. cereus in beef gravy at 8 degrees C, a
Torres, C.; Redondas, Alberto; Cuevas Agulló, Emilio; Yela, Margarita; Araujo, Jorge; Ochoa, Hector; Deferrari, Guillermo; Díaz González, Juan Pedro
Ponencia presentada en: 1er Encuentro sobre Meteorología y Atmósfera de Canarias, celebrado en el Puerto de la Cruz, los días 12,13 y 14 de noviembre de 2003. El encuentro estuvo organizado por el Centro Meteorológico Territorial en Canarias Occidental, con la colaboración del Observatorio Atmosférico de Izaña y del Grupo de Física de la Atmósfera de la Facultad de Física (Universidad de La Laguna) En los últimos años ha aumentado el interés por conocer en detalle la evolución del ozono ...
Full Text Available El seguimiento de los ecosistemas con imágenes procedentes del sensor MODIS (Moderate Resolution Imaging Spectroradiometer, espectroradiómetro de imágenes de resolución media está actualmente muy extendido tanto en tareas de investigación como de gestión. Los índices de vegetación NDVI (Normalized Difference Vegetation Index, índice de vegetación de la diferencia normalizada y EVI (Enhanced Vegetation Index, índice de vegetación mejorado son ampliamente usados para la caracterización del funcionamiento ecosistémico. Ambos índices se emplean como estimadores lineales de la fracción de radiación fotosintéticamente activa interceptada por la vegetación (fAPAR, el principal control de la producción primaria. A pesar de sus ventajas, las imágenes de índices de vegetación no están libres de errores. El producto índices de vegetación MOD13Q1 proporciona una capa QA (Quality assessment,evaluación de la calidad que informa sobre la calidad asociada a cada píxel. Esta información representa una gran ventaja para el usuario, al permitir filtrar aquellos datos que puedan inducir a errores al verse alterados por la presencia de aerosoles, nubes, nieve o sombras. Sin embargo, la realización de un filtrado homogéneo a lo largo de una gran región puede ocasionar la pérdida sistemática de información en determinadas zonas o épocas del año, introduciendo así un sesgo espacial o en la serie temporal. Esta situación puede ser especialmente crítica en regiones con alta heterogeneidad ambiental, como el Sureste Ibérico. En este trabajo evaluamos el efecto que el filtrado de calidad tiene sobre la información espacial y temporal de la base de datos del EVI en el periodo 2001-2010. Los esultados, expresados en porcentaje de información perdida (filtrada y como efecto de estas pérdidas sobre los valores del EVI, indican que mientras que las áreas de menor altitud no se ven afectadas por el filtrado, las regiones de alta
Gonzálo Vásquez Palacio
Full Text Available Tanto la citogenética convencional como la molecular en el estudio de los desórdenes hematológicos constituyen metodologías fundamentales para el diagnóstico y pronóstico de la enfermedad, así como para el seguimiento de la quimioterapia o transplantes de donantes de sexo diferente.
Conclusión: El grupo DBT con CRM presentó menor sobrevida alejada que el grupo no-DBT. El grupo de pacientes con lechos coronarios malos presentó mayor mortalidad hospitalaria y mayor incidencia de readmisión hospitalaria en el seguimiento a corto plazo.
Pavón Pedraza, María Teresa
[CASTELLÀ] Proyecto que se desarrolla en la empresa Vector. Este nuevo módulo permitirá a los socios de los centros deportivos tener un seguimiento de su estado físico. [ANGLÈS] Project that is developed in the company Vector. This new module allows physical monitoring of the partners.
Evaluation of the respiratory function in myasthenia gravis: an important tool for clinical feature and diagnosis of the disease Avaliação da função respiratória na miastenia gravis: importância na caracterização clínica e no diagnóstico da doença
Paulo A. P. Saraiva
Full Text Available Myasthenic gravis may affect both inspiratory and expiratory muscles. Respiratory involvement occurred in almost all patients with myasthenia gravis in all clinical forms of the disease: 332 lung function tests done in 324 myasthenic patients without respiratory symptoms (age 34.6 ± 18.3 years were examined. Lung volumes analysis showed that all the patients of both sexes with generalized or ocular myasthenia gravis showed "myasthenic pattern". Male patients with "ocular" form only presented the "myasthenic pattern" with lung impairment and had, from the lung function point of view, a more benign behaviour. Female patients with the "ocular" form exhibited a behaviour of respiratory variables similar to that of the generalized form. It was not observed modification of the variables that suggested obstruction of the higher airways. The "myasthenic pattern" was rarely observed in other neuromuscular diseases, except in patients with laryngeal stenosis.O comprometimento respiratório é fator limitante na evolução clinica da miastenia gravis (MG e as formas clínicas mais graves apresentavam acometimento bulbar e respiratório. Para avaliar a reserva respiratória foram examinados em 324 pacientes com MG (forma ocular 62, generalizada 246 e timomatosa 16 as seguintes variáveis da prova de função pulmonar (PFP: capacidade vital forçada (FVC; volume onde o fluxo expiratório é igual a 1 litro por segundo (VF=1; volume expiratório forçado no primeiro segundo (FEV1; fluxo expiratório forçado medido entre 0,2 e 1,2 litros (FEF; fluxo médio expiratório forçado, medido entre 25 e 75% da FVC (FMF; intervalo de tempo entre 25 e 75% da FVC (FMFT; tempo médio de trânsito na expiração forçada (MTT; capacidade pulmonar total (TLC; volume residual (RV; curva fluxo-volume para pesquisa do "padrão miastênico". A análise estatística realizada foi: "t pareado" entre paciente e seu padrão e "t não pareado" entre grupos. Conclusões: Todos os
Pacheco Ramirez, Jesus Horacio; Anaya Perez, Maria Elena; Benitez Baltazar, Victor Hugo [Universidad de onora, Hermosillo, Sonora (Mexico)]. E-mail: email@example.com; firstname.lastname@example.org; email@example.com
This work shows how artificial vision feedback can be applied to control systems. The control is applied to a solar panel in order to track the sun position. The algorithms to calculate the position of the sun and the image processing are developed in LabView. The responses obtained from the control show that it is possible to use vision for a control scheme in closed loop. [Spanish] El presente trabajo muestra la manera en la cual un sistema de control puede ser retroalimentado mediante vision artificial. El control es aplicado en un panel solar para realizar el seguimiento del sol a lo largo del dia. Los algoritmos para calcular la posicion del sol y para el tratamiento de la imagen fueron desarrollados en LabView. Las respuestas obtenidas del control muestran que es posible utilizar vision para un esquema de control en lazo cerrado.
Chigurupati, Keerthi; Gadhinglajkar, Shrinivas; Sreedhar, Rupa; Nair, Muraleedharan; Unnikrishnan, Madathipat; Pillai, Manjusha
To determine the criteria for postoperative mechanical ventilation after thymectomy in patients with Myasthenia Gravis. Retrospective study. Teritiary care centre. 77 Myasthenia gravis patients operated for thymectomy were studied. After obtaining clearance from Institutional ethics committee, medical records of 77 patients with MG, who were operated for thymectomy between January 2005 and December 2015 were reviewed in a retrospective manner. Perioperative variables collected from the patient records were demographic data, duration of the disease, Osserman and Genkin classification, Anti-acetylcholine antibody (AChR) positivity, preoperative daily dose of drug, history of preoperative myasthenic crisis, preoperative vital capacity, technique of anesthesia, drugs used for anesthesia, perioperative complications, and duration of postoperative mechanical ventilation. The patients were divided into two groups, group I and group II consisting of those who required postoperative ventilation for 300 minutes, respectively. The determinants of prolonged postoperative ventilation were studied. The requirement of mechanical ventilation was higher in patients with higher Osserman's grade of myasthenia gravis. Duration of the disease had no effect on the duration of mechanical ventilation in myasthenic patients post thymectomy (p = 0.89). The patients with a preoperative history of myasthenic crisis had a requirement for prolonged mechanical ventilation (p=0.03). Patients with preoperative vital capacity mechanical ventilation with p values mechanical ventilation (p=0.026). Preoperative dose of pyridostigmine and the choice of continuation or discontinuation of antcholinesterases on the day of surgery had no influence on the duration of mechanical ventilation (p value of 0.19 and 0.36 respectively). Epidural analgesia intra and postoperatively significantly reduced the requirement of mechanical ventilation (p=0.006). The predictors of postoperative ventilation in myasthenic
Druschky, K.F.; Stadler, H.W.; Daun, H.; Erlangen-Nuernberg Univ., Erlangen
Hyperplasia of the thymus gland is observed in 65% of all patients with myasthenia gravis, while the incidence of thymus tumor is reported to be 8.5-28%. Conventional radiological techniques provide little information in the diagnosis of mediastinal lesions. Even a clearly developed thymus tumor can escape clinical detection. Since March 1978 thoracic computer tomography has been performed in addition to X-rays of the chest in a series of 19 patients with myasthenia ravis, 10 women and 9 men ranging in age from 15-71 years and in 3 patients with suspected thymomas but without myasthenia gravis. These examinations were carried out with a Somatom II (Siemens) since September 1979. On the average 15-20 scans were made at the level of the upper two-third of the sternum. The chest X-rays in 2 planes revealed signs of a thymus tumor in 3 female patients. Thoracic computer tomography showed definite signs of a space-occupying lesion in the anterior mediastinum in 11 cases. At thymectomy 6 patients were found to have hyperplasia of the thymus, 2 patients had a benign thymoma and 3 patients a malignant thymoma. In 6 cases computer tomography showed only slight changes and in 5 patients no pathological findings could be demonstrated in the thymus gland. Thoracic computer tomography is a relatively harmless diagnostic measure without any risk for the patient. It has a high resolution and great accuracy in the evaluation of the thymus gland and is therefore the method of choice for the diagnosis of patients with myasthenia gravis. (orig.) [de
Full Text Available JUSTIFICATIVA E OBJETIVOS: Pacientes com doenças neuromusculares, como Miastenia Gravis, respondem de maneira anormal aos anestésicos, conforme a técnica e as drogas administradas. O objetivo deste relato é mostrar um caso de paciente portadora de Miastenia Gravis, submetida a timectomia sob anestesia venosa total com propofol e remifentanil. RELATO DO CASO: Paciente feminina, 52 anos, 72 kg, com história de Miastenia Gravis submetida a timectomia transesternal, sob anestesia venosa total, com o uso de propofol em infusão alvo controlada (3 g.ml-1 e remifentanil contínuo (0,3 µg.kg-1.min-1. Para a intubação traqueal foi utilizada succinilcolina (50 mg, sendo realizada sem dificuldade. Com a finalidade de realizar a analgesia pós-operatória, foram utilizados tramadol (50 mg, cetoprofeno (100 mg e dipirona (1 g, no per-operatório. Após a cirurgia, a infusão de propofol e remifentanil foi encerrada e, 15 minutos depois, a paciente foi extubada. A paciente apresentava-se eupnéica, acordada, sem dor, movimentando membros, com freqüência respiratória de 14 mpm e mantendo saturação de oxigênio de 97%. Permaneceu com cateter nasal de O2 a 2 L.min-1 na UTI intermediária, durante 36 horas, e recebeu alta hospitalar no 4º dia do pós-operatório. CONCLUSÕES: A anestesia venosa total, com agentes anestésicos de curta duração e sem metabólitos ativos, favoreceu a recuperação e a extubação precoce da paciente
Chae, Chang-Suk; Kwon, Ho-Keun; Hwang, Ji-Sun; Kim, Jung-Eun; Im, Sin-Hyeog
Probiotics are live bacteria that confer health benefits to the host physiology. Although protective role of probiotics have been reported in diverse diseases, no information is available whether probiotics can modulate neuromuscular immune disorders. We have recently demonstrated that IRT5 probiotics, a mixture of 5 probiotics, could suppress diverse experimental disorders in mice model. In this study we further investigated whether IRT5 probiotics could modulate the progression of experimental autoimmune myasthenia gravis (EAMG). Myasthenia gravis (MG) is a T cell dependent antibody mediated autoimmune disorder in which acetylcholine receptor (AChR) at the neuromuscular junction is the major auto-antigen. Oral administration of IRT5 probiotics significantly reduced clinical symptoms of EAMG such as weight loss, body trembling and grip strength. Prophylactic effect of IRT5 probiotics on EMAG is mediated by down-regulation of effector function of AChR-reactive T cells and B cells. Administration of IRT5 probiotics decreased AChR-reactive lymphocyte proliferation, anti-AChR reactive IgG levels and inflammatory cytokine levels such as IFN-γ, TNF-α, IL-6 and IL-17. Down-regulation of inflammatory mediators in AChR-reactive lymphocytes by IRT5 probiotics is mediated by the generation of regulatory dendritic cells (rDCs) that express increased levels of IL-10, TGF-β, arginase 1 and aldh1a2. Furthermore, DCs isolated from IRT5 probiotics-fed group effectively converted CD4+ T cells into CD4+Foxp3+ regulatory T cells compared with control DCs. Our data suggest that IRT5 probiotics could be applicable to modulate antibody mediated autoimmune diseases including myasthenia gravis. PMID:23284891
Phillips, William D.; Vincent, Angela
Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (Ig)G1 and IgG3 antibodies to the acetylcholine receptor (AChR). They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK), or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4), are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms. PMID:27408701
Druschky, K.F.; Stadler, H.W.; Daun, H.
Hyperplasia of the thymus gland is observed in 65% of all patients with myasthenia gravis, while the incidence of thymus tumor is reported to be 8.5-28%. Conventional radiological techniques provide little information in the diagnosis of mediastinal lesions. Even a clearly developed thymus tumor can escape clinical detection. Since March 1978 thoracic computer tomography has been performed in addition to X-rays of the chest in a series of 19 patients with myasthenia ravis, 10 women and 9 men ranging in age from 15-71 years and in 3 patients with suspected thymomas but without myasthenia gravis. These examinations were carried out with a Somatom II (Siemens) since September 1979. On the average 15-20 scans were made at the level of the upper two-third of the sternum. The chest X-rays in 2 planes revealed signs of a thymus tumor in 3 female patients. Thoracic computer tomography showed definite signs of a space-occupying lesion in the anterior mediastinum in 11 cases. At thymectomy 6 patients were found to have hyperplasia of the thymus, 2 patients had a benign thymoma and 3 patients a malignant thymoma. In 6 cases computer tomography showed only slight changes and in 5 patients no pathological findings could be demonstrated in the thymus gland. Thoracic computer tomography is a relatively harmless diagnostic measure without any risk for the patient. It has a high resolution and great accuracy in the evaluation of the thymus gland and is therefore the method of choice for the diagnosis of patients with myasthenia gravis.
Phillips, William D; Vincent, Angela
Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ) caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (Ig)G1 and IgG3 antibodies to the acetylcholine receptor (AChR). They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK), or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4), are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms.
William D. Phillips
Full Text Available Myasthenia gravis is an autoimmune disease of the neuromuscular junction (NMJ caused by antibodies that attack components of the postsynaptic membrane, impair neuromuscular transmission, and lead to weakness and fatigue of skeletal muscle. This can be generalised or localised to certain muscle groups, and involvement of the bulbar and respiratory muscles can be life threatening. The pathogenesis of myasthenia gravis depends upon the target and isotype of the autoantibodies. Most cases are caused by immunoglobulin (IgG1 and IgG3 antibodies to the acetylcholine receptor (AChR. They produce complement-mediated damage and increase the rate of AChR turnover, both mechanisms causing loss of AChR from the postsynaptic membrane. The thymus gland is involved in many patients, and there are experimental and genetic approaches to understand the failure of immune tolerance to the AChR. In a proportion of those patients without AChR antibodies, antibodies to muscle-specific kinase (MuSK, or related proteins such as agrin and low-density lipoprotein receptor-related protein 4 (LRP4, are present. MuSK antibodies are predominantly IgG4 and cause disassembly of the neuromuscular junction by disrupting the physiological function of MuSK in synapse maintenance and adaptation. Here we discuss how knowledge of neuromuscular junction structure and function has fed into understanding the mechanisms of AChR and MuSK antibodies. Myasthenia gravis remains a paradigm for autoantibody-mediated conditions and these observations show how much there is still to learn about synaptic function and pathological mechanisms.
Li, Yan; Tu, Zhidan; Qian, Shiguang; Fung, John J; Markowitz, Sanford D; Kusner, Linda L; Kaminski, Henry J; Lu, Lina; Lin, Feng
We recently demonstrated that hepatic stellate cells induce the differentiation of myeloid-derived suppressor cells (MDSCs) from myeloid progenitors. In this study, we found that adoptive transfer of these MDSCs effectively reversed disease progression in experimental autoimmune myasthenia gravis (EAMG), a T cell-dependent and B cell-mediated model for myasthenia gravis. In addition to ameliorated disease severity, MDSC-treated EAMG mice showed suppressed acetylcholine receptor (AChR)-specific T cell responses, decreased levels of serum anti-AChR IgGs, and reduced complement activation at the neuromuscular junctions. Incubating MDSCs with B cells activated by anti-IgM or anti-CD40 Abs inhibited the proliferation of these in vitro-activated B cells. Administering MDSCs into mice immunized with a T cell-independent Ag inhibited the Ag-specific Ab production in vivo. MDSCs directly inhibit B cells through multiple mechanisms, including PGE2, inducible NO synthase, and arginase. Interestingly, MDSC treatment in EAMG mice does not appear to significantly inhibit their immune response to a nonrelevant Ag, OVA. These results demonstrated that hepatic stellate cell-induced MDSCs concurrently suppress both T and B cell autoimmunity, leading to effective treatment of established EAMG, and that the MDSCs inhibit AChR-specific immune responses at least partially in an Ag-specific manner. These data suggest that MDSCs could be further developed as a novel approach to treating myasthenia gravis and, even more broadly, other diseases in which T and B cells are involved in pathogenesis. Copyright © 2014 by The American Association of Immunologists, Inc.
Surkiewicz, B F; Harris, M E; Johnston, R W
During visits to 34 federally inspected establishments producing frozen meat and gravy, 541 production line samples and 535 finished product units were collected for bacteriological analyses. It was found that more than 70% of the sets of finished product (10 units/set) produced under good manufacturing practices had: (i) four or fewer coliform-positive units, (ii) two or fewer Escherichia coli-positive units, (iii) three or fewer Staphylococcus aureus-positive units, and (iv) an aerobic plate count of fewer than 50,000/g (geometric mean of 10 units). All finished product units were negative for salmonellae.
Leandro M. Goldstraj
Full Text Available IntroducciónEn estudios epidemiológicos previos se observó que poblaciones con igual riesgo por suma de factores clásicos poseían una incidencia y tipos de expresión de enfermedades cardiovasculares diferentes.ObjetivoDeterminar la incidencia de MACE, compuesta por infarto agudo de miocardio, angina inestable, accidente cerebrovascular, angioplastia transluminal coronaria, cirugía de revascularización miocárdica o muerte cardiovascular, y su asociación con los factores de riesgo en una población cerrada. Material y métodosEl presente es un estudio de cohorte prospectivo en el que fueron relevados hombres, de profesión policías, durante el año 1997. Luego se ingresaron en la base de datos hospitalaria aquellos que presentaron eventos en los 10 años posteriores. Se empleó la prueba de la t de Student para variables cuantitativas y regresión logística para las cualitativas. ResultadosSe incluyeron 2.379 personas de sexo masculino. La edad promedio fue de 39,5 ± 9,25 años. La prevalencia de factores de riesgo conocidos por las personas fue: HTA 11,1%, dislipidemia 20,3%, diabetes 2,4%, tabaquismo 43,3%. Noventa pacientes presentaron algún tipo de MACE. El odds ratio para diabetes fue 4,54 (IC 95% 2,1-9,81, HTA 2,3 (IC 95% 1,38-3,85, dislipidemia 2,74 (IC 95% 1,77-4,25 y para tabaquismo 1,48 (IC 95% 0,97-2,28. La edad, el LDL, el fibrinógeno y las horas trabajo diarias mostraron asociaciones significativas. El área bajo la curva ROC del puntaje de Framingham fue de 0,72 y para el puntaje europeo fue de 0,71. ConclusiónLos factores de riesgo de enfermedad cardiovascular clásicos tuvieron una importancia similar a los ya comunicados por los estudios epidemiológicos; es importante destacar algunos no clásicos como el fibrinógeno. Los puntajes de riesgo fueron predictores regulares en esta población. Las características especiales de la población analizada permiten la ejecución de programas de prevención acordes.
Huerga López, Sandra
El proyecto del centro comercial que os presento a continuación es un proyecto real en el cual hay partes que las realicé durante mi convenio universidad-empresa (considerando esta parte como un practicum), otra parte que la realicé solo para el Trabajo Final de Grado y la última parte de documentación aportada por la empresa (El segundo cuaderno). Nos centraremos en las dos primeras partes. Los planos del proyecto ejecutivo los realicé en la empresa con la supervisión del ingeniero....
Pedro Fuentes Durá
Full Text Available La costa este de España sufre periódicamente episodios de proliferación masiva de algas, que provocan graves perjuicios para los sectores turístico y pesquero, e incluso suponen un riesgo sanitario para la población. Este trabajo muestra un método que permite la detección de variaciones significativas en el estado del ecosistema, como cambios en las concentraciones de nutrientes y densidad de fitoplancton. Se expone el uso de gráficos de control estadístico de procesos basados en componentes principales (PC de las variables que se miden regularmente en estas playas. Los PC encontrados facilitan la interpretación de las razones por las que se producen estos episodios. Por otra parte, los gráficos de control propuestos tienen la capacidad de detectar situaciones anómalas que favorecen las proliferaciones masivas, permitiendo un eficaz control medioambiental en estas playas.
La costa este de España sufre periódicamente episodios de proliferación masiva de algas, que provocan graves perjuicios para los sectores turístico y pesquero, e incluso suponen un riesgo sanitario para la población. Este trabajo muestra un método que permite la detección de variaciones significativas en el estado del ecosistema, como cambios en las concentraciones de nutrientes y densidad de fitoplancton. Se expone el uso de gráficos de control estadístico de procesos basados en componentes principales (PC de las variables que se miden regularmente en estas playas. Los PC encontrados facilitan la interpretación de las razones por las que se producen estos episodios. Por otra parte, los gráficos de control propuestos tienen la capacidad de detectar situaciones anómalas que favorecen las proliferaciones masivas, permitiendo un eficaz control medioambiental en estas playas.
José Teotonio de Oliveira
Full Text Available Durante o período de 1971 a 1993, 52 miastênicos sem timoma (7 homens e 45 mulheres foram timectomizados por via esternal. Os resultados foram classificados em remissão e não-remissão . A taxa de remissão foi 48%. No grupo de remissões havia 5 homens e 20 mulheres. O tempo de seguimento foi de 5,5 anos em ambos os grupos. Os pacientes foram classificados clinicamente segundo a classificação de Osserman. De 16 pacientes na categoria II A, 11 entraram em remissão; de 36 pacientes nas categorias II B e III, 14 entraram em remissão. O tempo de duração de doença foi de 1,8 e 4,3 anos nos grupos de remissões e não-remissões, respectivamente. Todos os pacientes que entraram em remissão tinham menos de quatro anos de doença. Dos 43 pacientes com menos de quatro anos de doença, 42% não apresentaram remissão significando que um tempo de doença de menos de quatro anos não é indicador de remissão. Nossos dados indicam um melhor prognóstico quando a timectomia é feita nos primeiros quatro anos de doença.
Baccus-Taylor, G S H; Falloon, O C; Henry, N
(i) To study the effects of cold shock on Escherichia coli O157:H7 cells. (ii) To determine if cold-shocked E. coli O157:H7 cells at stationary and exponential phases are more pressure-resistant than their non-cold-shocked counterparts. (iii) To investigate the baro-protective role of growth media (0·1% peptone water, beef gravy and ground beef). Quantitative estimates of lethality and sublethal injury were made using the differential plating method. There were no significant differences (P > 0·05) in the number of cells killed; cold-shocked or non-cold-shocked. Cells grown in ground beef (stationary and exponential phases) experienced lowest death compared with peptone water and beef gravy. Cold-shock treatment increased the sublethal injury to cells cultured in peptone water (stationary and exponential phases) and ground beef (exponential phase), but decreased the sublethal injury to cells in beef gravy (stationary phase). Cold shock did not confer greater resistance to stationary or exponential phase cells pressurized in peptone water, beef gravy or ground beef. Ground beef had the greatest baro-protective effect. Real food systems should be used in establishing food safety parameters for high-pressure treatments; micro-organisms are less resistant in model food systems, the use of which may underestimate the organisms' resistance. © 2015 The Society for Applied Microbiology.
Maneh, Nidain; Apetse, Kossivi; Diatewa, Bénédicte Marèbe; Domingo, Sidik Abou-Bakr; Agba, Aidé Isabelle; Ayena, Koffi Didier; Balogou, Koffi Agnon; Balo, Komi Patrice
Myasthenia gravis is a rare acquired autoimmune pathology causing neuromuscular transmission impairment. Juvenile onset of myasthenia gravis is often characterized by ocular involvement. We report two cases of ocular juvenile myasthenia gravis (JMG) in two siblings. They were two young girls, XA and XB, aged 11 and 9 years, of Malian origin, residing in Togo, born from first-degree of consanguinity presenting to Ophthalmology due to progressive decrease in visual acuity. XA showed visual acuity 8/10 on both eyes while XB showed improvement in visual acuity from 3/10 to 7/10 using a pinhole occluder, suggesting ametropia. XA had a 2-year history of bilateral ptosis lifting the upper eyelid of 7 mm, while XB had a 3-year history of bilateral ptosis with no lifting of the upper eyelid. Ice pack test was strongly positive in both patients. They had Cogan's lid twitch with paresis of the oculomotor nerve without diplopia. The dosage of acetylcholine receptor autoantibodies was normal. The diagnosis of JMG associated with ametropia was suspected. Ametropia was corrected by glasses and a specific treatment with pyridostigmine was initiated, but both patients were lost to follow-up. Autoimmune myasthenia gravis with inaugural ophthalmologic manifestation is rare but it can occur among children living in sub-Saharan Africa. Studies should be conducted to establish the features of this disease.
Kanatli, Irem; Akkaya, Bahar; Uysal, Hilmi; Kahraman, Sevim; Sanlioglu, Ahter Dilsad
Myasthenia Gravis is an autoantibody-mediated, neuromuscular junction disease, and is usually associated with thymic abnormalities presented as thymic tumors (~10%) or hyperplastic thymus (~65%). The exact role of thymus in Myasthenia Gravis development is not clear, yet many patients benefit from thymectomy. The apoptotic ligand TNF-Related Apoptosis-Inducing Ligand is thought to be involved in the regulation of thymocyte counts, although conflicting results are reported. We investigated differential expression profiles of TNF-Related Apoptosis-Inducing Ligand and its transmembrane receptors, Nuclear Factor-kB activation status, and apoptotic cell counts in healthy thymic tissue and pathological thymus from Myasthenia Gravis patients. All tissues expressed TNF-Related Apoptosis-Inducing Ligand and its receptors, with hyperplastic tissue having the highest expression levels of death receptors DR4 and DR5. No detectable Nuclear Factor-kB activation, at least via the canonical Protein Kinase A-mediated p65 Ser276 phosphorylation, was evident in any of the tissues studied. Apoptotic cell counts were higher in MG-associated tissue compared to the normal thymus. Possible use of the TNF-Related Apoptosis-Inducing Ligand within the concept of an apoptotic ligand-mediated medical thymectomy in thymoma- or thymic hyperplasia-associated Myasthenia Gravis is also discussed. Copyright © 2016 Elsevier B.V. All rights reserved.
Yang, Dehao; Su, Zhongqian; Wu, Shengjie; Bi, Yong; Li, Xiang; Li, Jia; Lou, Kangliang; Zhang, Hongyu; Zhang, Xu
Oxidative stress and low antioxidant status play a major role in the pathogenesis of inflammatory and autoimmune diseases. Myasthenia gravis (MG) is an autoimmune condition targeting the neuromuscular junction, and its antioxidant status is still controversial. Our study aimed to investigate the correlation between the clinical characteristics of MG and the serum antioxidant status of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine. We measured serum antioxidant molecule levels of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine in 380 individuals, including 166 MG and 214 healthy controls. We found that MG patients had significantly lower serum levels of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine than healthy controls, whether male or female. Moreover, it was also shown in our study that uric acid, albumin and creatinine levels in patients with MG were correlated with disease activity and classifications performed by the Myasthenia Gravis Foundation of America. Our findings demonstrated that serum levels of bilirubin (Tbil, Dbil and Ibil), uric acid, albumin and creatinine were reduced in patients with MG. This suggested an active oxidative process in MG patients who had low antioxidant status.
Błaszczyk, Urszula; Janoszka, Beata
A method for analysis of six azaarenes (benzo[h]quinoline, benzo[a]acridine, benzo[c]acridine, dibenzo[a,c]acridine, dibenzo[a,j]acridine and dibenzo[a,h]acridine) in thermally treated high-protein food has been described. The clean-up procedure used based on alkaline hydrolysis, tandem solid phase extraction on columns filled with Extrelut - diatomaceous earth and cation exchanger (propyl sulfonic acid), enabled a selective isolation of carcinogenic compounds belonging to benzoacridines and dibenzoacridines from samples of cooked meat and its gravy. The isolated fractions of aza-PAHs were analysed by high-performance liquid chromatography with fluorescence detection. The detection limits for the azaarenes were between 0.0001ng and 0.005ng loaded on column. The recoveries for the four-ring and five-ring azaarenes were from 55% to 67%. Two types of dishes prepared from pork by pan-frying were investigated. Total contents of the benzoacridines and dibenzoacridines determined in cooked meat were 1.57 and 2.50ng/g in collar and chop samples, respectively; their gravies contained 0.34 and 0.59ng of these azaarenes per g of cooked meat. Copyright © 2007 Elsevier Ltd. All rights reserved.
The flux of cosmic rays beyond the GZK cutoff (∼ 10 20 eV) may be explained through their production by ultra high energy cosmic neutrinos, annihilating on the relic neutrino background, in the vicinity of our galaxy. This process is mediated through the production of a Z boson at resonance, and is generally known as the Z-Burst mechanism. We show that a similar mechanism can also contribute to the super-GZK spectrum at even higher, ultra-GZK energies, where the particles produced at resonance are the Kaluza-Klein gravitons of weak scale mass and coupling from the Randall-Sundrum (RS) hierarchy model of localized gravity model. We call this mechanism Gravi-Burst. We discuss the parameter space of relevance to Gravi-Bursts, and comment on the possibility of its contribution to the present and future super-GZK cosmic ray data and place bounds on the RS model parameters. Under certain assumptions about the energy spectrum of the primary neutrinos we find that cosmic ray data could be potentially as powerful as the LHC in probing the RS model
Paik, Julie J.; Corse, Andrea M.; Mammen, Andrew L.
Objective Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients. Methods We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features. Results All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%). High dose steroids were associated with worsening weakness in 2/6 (33%) patients. Conclusions Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis-MG overlap, high dose steroids may exacerbate symptoms and pryidostigmine may play an important therapeutic role. PMID:24412588
Lennon, V A; Lambert, E H; Leiby, K R; Okarma, T B; Talib, S
A synthetic gene encoding the 210 N-terminal residues of the alpha-subunit of the nicotinic acetylcholine receptor (AChR) of human skeletal muscle was cloned into an inducible expression plasmid to produce a fusion protein in high yield in Escherichia coli. Like native human AChR, the recombinant human alpha 1-210 protein induced AChR-binding, AChR-modulating, and AChR-blocking autoantibodies in rats when injected once intradermally as an emulsion in CFA, with Bordetella pertussis vaccine as supplementary adjuvant. The minimum dose of recombinant protein required to induce biochemical signs of experimental autoimmune myasthenia gravis (EAMG) with 100% incidence was 2.2 micrograms. With 6.6 to 22 micrograms, serum levels of autoantibodies were persistent, and clinically apparent EAMG lasted more than a month. Clinical, electrophysiological, and biochemical indices of EAMG induced by doses of 66 micrograms or more were more uniformly severe and persistent, with 33% fatality. Rats receiving a control extract of E. coli containing plasmid without the alpha 1-210 codon insert, with adjuvants, did not develop autoantibodies or signs of EAMG. This highly reproducible new model of EAMG induced by a recombinant human autoantigen should be valuable for testing Ag-specific immunotherapeutic strategies that might be applicable to treating acquired myasthenia gravis in humans.
Raggi, Alberto; Leonardi, Matilde; Ayadi, Roberta; Antozzi, Carlo; Maggi, Lorenzo; Baggi, Fulvio; Mantegazza, Renato
In this study we assess the Italian version of the 15-item Myasthenia Gravis Quality-of-Life questionnaire (MG-QOL15). The validation protocol included the MG-QOL15, the 36-item Short Form (SF-36), the Besta Neurological Institute Rating Scale for Myasthenia Gravis, and the MG-Composite. We used the Cronbach α to test reliability, the Spearman correlation to test short-term test-retest, the Kruskal-Wallis test to assess differences in MG-QOL15 between patients with different disease severity, and the Wilcoxon signed-rank test to assess sensitivity to change. Seventy-two patients were enrolled in the study. The mean MG-QOL15 score was 15.2 ± 12.2, with α = 0.93 and test-retest correlation = 0.93. Compared with the SF-36, the MG-QOL15 was superior in differentiating patients with different MG types (P = 0.041) and severity (P = 0.004), showed higher sensitivity to change (P = 0.003 for improved and P = 0.024 for worsened patients), and had higher correlations with the MG-Composite (rho = 0.367 vs. -0.213 and -0.154). The Italian version of the MG-QOL15 is valid, reliable, stable, and sensitive to changes. Muscle Nerve 56: 716-720, 2017. © 2016 Wiley Periodicals, Inc.
Kosachev, V D; Alekseeva, T M; Khalmurzina, A N
In the present work the results of the clinic-epidemiological analysis of 223 patients with the onset of the myasthenia at 60 y. o. and later, admitted and treated in the clinic of neurology for the passed 25years are represented. A dynamic growth of incidence of the late-onset myasthenia through the passed 10 years was administered. We administered a prevalence of the generalized form of the myasthenia gravis (61,5 %). The whole clinical table of the myasthenia was developed during an year in 76,7 % of the cases. A wide range of the concomitant somatic pathology in this group of the patients (especially, with a cardio-vascular pathology - 93,3 %) was found to worsen the course of the myasthenia itself. We found that the set of the therapeutic measures in myasthenia in the elderly is determined by the course of the myasthenia and the multiple organ failure due to the concomitant diseases. The scheme of complex corrective therapy of myasthenia gravis in elderly was developed.
Yamada, Chisa; Teener, James W; Davenport, Robertson D; Cooling, Laura
Anti-muscle specific kinase antibody positive myasthenia gravis (MuSK MG) is often characterized by a relatively severe and progressive course, refractoriness to standard myasthenia gravis (MG) medications, and an increased risk of myasthenic crisis. We report here successful management of three MuSK MG patients using maintenance therapeutic plasma exchange (TPE) treatment for up to 4.5 years. The study was a 5-year retrospective review of all MG patients treated with TPE between 2008 and 2013 at University of Michigan. Inclusion criteria of MuSK MG were positive for anti-MuSK antibodies and a diagnosis of MuSK MG by staff neurologists. Patient data included age, gender, diagnostic testing results, medications, and the dates and response to TPE treatments. A total of 153 MG patients underwent at least one course of TPE between 2008 and 2013. A total of 12 patients (7.8%) were positive for anti-MuSK antibodies. Patients were predominantly female (83.3%) and a median age of onset was 46-years old. Three MuSK MG patients were successfully managed with maintenance TPE. Maintenance TPE may be an effective option for MuSK MG patients. The key of successful maintenance treatment at our institution has been to tailor the TPE frequency for each individual, and to modify the treatment interval in conjunction with medical management. © 2014 Wiley Periodicals, Inc.
José Lamartine de Assis
Full Text Available Two pairs of siblings with myasthenia gravis, belonging to two different families, are reported. This is the only record of familial myasthenia during the past twenty years, in a total of 145 patients seen at the Neurological Clinic of the São Paulo Medical School. In spite of the fact that myasthenia gravis does not show hereditary characteristics, the peculiar features of the four cases justify the present report. The two pairs of siblings were born from non myasthenic nor consanguineous parents. The disease started at birth showing bilateral partial eyelid ptosis in all patients. The course of the illness has been favorable. There was no thymoma.Os autores registram dois pares de gêmeos com miastenia grave, pertencentes a duas famílias diferentes. Este é o único registro de miastenia familial durante os últimos 20 anos, num total de 145 pacientes examinados na Clínica Neurológica da FMUSP. Apesar do fato de a miastenia grave não ter características hereditárias, os aspectos peculiares dos quatro pacientes justificam o presente registro. Os dois pares de gêmeos nasceram de pais não miastênicos e sem consanguinidade. A doença iniciou-se no nascimento, evoluindo com ptose bilateral parcial da pálpebra superior precocemente em todos os pacientes. O curso da moléstia tem sido favorável. Não havia timoma.
Heckmann, J M; Uwimpuhwe, H; Ballo, R; Kaur, M; Bajic, Vladimir B.; Prince, S
Complement activation in myasthenia gravis (MG) may damage muscle endplate and complement regulatory proteins such as decay-accelerating factor (DAF) or CD55 may be protective. We hypothesize that the increased prevalence of severe extraocular
Rodriguez, Sonia Pilar; Zuluaga, Claudia Patricia; Uriza, Luis Felipe C; Sanchez M, Jully Mariana
Thymoma are thymic tumors that arise from epithelial cells, they have different morphological characteristics. It is known for its association with autoimmune diseases such as myasthenia gravis, pure red cell aplasia, systemic lupus erythematosus, or hipogamaglobulinemia pemphigus foliaceus. The association thymoma-myasthenia gravis-pure red cell aplasia is a rare one; there will be a case report with the corresponding discussion and review of the literature
De Roxas, Ranhel C; Bagnas, Marjorie Anne C; Baldonado, Jobelle Joyce Anne R; Rivera, Jonathan P; Roxas, Artemio A
Myasthenia gravis is an autoimmune neuromuscular disorder characterized by the production of abnormal autoantibodies directed against the receptors present in the neuromuscular junction. It has been the standard practice to offer thymectomy in all generalized myasthenia gravis patients despite the lack of robust evidence. The objectives of this study are to describe the clinical profile and differentiate the clinical outcomes of thymectomy versus non-thymectomy and thymomatous versus non-thymomatous myasthenia gravis patients in the Philippine General Hospital. Between 2009 and 2014, a total of 69 postthymectomy and 16 non-thymectomy patient records were successfully retrieved. The demographic characteristics, surgical approach, and histopathologic results were obtained. The clinical outcome after 6 months or 1 year-follow-up was also determined and grouped according to the following: (1) complete remission, (2) pharmacological remission, (3) no clinical change, (4) worsening symptoms, and (5) mortality. Majority of the patients were females (68.0%) with a mean age of 39.8 years and a mean duration of myasthenic symptoms of 21 months. Using the Myasthenia Gravis Foundation of America classification, 54.1% of patients fell under Class II and 48.2% of them presented with generalized weakness. In this study, 60.8% of postthymectomy myasthenia gravis patients had either complete remission or pharmacologic remission compared with 12.5% among non-thymectomy patients (p-value myasthenia gravis after thymectomy (p-value = 0.29). This study showed that both thymomatous and non-thymomatous myasthenia gravis patients who underwent thymectomy had a higher incidence of complete stable remission and pharmacologic remission as compared with myasthenia gravis patients who did not undergo thymectomy.
Full Text Available Valoramos por ecografía y estadísticamente la eficacia de la liposucción láser-asistida en casos de ginecomastia, teniendo en cuenta la respuesta de retracción de la piel, la rapidez de la recuperación del paciente, y el grado de satisfacción que originan los resultados. Presentamos un estudio prospectivo de 28 pacientes con ginecomastia de diferente grado, tratados mediante liposucción asistida por láser de Diodo de 1470 nm, previa sedación y con anestesia tumescente. El láser se programó a 15 Watios (W en emisión continua y se administraron 8 a 12 kilojulios (kJ por cada mama. No se realizaron escisiones de piel ni se emplearon drenajes. Se realizó valoración objetiva y subjetiva empleando la misma escala visual analógica (VAS completada por médicos y pacientes. Se tomaron fotografías antes y 6 meses después de la intervención, y se realizaron mediciones del perímetro torácico y del diámetro de las areolas. El seguimiento ecográfico de las áreas tratadas fue comparativo antes y 6 meses después del tratamiento. Para el estudio estadístico se empleó t de Student como test de contraste. No observamos complicaciones, como signos de isquemia o quemaduras cutáneas. La disminución del perímetro torácico y del diámetro de las areolas fue estadísticamente significativa. Tanto la valoración objetiva como la subjetiva alcanzaron resultados superiores al 90 %. Mediante la ecografía también se demostró disminución, estadísticamente significativa, de la banda de tejido graso subcutáneo (p < 0,05 al comparar las imágenes de antes y 6 meses después del tratamiento. La recuperación laboral se realizó en un promedio de 3 días. En conclusión, creemos que la liposucción láser-asistida es un método eficaz y reproducible, comprobado ecográfica y estadísticamente, que alcanza buenos resultados con menos trauma quirúrgico, lo que conlleva un alto grado de satisfacción por parte de los pacientes.
Plazola-Camacho, Noemí Guadalupe; Ortiz-Ibarra, Federico Javier
Introducción. Objetivos: revisar las recomendaciones internacionales e informar la experiencia que se tiene en el manejo y seguimiento de mujeres embarazadas infectadas por virus de inmunodeficiencia humana (VIH)/síndrome de inmunodeficiencia adquirida (SIDA) en el Instituto Nacional de Perinatología Isidro Espinosa de los Reyes (INPerIER). Métodos. Se presenta la experiencia clínica de 20 años (1988-2008) en el manejo de una cohorte de mujeres embarazadas infectadas por VIH atendidas en el I...
Christlbauer, Monika; Schieberle, Peter
Although the aroma compounds of meat processed as such have been studied previously, data on complete homemade dishes containing beef and pork meat were scarcely studied. Recently, 38 odor-active compounds were characterized in beef and pork vegetable gravies using GC-olfactometry. In the present investigation, the most odor-active compounds were quantitated in a freshly prepared stewed beef vegetable gravy (BVG) as well as a stewed pork vegetable gravy (PVG) by means of stable isotope dilution assays. Calculation of odor activity values (OAVs; ratio of concentration to odor threshold) revealed 3-mercapto-2-methylpentan-1-ol, (E,E)-2,4-decadienal, (E,Z)-2,6-nonadienal, (E)-2-decenal, (E)-2-undecanal, and 3-hydroxy-4,5-dimethyl-2(5H)-furanone as the most potent odorants in both gravies. However, significantly different OAVs were found for 12-methyltridecanal, which was much higher in the BVG, whereas (E,Z)-2,4-decadienal showed a clearly higher OAV in the PVG. Aroma recombination experiments performed on the basis of the actual concentrations of the odorants in both gravies revealed a good similarity of the aromas of both model mixtures containing all odorants with OAVs > 1 with those of the original gravies.
Zahid, Imran; Sharif, Sumera; Routledge, Tom; Scarci, Marco
A best evidence topic in thoracic surgery was written according to a structured protocol. The question addressed was how video-assisted thoracoscopic surgery (VATS) compares to median sternotomy in the surgical management of patients with myasthenia gravis (MG)? Overall 74 papers were found using the reported search, of which 15 represented the best evidence to answer the clinical question. The authors, journal, date and country of publication, patient group studied, study type, relevant outcomes and results are tabulated. We conclude that VATS produces equivalent postoperative mortality and complete stable remission (CSR) rates, with superior results in terms of hospital stay, operative blood loss and patient satisfaction at the expense of a doubling of operative time. Six studies comparing VATS and transsternal sternotomy in non-thymomatous myasthenia gravis (NTMG) patients found VATS to have lower operative blood loss (73.8±70.7 vs. 155.3±91.7 ml; P0.05). One study comparing video-assisted thoracoscopic extended thymectomy to transsternal thymectomy in only thymoma-associated myasthenia gravis (T-MG) patients found equivalent CSR (11.3 vs. 8.7%, P=0.1090) at six-year follow-up. Thymoma recurrence rate (9.64%) was not significantly different (P=0.1523) between the two groups. Eight studies comparing VATS and transsternal approach in mixed T-MG and NTMG patients found a lower hospital stay (1.9±2.6 vs. 4.6±4.2 days, P<0.001), reduced need for postoperative medication (76.5 vs. 35.7%, P=0.022), lower intensive care unit stay (1.5 vs. 3.2 days, P=0.018), greater symptom improvement (100 vs. 77.9%, P=0.019) and better cosmetic satisfaction (100 vs. 83, P=0.042) with VATS. In concordance with NTMG and T-MG alone patient groups, VATS and transsternal methods had equivalent complication rates (23 vs. 19%, P=0.765) with no mortalities in either group. Even though VATS has a longer operative time (268±51 vs. 177±92 min, P<0.05), its improved cosmesis, reduced need
Full Text Available Background: Respiratory failure and crisis is one of major complications of thymectomy in myasthenia gravis patients. There are different medication regimes for preparing these patients for surgery and reducing post-operative side effects. The goal of this study is to compare respiratory complications of oral vs. Parenteral preoperative administration of anticholinesterase agents for thymectomy in myasthenia gravis patients. Methods: This randomized controlled trial included 101 patients in class IIA or IIB of myasthenia gravis according to the Osserman classification system. The control group fasted for eight hours before surgery and oral anticholines-terase agents were replaced with parenteral ones. The case group also fasted for 8 hours before surgery, but pyridostigmine was continued at its usual dose until the time of operation and the last dose was given to patients with a small amount of water in the operating room on the operating bed. Results: There was no statistically meaningful difference between the two groups in terms of age, sex and pathologic findings. In comparison, the mean hospital stay for the case group was 3.98 days and 6.34 for the control group (p value = 0.003. There were eight cases of respiratory crisis or failure (16% in the control group but only 1 case (2% was observed in case group (p value = 0.014. Only one patient in the case group required re-intubation after the surgery; however, six patients in control group were re-intubated (p value = 0.053. Plasmapheresis was required for five patients in the control group and one patient in the case group (p value = 0.098. Tracheostomy was performed on two patients in the control group to accommodate prolonged intubation, but none of the case group required this procedure. Conclusion: This study shows that continuing oral anticholinesterase agents up to the time of operation, with the last dose at the operative theater, lowers the incidence of post-operative myasthenia
Full Text Available Objective To analyze the clinical characteristics, drug efficacy and prognosis of patients with myasthenia gravis (MG associated with other autoimmune diseases. Methods Eighty-three MG patients were divided into 2 groups. One group included MG patients with autoimmune diseases (AIDMG, N = 24, and the other included MG patients without autoimmune diseases (NAIDMG, N = 59. Firstly, clinical features such as sex, age of onset, initial symptoms and thymus abnormalities were compared between patients with AIDMG and NAIDMG. Secondly, effect of different therapies, including pyridostigmine, corticosteroids, immunoglobulin, immunosuppressants and thymectomy was compared between 2 groups. Finally, prognosis including relapse rate and recurrence time during the first 2 years after MG onset was compared. Whether and when ocular myasthenia gravis (OMG progressing to general myasthenia gravis (GMG and the first onset of GMG symptoms during the first 2 years were also compared between 2 groups. Results The difference of gender predominance (χ2 = 8.467, P = 0.004, ptosis affecting left or right or both sides (χ2 = 9.830, P = 0.007 and disease course within 2 years after onset (χ2 = 15.255, P = 0.001 between AIDMG group and NAIDMG group were statistically significant. Other clinical features such as age of onset (χ2 = 1.728, P = 0.228, initial symptoms (χ2 = 0.252, P = 0.791, thymus abnormalities (χ2 = 3.200, P = 0.202 were not significantly different between 2 groups. Differences of therapeutical effect such as pyridostigmine (χ2 = 0.411, P = 0.395, corticosteroids (χ2 = 0.156, P = 0.513, immunoglobulin (χ2 = 0.359, P = 0.462, immunosuppressants (χ2 = 0.081, P = 0.526 and thymectomy (χ2 = 0.337, P = 0.391 between 2 groups were not statistically significant. The ratio of OMG progressing to GMG (χ2 = 1.826, P = 0.148, time of progressing (Fisher's exact test: P = 0.639, first onset symptom (Fisher's exact test: P = 0.196 and recurrence
Bes-Rastrollo, M. (Maira); Basterra-Gortari, F.J. (Francisco Javier); Sanchez-Villegas, A. (Almudena); Marti, A. (Amelia); Martinez, J.A. (José Alfredo); Martinez-Gonzalez, M.A. (Miguel Ángel)
OBJECTIVE: The traditional Mediterranean food pattern is more easily preserved when meals are eaten at home; however, as a result of recent socio-economic changes, away-from-home meal consumption has increased rapidly in Mediterranean countries. Little research has been conducted so far to investigate the long-term health effects of these changes in the Mediterranean area. DESIGN: In a prospective Spanish dynamic cohort of 9182 university graduates (the SUN Study; Seguimiento Univer...
Martinez-Gonzalez, M.A. (Miguel Ángel); Lopez-Iracheta, R. (Roberto); Zazpe, I. (Itziar); Sayon-Orea, C. (Carmen); Gea, A. (Alfredo); Bes-Rastrollo, M. (Maira); Pimenta, A.M. (Adriano Marçal)
OBJECTIVE: To evaluate the association of snacking between main meals with the risk of developing metabolic syndrome. DESIGN: A dynamic prospective cohort study (the SUN Project; Seguimiento Universidad de Navarra). Snack consumption was evaluated using the question: 'Do you have the habit of snacking between main meals?' Metabolic syndrome was defined according to the updated harmonizing criteria. We estimated multivariable-adjusted relative risks (RR) of metabolic syndrome and th...
Unzueta, C R; Lahortiga-Ramos, F; Santiago, S; Zazpe, I; Molero, P; Sánchez-Villegas, A; Martínez-González, M A
The objective of this study is to assess the differences in lifestyles according to levels of self-perceived competitiveness, psychological tension, and dependency in a Mediterranean cohort of university graduates. Levels of personality traits, food consumption, nutrient intake, eating attitudes, physical activity, sedentary lifestyle, and alcohol and tobacco consumption were assessed through a questionnaire administered at baseline. This was a cross-sectional study in the context of the Seguimiento Universidad de Navarra cohort. Participants are 15,346 Spanish adults. Participants with a high level of self-perceived competitiveness consumed more vegetables and fish but less refined grains; they had higher protein intake and healthier eating attitudes. They were more physically active and less likely to be smokers. Participants with a high level of tension or dependency were less physically active, and participants more dependent also had poorer adherence to the Mediterranean diet. Self-perceived personality traits, especially the trait of competitiveness, are likely to be associated with healthier dietary patterns, better nutrient profile, better eating attitudes, physical activity, and less exposure to smoking. The use of short questions about self-perceived levels of competitiveness, psychological tension, and dependency can contribute to add additional information when assessing lifestyles and diet in adults. Copyright © 2018 The Royal Society for Public Health. Published by Elsevier Ltd. All rights reserved.
Full Text Available Resumen: En este trabajo se presenta en detalle el diseÃ±o de un control difuso para el seguimiento de guiÃ±ada de un vehÃculo autÃ³nomo submarino. Este control estÃ¡ desarrollado a partir de la descripciÃ³n matemÃ¡tica del modelo hidrodinÃ¡mico del vehÃculo, que se estudia y discute bajo diferentes situaciones de velocidad de avance o cambios en la referencia de guiÃ±ada. Se linealiza el modelo matemÃ¡tico y se estudian diferentes controles lineales que son diseÃ±ados para actuar en situaciones concretas, de forma que el control difuso se encargue de manejar dichos controles de manera global. Abstract: This work presents in detail the fuzzy control design for yaw tracking of an autonomous underwater vehicle. This control has been developed from the mathematical description of the hydrodynamic model of the vehicle, which is studied and discussed from different situations both in surge velocity as in changes in yaw reference. The model is linearized and several linear controls are designed for their actuation at certain situations, in a way that the fuzzy control allows to handle those controls globally. Palabras clave: fuzzy control, autonomous vehicles, linear control systems, mathematical models, continuous path control, Keywords: fuzzy control, autonomous vehicles, linear control systems, mathematical models, continuous path control.
Artículo científico -- Universidad de Costa Rica. Instituto de Investigaciones en Salud, 1989 Este trabajo describe aspectos psicosociales que resaltan de la interacción entre los proveedores de atencion en salud (el personal de salud) y las personas afectadas por el virus de la inmunodeficiencia human (HIV). La discusión se basa en las experiencias del autor obtenidas mediante el seguimiento de una falange de casi 1000 hombres homosexuales o bisexuales en Costa Rica, así como de la intera...
Hirose, Genjiro; Yamamoto, Teiji; Kosoegawa, Hiroshi; Saeki, Mitsuo; Hayashi, Toshiharu
Nine patients with myasthenia gravis (MG) had plain chest X-ray, tomography, computed tomography (CT) of the thorax, pneumomediastinography (PMG) and post-PMG CT scans. Routine chest radiography detected mediastinal masses in two patients and conventional laminogram provided no additional informations. CT yielded anterior mediastinal increased densities in seven patients (78%), whereas post-PMG CT delineated masses (thymoma or thymic hyperplasia) or thymic gland in all cases (100%), but these procedures could not distinguish thymic tumor from nontumorous conditions. PMG and post-PMG CT scans are superior to other radiographic techniques in detecting mediastinal abnormalities with more clear delineation of thymoma or thymic gland. When an abnormal density is present in the mediastinum of a patient with MG, PMG and post-PMG CT scans should be done to outline the mediastinal anatomy, to determine the size, extent, invasiveness of thymoma or thymic gland prior to thymectomy. (author)
FRANCISCO MARCOS B. CUNHA
Full Text Available Foram pesquisados aspectos históricos da miastenia grave desde as primeiras descrições da doença em 1672, pelo clínico inglês Thomas Willis. São descritas as dificuldades encontradas no manuseio dos primeiros pacientes diagnosticados. Pesquisaram-se fatos históricos ligados à investigação da doença, o tratamento, bem como curiosidades pouco citadas na literatura.We studied historical aspects of myasthenia gravis starting from its first description by the English physician, Sir Thomas Willis, in 1672. We also describe the difficulties in managing triating the first diagnosed patient. Historical facts related to the investigation and the initial treatment of this disorder as well as curiosities seldom mentioned in the literature are part of this paper.
Néstor R. Sánchez Dacal
Full Text Available Myasthenia Gravis MG is an autoimmune and chronic neuromuscular disease characterized by variable of weakness in the skeletal muscles that control the eye movements and it is confused with an ophthalmological disorder. With this presentation we pretend to systematize the Theoretical references about MG which allow making a correct diagnosis of the disease from the experience of a clinical case. The theory about MG regarding the presentation of the disease is discussed, emphasising on the significance of its differential diagnosis with an ophthalmopathy, which will contribute to apply a proper treatment and a satisfactory evolution of the patient, arriving to the conclusion that affectation of the III cranial pair is a way of frequent presentation of MG, being valuable the differential diagnosis of the ophthalmopaties in these entities.
Werneck, L C; Bittencourt, P C; Nóvak, E M
It is reported a case of a 61 years-old man with progressive asthenia, disfagia, disphonia and diplopia, of variable intensity during the day, who had a very good response to anticolinesterasic drugs and corticosteroids. The repetitive stimulation tests at low frequency, resulted in large increment (maximum 275%) of the basal voltage after exercise. At high frequency he also had a large increment. Radiological and laboratory investigation three times in a seven-year period was normal, without evidence of any neoplasia. Muscle biopsy showed a type II muscle fiber atrophy. The repetitive stimulation tests repeated three times, was typical of myasthenia gravis in one occasion and in the other two, typical of myasthenic syndrome (increment of 418%). A discussion about other cases with similar findings is made after a review of the literature.
André P.C. Matta
Full Text Available Myasthenia gravis (MG is a relatively uncommon disorder with an annual incidence of approximately 7 to 9 new cases per million. The prevalence is about 70 to 165 per million. The prevalence of the disease has been increasing over the past five decades. This is thought to be due to better recognition of the condition, aging of the population, and the longer life span of affected patients. MG causes weakness, predominantly in bulbar, facial, and extra-ocular muscles, often fluctuating over minutes to weeks, in the absence of wasting, sensory loss, or reflex changes. The picture of fluctuating, asymmetric external ophthalmoplegia with ptosis and weak eye closure is virtually diagnostic of myasthenia. We report an atypical MG case with three semiological cardinal signs.
Guevorkian, Karine; Valles, James M., Jr.
Paramecium Caudatum, a single celled ciliate, alters its swimming behavior when subjected to different gravity environments (e.g. centrifugation and micro-gravity). To dissect the mechanisms behind this gravi-response and that of other biological systems, we are developing the use of magnetic body forces as a means of creating a rapidly tunable, simulated variable gravity environment. Since biological materials are weakly diamagnetic, we must subject them to intense inhomogeneous magnetic fields with characteristic field-field gradient products on the order of 16 T^2/cm. We will describe experiments on Paramecium Caudatum in which we adjust their net buoyancy with magnetic forces and measure the resulting changes in their swimming behavior.
Full Text Available The relationship between myasthenia gravis (MG and other autoimmune disorders like hyperthyroidism is well known. It may manifest earlier, concurrently orafter the appearance of MG. The effect of treatment of hyperthyroidism on the control of MG is variable. There may be resolution or conversely, deterioration of the symptoms also. We present a patient who was diagnosed to be hyperthyroid two and half years before the appearance of myasthenic symptoms. Pharmacotherapy for three months neither improved the myasthenic symptoms nor the thyroid function tests. Thymectomy resulted in control of MG as well as hyperthyroidism. In conclusion, effective control of hyperthyroidism in the presence of MG may be difficult. The authors opine that careful peri-operative management of thymectomy is possible in a hyperthyroid state.
Datt, Vishnu; Tempe, Deepak K; Singh, Baljit; Tomar, Akhlesh S; Banerjee, Amit; Dutta, Devesh; Bhandari, Hricha
The relationship between myasthenia gravis (MG) and other autoimmune disorders like hyperthyroidism is well known. It may manifest earlier, concurrently or after the appearance of MG. The effect of treatment of hyperthyroidism on the control of MG is variable. There may be resolution or conversely, deterioration of the symptoms also. We present a patient who was diagnosed to be hyperthyroid two and half years before the appearance of myasthenic symptoms. Pharmacotherapy for three months neither improved the myasthenic symptoms nor the thyroid function tests. Thymectomy resulted in control of MG as well as hyperthyroidism. In conclusion, effective control of hyperthyroidism in the presence of MG may be difficult. The authors opine that careful peri-operative management of thymectomy is possible in a hyperthyroid state.
Full Text Available Circulating antibodies of the acetylcholine receptor (AchRAb are detectable in most patients with generalized myasthenia gravis (MG. A newly discovered antibody against muscle-specific kinase (MuSKAb has been detected in 40–70% of AchRAb-negative MG patients. We report a series of Taiwanese MuSKAb-positive patients, and compare their clinical features with MuSKAb-negative patients and also with MuSKAb-positive Caucasians. Five out of 44 seronegative generalized MG patients (11.4% were positive for MuSKAb. Patients with MuSKAb tended to have severe disability and bulbar involvement, and more often experienced crisis or impending crisis. Although all of these patients showed an initial response to immunosuppressant therapy, they had greater disability at follow-up. The clinical features of Taiwanese MuSKAb-positive patients were not different from those of Caucasians, except for a lower prevalence.
Chang, Elaine; Sabichi, Anita L; Sada, Yvonne H
Checkpoint inhibitors have become standard therapy for multiple cancers, and their use will increase in the next year as regulatory approvals for additional indications are expected. It is essential for clinicians to be aware of the potential for rare immune-related adverse effects. Here, we report the case of a new diagnosis of myasthenia gravis (MG) after the use of nivolumab for squamous cell carcinoma of the bladder. A review the literature identified 10 cases of MG diagnosed after programmed cell death protein 1 inhibitor therapy. This is the first case, to our knowledge, reported in association with bladder cancer. The precise diagnosis of MG has important implications on management, as treatment with steroids can transiently worsen myasthenia in nearly 50% of cases.
Vinge, Lotte; Jakobsen, Johannes; Pedersen, Asger Roer; Andersen, Henning
In patients with myasthenia gravis (MG), muscle strength is expected to decrease gradually during the day due to physical activities. Isometric muscle strength at the shoulder, knee, and ankle was determined in 10 MG patients (MGFA class II-IV) who were receiving usual medical treatment and in 10 control subjects. To determine diurnal and day-to-day variation, muscle strength was measured 4 times during day 1 and once at day 2. Knee extension strength decreased during the day in both patients and controls. Neither diurnal nor day-to-day variation of muscle strength was higher in patients compared with controls. Patients with mild to moderate MG did not have increased variation of isometric muscle strength during the day or from day-to-day compared with controls. This suggests that isometric muscle performance can be determined with high reproducibility in similar groups of MG patients without regard to time of day. © 2015 Wiley Periodicals, Inc.
Lorenzoni, Paulo José; Ducci, Renata Dal-Prá; Tensini, Tallulah Spina; Dalledone, Giuliano; Kay, Claudia Suemi Kamoi; de Paola, Luciano; Werneck, Lineu Cesar; Scola, Rosana Herminia; Silvado, Carlos
The relationship between myasthenia gravis (MG) and epilepsy has been rarely reported. As consequence, there are no specific guidelines for the management of these conditions when they mutually occur. We reported on three patients in whom epilepsy and MG are coexisting, but in different clinical settings. Two patients were treated with antiepileptic drugs which improved their symptoms. One patient has controlled the seizures after a successful anterior temporal lobectomy with no appreciable consequences to her MG. We discuss the difficulties in the management of epilepsy in patients with MG. In addition, we report on the first epileptic surgery in a MG patient, indicating that this surgical procedure as a safe option for the treatment of intractable epilepsy in patients with MG. Copyright © 2017. Published by Elsevier Ltd.
Full Text Available Electrical myotonia is known to occur in a number of inherited and acquired disorders including myotonic dystrophies, channelopathies, and metabolic, toxic, and inflammatory myopathies. Yet, electrical myotonia in myasthenia gravis associated with antibodies against muscle-specific tyrosine kinase (MuSK has not been previously reported. We describe two such patients, both of whom had a typical presentation of proximal muscle weakness with respiratory failure in the context of a significant electrodecrement in repetitive nerve stimulation. In both cases, concentric needle examination revealed electrical myotonia combined with myopathic motor unit morphology and early recruitment. These findings suggest that MuSK myasthenia should be included within the differential diagnosis of disorders with electrical myotonia.
Lazaridis, Konstantinos; Dalianoudis, Ioannis; Baltatzidi, Vasiliki; Tzartos, Socrates J
Myasthenia gravis (MG) is caused by autoantibodies, the majority of which target the muscle acetylcholine receptor (AChR). Plasmapheresis and IgG-immunoadsorption are useful therapy options, but are highly non-specific. Antigen-specific immunoadsorption would remove only the pathogenic autoantibodies, reducing the possibility of side effects while maximizing the benefit. We have extensively characterized such adsorbents, but in vivo studies are missing. We used rats with experimental autoimmune MG to perform antigen-specific immunoadsorptions over three weeks, regularly monitoring symptoms and autoantibody titers. Immunoadsorption was effective, resulting in a marked autoantibody titer decrease while the immunoadsorbed, but not the mock-treated, animals showed a dramatic symptom improvement. Overall, the procedure was found to be efficient, suggesting the subsequent initiation of clinical trials. Copyright © 2017 Elsevier B.V. All rights reserved.
Hirose, Genjiro; Yamamoto, Teiji; Kosoegawa, Hiroshi; Saeki, Mitsuo; Hayashi, Toshiharu (Kanazawa Medical Univ., Ishikawa (Japan))
Nine patients with myasthenia gravis (MG) had plain chest X-ray, tomography, computed tomography (CT) of the thorax, pneumomediastinography (PMG) and post-PMG CT scans. Routine chest radiography detected mediastinal masses in two patients and conventional laminogram provided no additional information. CT yielded anterior mediastinal increased densities in seven patients (78%), whereas post-PMG CT delineated masses (thymoma or thymic hyperplasia) or thymic gland in all cases (100%), but these procedures could not distinguish thymic tumor from nontumorous conditions. PMG and post-PMG CT scans are superior to other radiographic techniques in detecting mediastinal abnormalities with more clear delineation of thymoma or thymic gland. When an abnormal density is present in the mediastinum of a patient with MG, PMG and post-PMG CT scans should be done to outline the mediastinal anatomy, to determine the size, extent, invasiveness of thymoma or thymic gland prior to thymectomy.
Szobor, A.; Fornet, B.
The 75 Se-seleno-methionine isotope thymus scanning was examined in a series of patients with myasthenia gravis. The patients were given 4 μC/kg hence a total of 250-300 μC 75 Se-seleno-methionine, intravenously. The method proved useful and informative in the diagnostics of myasthenia. Prior to thymectomy, the thymic tumour or a large gland could be observed and some hints could be gained for the biological activity of the gland. After the operation, the success of thymectomy could be checked and later a possible recidive could be shown or excluded. In non-operative cases the change in thymic activity could be followed which was an important sign of a malignant or tumorous growth of the thymus. (author)
Fon, G.T.; Bein, M.E.; Mancuso, A.A.; Keesey, J.C.; Lupetin, A.R.; Wong, W.S.
Chest radiographs and computed tomographic (CT) scans of the mediastinum were correlated with pathologic findings of the thymus following thymectomy in 57 patients with myasthenia gravis. Based on the patient's age and the overall morphology of the anterior mediastinum, CT scans were assigned one of four grades in an attempt to predict thymus pathologic findings. Using this grading, 14 of 16 cases of thymoma were suspected or definitely diagnosed. One of the two cases not diagnosed on CT was a microscopic tumor. There were no false-positive diagnoses in 11 cases graded as definitely thymoma. We conclude that thymoma can be sensitively diagnosed in patients older than 40 years of age. However, thymoma cannot be predicted with a high level of confidence in patients younger than 40 because of the difficulty in differentiating normal thymus or hyperplasia from thymoma. Recommendations for the use of CT in the preoperative evaluation of myasthenic patients are presented.
Full Text Available Prikazali smo slučaj 22-godišnjeg muskarca sa kliničkom slikom otežanog gutanja i dvoslika. Bolest myasthenia gravis (MG je dijagnostikovana na osnovu pozitivnog faramakološkog testa, pozitivnog testa neuromišićne transmisije, kompjuterizovane tomografije medijastinuma. Antitela protiv acetilholinskog receptora (AChR i antitela protiv mišićno specifičnog receptora za tirozin kinazu (MuSK. bila su negativna. Zabeleženo je delimično poboljšanje u neurološkom nalazu na ordiniranu terapiju Piridostigminom, Ciklosporinom, kortikosteroidima. Bolesnik je imao povoljan odgovor na izmenu plazme sa povlačenjem svih kliničkih siimptoma što potvrdjuje hipotezu o učešću humoralnih faktora u patogenezi dvostruko negativne MG.
Fon, G.T.; Bein, M.E.; Mancuso, A.A.; Keesey, J.C.; Lupetin, A.R.; Wong, W.S.
Chest radiographs and computed tomographic (CT) scans of the mediastinum were correlated with pathologic findings of the thymus following thymectomy in 57 patients with myasthenia gravis. Based on the patient's age and the overall morphology of the anterior mediastinum, CT scans were assigned one of four grades in an attempt to predict thymus pathologic findings. Using this grading, 14 of 16 cases of thymoma were suspected or definitely diagnosed. One of the two cases not diagnosed on CT was a microscopic tumor. There were no false-positive diagnoses in 11 cases graded as definitely thymoma. We conclude that thymoma can be sensitively diagnosed in patients older than 40 years of age. However, thymoma cannot be predicted with a high level of confidence in patients younger than 40 because of the difficulty in differentiating normal thymus or hyperplasia from thymoma. Recommendations for the use of CT in the preoperative evaluation of myasthenic patients are presented
MARCIA RUBIA R. GONÇALVES
Full Text Available We report the case of a 49-year-old woman who has the rare combination of myasthenia gravis and cervical dystonia. She was treated with botulinum toxin type A with good response and no evidence of deterioration of the myasthenic symptoms. We therefore conclude that it is possible to use botulinum toxin in the presence of defective neuromuscular transmission.Relatamos o caso de uma mulher de 49 anos com rara combinação de miastenia gravis e distonia cervical tratada com toxina botulínica tipo A, apresentando boa resposta e nenhuma evidência de piora do quadro miastênico. A partir dessas observações concluimos que é possível o uso de toxina botulínica na presença de doença da transmissão neuromuscular.
Sekine, Yasuo; Kawaguchi, Naoki; Hamada, Chikuma; Sekiguchi, Hiromi; Yasufuku, Kazuhiro; Iyoda, Akira; Shibuya, Kiyoshi; Fujisawa, Takehiko
The purpose of this study was to clarify the clinical benefits of perioperative administration of high-dose prednisolone (PSL) combined with extended thymectomy on the long-term outcomes of 116 consecutive patients with generalized myasthenia gravis (MG). A retrospective review was conducted on 116 patients diagnosed with generalized MG who received alternate-day oral administration of high-dose PSL (100 mg/alternate days) and had undergone transsternal extended thymectomy. Incidences of postoperative myasthenic crisis, adverse effects of steroid, long-term outcomes, such as complete stable remission (CSR), pharmacologic remission (PR) or improvement (Imp), and disease recurrence after CSR were evaluated. Six patients (5.2%) experienced post-thymectomy myasthenic crisis. Crude cumulative CSR and PR + CSR rates were 44.8 and 62.7%, respectively. Life table analysis showed that 41.8, 52.8 and 63.4% of the patients were in CSR at 3, 5 and 10 years, respectively. Multivariate analysis revealed that age and pretreatment classification according to the Myasthenia Gravis Foundation of America (MGFA) criteria tended to be independent predictors of CSR. There were 6.9% with compressive vertebral fracture, 13.8% with cataract, and 5.2% with steroid-induced diabetes. Life table analysis revealed that recurrence rates after CSR were 36.8 and 46.0% at 3 and 5 years, respectively. Patients with thymoma had a significantly higher rate of recurrence than those without thymoma (p = 0.001). Alternate-day administration of high-dose prednisolone reduced the risk of post-thymectomy myasthenic crisis. Presence of thymoma was a risk factor for MG recurrence after CSR.
Fuentealba, Maximiliano; Troncoso, Miguel; Vallejos, Joaquin; Ponce, Sebastian; Villablanca, Nelson; Melita, Pablo
Cervical incompetence it's a dilatation of the cervix during the third trimester of pregnancy that ends with the interruption of it. The incidence in Chile is about 0.1-2% of the total pregnancies and it's one of the causes of preterm birth. A 34 years old pregnant patient. Timectomized at age 18 to treat her miastenia gravis, previously trated with medication, had 4 previous preterm labours all of them under 25 weeks and vaginal births. All fetuses died postpartum. A cerclage was made during the third, fourth and fifth pregnancies. She didn't present hypertension during the gestation and no cervical diameter under 15mm. Since the fourth gestation the following tests are taken: Antifosfolipidic antibodies, APTT,PT. All the results are either normal or negative. Microbial cultures were negative. No amniocentesis was made. A McDonald cervical cerclage was made during pregnancies number 3, 4 and 5 on the 16th week to delay the labor. Also oral micronized progesterone, on a 400mg/24 hours dosis, was administered to avoid preterm birth. On the 24th week the pharmacological treatment started including Intramuscular Betamethasone, 12 mg/24 hours (2 doses), to induce lung maturity on the fetus. It is thought that the administration of progesterone could have improved the situation of the patient, because it acts as a labour repressants. The use of cerclage could have helped, but the factors that may influence the effectiveness of this method are unknown. Perhaps there is some immunologic factor associated with the miastenia gravis that alters the normal course of pregnancy.
Full Text Available Objective To compare the sensitivity and specificity of ice test, neostigmine test and single fiber electromyography (SFEMG in the diagnosis of ocular myasthenia gravis (OMG, so as to establish an appropriate process for the diagnosis of OMG. Methods A total of 116 patients with newly onset ptosis and/or diplopia were detected by ice test, neostigmine test and SFEMG. Patients were followed up for 6 months to observe the disease changes and experimental treatment effects. Results Apart from patients whose diagnosis was in doubt or lost to follow - up, and who were diagnosed as generalized myasthenia gravis (GMG, the definite diagnosis was finally made in 81 patients, including 21 OMG patients and 60 patients with ptosis and/or diplopia caused by other diseases. The sensitivity of ice test for diagnosis of OMG was 95.24% (20/21, and specificity was 98.33%(59/60. The sensitivity of neostigmine test for diagnosis of OMG was 90.48% (19/21, and specificity was 85% (51/60. The sensitivity of SFEMG for diagnosis of OMG was 95.24% (20/21, and specificity was 80% (48/60. Among 3 tests, only specificity was found significant difference (χ2 = 5.232, P = 0.022. The specificity of ice test was better than that of neostigmine test (χ2 = 5.707, P = 0.017 and SFEMG (χ2 = 6.023, P = 0.014. Conclusions The ice test achieved high sensitivity and specificity in the diagnosis of OMG. The combination of ice test, neostigmine test and SFEMG may have important clinical value for the early diagnosis of OMG. DOI: 10.3969/j.issn.1672-6731.2016.10.008
Attia, Mohamed; Maurer, Marie; Robinet, Marieke; Le Grand, Fabien; Fadel, Elie; Le Panse, Rozen; Butler-Browne, Gillian; Berrih-Aknin, Sonia
Myasthenia gravis (MG) is a neuromuscular disease caused in most cases by anti-acetyl-choline receptor (AChR) autoantibodies that impair neuromuscular signal transmission and affect skeletal muscle homeostasis. Myogenesis is carried out by muscle stem cells called satellite cells (SCs). However, myogenesis in MG had never been explored. The aim of this study was to characterise the functional properties of myasthenic SCs as well as their abilities in muscle regeneration. SCs were isolated from muscle biopsies of MG patients and age-matched controls. We first showed that the number of Pax7+ SCs was increased in muscle sections from MG and its experimental autoimmune myasthenia gravis (EAMG) mouse model. Myoblasts isolated from MG muscles proliferate and differentiate more actively than myoblasts from control muscles. MyoD and MyoG were expressed at a higher level in MG myoblasts as well as in MG muscle biopsies compared to controls. We found that treatment of control myoblasts with MG sera or monoclonal anti-AChR antibodies increased the differentiation and MyoG mRNA expression compared to control sera. To investigate the functional ability of SCs from MG muscle to regenerate, we induced muscle regeneration using acute cardiotoxin injury in the EAMG mouse model. We observed a delay in maturation evidenced by a decrease in fibre size and MyoG mRNA expression as well as an increase in fibre number and embryonic myosin heavy-chain mRNA expression. These findings demonstrate for the first time the altered function of SCs from MG compared to control muscles. These alterations could be due to the anti-AChR antibodies via the modulation of myogenic markers resulting in muscle regeneration impairment. In conclusion, the autoimmune attack in MG appears to have unsuspected pathogenic effects on SCs and muscle regeneration, with potential consequences on myogenic signalling pathways, and subsequently on clinical outcome, especially in the case of muscle stress.
Bradshaw, J G; Peeler, J T; Twedt, R M
The thermal resistance of spore crops produced from each of two ileal loop-reactive strains of Clostridium perfringens type A was determined in two suspending vehicles consisting of 0.067 M (pH 7.0) phosphate buffer and a commercial beef gravy. D115.6 values obtained in buffer and enumerated after pretreatment with sodium ethylenediaminetetraacetate and recovery in plating medium containing lysozyme were two- to threefold greater than those obtained without this treatment. D115.6 values obtained with beef gravy were less than those obtained in buffer with or without lysozyme; however, the D98.9 and D104.4 values were 1.3 to 2 times greater than those obtained in buffer with lysozyme. The z values were within the ranges reported by previous investigators.
Singh, Paramveer; Idowu, Olakunle; Malik, Imrana; Nates, Joseph L
Magnesium is known to act at the neuromuscular junction by inhibiting the presynaptic release of acetylcholine and desensitizing the postsynaptic membrane. Because of these effects, magnesium has been postulated to potentiate neuromuscular weakness. We describe the case of a 62-year-old woman with myasthenia gravis and a metastatic thymoma who was admitted to our intensive care unit for management of a myasthenic crisis. The patient's neuromuscular weakness worsened in association with standard intravenous magnesium replacement, and the exacerbated respiratory failure necessitated intubation, mechanical ventilation, and an extended stay in the intensive care unit. The effect of magnesium replacement on myasthenia gravis patients has not been well documented, and we present this case to increase awareness and stimulate research. In addition, we discuss the relevant medical literature.
Bucuk, Mira; Gasparovic, Iva; Sonnenschein, Ivan; Perkovic, Olivio
Myasthenia gravis is a chronic autoimmune disease characterized by weakening of voluntary muscles during the day and a marked restitution of function during the night and after rest. The symptoms may worsen over days or weeks, sometimes even in a few hours, and are usually well controlled by appropriate therapy. Arachnoid cysts are congenital or acquired deformities of the arachnoid membrane and are usually too small to cause distinct clinical symptomatology. We describe a case of a 76-year-old myasthenia gravis patient with an arachnoid cyst. To the best of our knowledge this is the first reported case of these two comorbidities together.
Beuchat, L R; Clavero, M R; Jaquette, C B
The presence of psychrotrophic enterotoxigenic Bacillus cereus in ready-to-serve meats and meat products that have not been subjected to sterilization treatment is a public health concern. A study was undertaken to determine the survival, growth, and diarrheal enterotoxin production characteristics of four strains of psychrotrophic B. cereus in brain heart infusion (BHI) broth and beef gravy as affected by temperature and supplementation with nisin. A portion of unheated vegetative cells from...
Full Text Available 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.Megaesôfago é uma causa comum de regurgitação sendo uma das possíveis etiologias a miastenia grave caracterizada por uma desordem neuromuscular que resulta em fraqueza dos músculos esqueléticos, do esôfago, da laringe e da faringe. O presente relato descreve um caso de uma cadela da raça Pastor Alemão, com histórico de fraqueza muscular e vômitos freqüentes, que foi diagnosticada como megaesôfago secundário à miastenia grave. A radiografia do animal apresentou um acentuado megaesôfago cervical e torácico. Após a instituição do protocolo terapêutico recomendado o animal apresentou 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. A proprietária optou pela eutanásia do animal e a necropsia revelou severa pneumonia e dilatação acentuada da por
Full Text Available Este estudio fue realizado con dos grupos de tití gris (Saguinus leucopus, procedentes de decomisos y entregas voluntarias. Los animales fueron rehabilitados durante aproximadamente un año en la ciudad de Bogotá, con el fin de estimular el desarrollo y recuperación de las habilidades necesarias para su sobrevivencia al ser devueltos al medio ambiente natural. Se construyeron dos jaulas como plataformas de liberación en un bosque en el departamento del Tolima, y los animales fueron alojados en ellas para iniciar su proceso de acostumbramiento al medio. Durante este tiempo se realizó un estudio etológico, con el propósito de determinar las posibles variaciones comportamentales derivadas del cambio en el entorno. Los animales fueron liberados y se llevó a cabo el seguimiento visual para evaluar el proceso de rehabilitación y la adaptación de los individuos al nuevo hábitat. Se observó una variación en la presentación de comportamientos relacionados con actividades de desplazamiento, descanso e interacciones sociales en comparación con estudios realizados en cautiverio. Después de la liberación, los animales fueron vistos en sitios próximos a las jaulas de acostumbramiento, lo cual podría indicar que establecieron sus territorios en estas áreas; no se registraron variaciones significativas en la conformación de los grupos ni enfrentamientos entre las dos tropas. Estas observaciones indicarían que el proceso de liberación cumplió con su objetivo, y puede considerarse exitoso.
Mendonça, Raquel de Deus; Lopes, Aline Cristine Souza; Pimenta, Adriano Marçal; Gea, Alfredo; Martinez-Gonzalez, Miguel Angel; Bes-Rastrollo, Maira
Some available evidence suggests that high consumption of ultra-processed foods (UPFs) is associated with a higher risk of obesity. Collectively, this association and the nutritional characteristics of UPFs suggest that UPFs might also be associated with hypertension. We prospectively evaluated the relationship between UPF consumption and the risk of hypertension in a prospective Spanish cohort, the Seguimiento Universidad de Navarra project. We included 14,790 Spanish adult university graduates who were initially free of hypertension at baseline who were followed for a mean of 9.1 years (SD, 3.9 years; total person-years: 134,784). UPF (industrial formulations of chemical compounds which, beyond substances of common culinary use such as salt, sugar, oils, and fats, include substances also derived from foods but not used in culinary preparations) consumption was assessed using a validated semi-quantitative 136-item food-frequency questionnaire. Cox proportional hazards models were used to estimate adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) for hypertension incidence. During follow-up, 1,702 incident cases of hypertension were identified. Participants in the highest tertile of UPF consumption had a higher risk of developing hypertension (adjusted HR, 1.21; 95% CI, 1.06, 1.37; P for trend = 0.004) than those in the lowest tertile after adjusting for potential confounders. In this large prospective cohort of Spanish middle-aged adult university graduates, a positive association between UPF consumption and hypertension risk was observed. Additional longitudinal studies are needed to confirm our results. © American Journal of Hypertension, Ltd 2016. All rights reserved. For Permissions, please email: firstname.lastname@example.org
Juneja, V K; Klein, P G; Marmer, B S
Duplicate beef gravy or ground beef samples inoculated with a suspension of a four-strain cocktail of Escherichia coli O157:H7 were subjected to sublethal heating at 46 degrees C for 15-30 min, and then heated to a final internal temperature of 60 degrees C. Survivor curves were fitted using a linear model that incorporated a lag period (TL), and D-values and 'time to a 4D inactivation' (T4D) were calculated. Heat-shocking allowed the organism to survive longer than non-heat-shocked cells; the T4D values at 60 degrees C increased 1.56- and 1.50-fold in beef gravy and ground beef, respectively. In ground beef stored at 4 degrees C, thermotolerance was lost after storage for 14 h. However, heat-shocked cells appeared to maintain their thermotolerance for at least 24 h in ground beef held to 15 or 28 degrees C. A 25 min heat shock at 46 degrees C in beef gravy resulted in an increase in the levels of two proteins with apparent molecular masses of 60 and 69 kDa. These two proteins were shown to be immunologically related to GroEL and DnaK, respectively. Increased heat resistance due to heat shock must be considered while designing thermal processes to assure the microbiological safety of thermally processed foods.
Luo, Mengchuan; Liu, Xiaofang; Meng, Huanyu; Xu, Liqun; Li, Yi; Li, Zhibin; Liu, Chang; Luo, Yue-Bei; Hu, Bo; Xue, Yuanyuan; Liu, Yu; Luo, Zhaohui; Yang, Huan
Abnormal CD4 + T cell activation is known to play roles in the pathogenesis of myasthenia gravis (MG). However, little is known about the mechanisms underlying the roles of lncRNAs in regulating CD4 + T cell. In this study, we discovered that the lncRNA IFNG-AS1 is abnormally expressed in MG patients associated with quantitative myasthenia gravis (QMG) and the positive anti-AchR Ab levels patients. IFNG-AS1 influenced Th1/Treg cell proliferation and regulated the expression levels of their transcription factors in an experimental autoimmune myasthenia gravis (EAMG)model. IFNG-AS1 could reduce the expression of HLA-DRB and HLA-DOB and they had a negative correlation in MG. Furthermore IFNG-AS1 influenced the expression levels of CD40L and CD4 + T cells activation in MG patient partly depend on effecting the HLA-DRB1 expression. It suggests that IFNG-AS1 may be involved in CD4 + T cell-mediated immune responses in MG. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.
Modelagem Bayesiana do risco de infecção tuberculosa para estudos com perdas de seguimento Modelaje Bayesiano del riesgo de infección tuberculosa para estudios con pérdidas de seguimiento Bayesian model for the risk of tuberculosis infection for studies with individuals lost to follow-up
Edson Zangiacomi Martinez
Full Text Available OBJETIVO: Desenvolver um modelo estatístico baseado em métodos Bayesianos para estimar o risco de infecção tuberculosa em estudos com perdas de seguimento, comparando-o com um modelo clássico determinístico. MÉTODOS: O modelo estocástico proposto é baseado em um algoritmo de amostradores de Gibbs, utilizando as informações de perdas de seguimento ao final de um estudo longitudinal. Para simular o número desconhecido de indivíduos reatores ao final do estudo e perdas de seguimento, mas não reatores no tempo inicial, uma variável latente foi introduzida no novo modelo. Apresenta-se um exercício de aplicação de ambos os modelos para comparação das estimativas geradas. RESULTADOS: As estimativas pontuais fornecidas por ambos os modelos são próximas, mas o modelo Bayesiano apresentou a vantagem de trazer os intervalos de credibilidade como medidas da variabilidade amostral dos parâmetros estimados. CONCLUSÕES: O modelo Bayesiano pode ser útil em estudos longitudinais com baixa adesão ao seguimento.OBJETIVO: Desarrollar un modelo estadístico en basado en métodos Bayesianos para estimar el riesgo de infección tuberculosa en estudios con pérdidas de seguimiento, comparándolo con un modelo clásico deterministico. MÉTODOS: El modelo estocástico propuesto se basa en un algoritmo de muestreadotes de Gibbs, utilizando las informaciones de pérdidas de seguimiento al final de un estudio longitudinal. Para simular el número desconocido de individuos reactores al final del estudio y pérdidas de seguimiento, pero no reactores en el tiempo inicial, una variable latente fue introducida en el nuevo modelo. Se presenta un ejercicio de aplicación de ambos modelos para comparación de las estimaciones generadas. RESULTADOS: Las estimaciones puntuales suministradas por ambos modelos son próximas, pero el modelo Bayesiano presentó la ventaja de traer los intervalos de credibilidad como medidas de variabilidad muestral de los par
Mahakarnchanakul, W; Beuchat, L R
A study was done to determine the influence of temperature on growth and toxin production characteristics of psychrotrophic and mesophilic strains of Bacillus cereus when inoculated into mashed potatoes and chicken gravy containing various concentrations of sodium chloride and held at temperatures different from those at which cells had been cultured. Logarithmic growth phase cells (10 h, 30 degrees C) of psychrotrophic (F3802A/84) and mesophilic (B4ac-1) strains of Bacillus cereus were inoculated into rehydrated commercially processed instant mashed potatoes and chicken gravy supplemented with 0, 2, or 4% sodium chloride. Growth, survival, and diarrheal toxin production in potatoes and gravy held at 30, 37, and 10 degrees C (strain F3802A/84) or 30, 40, and 10 degrees C (strain B4ac-1) were monitored. Both strains grew in both foods containing no added sodium chloride or 2% sodium chloride when held at 30, 37, or 40 degrees C for 2 days. Strain B4ac-1 grew better than strain F3802A/84 in foods containing 4% sodium chloride. Maximum amounts of enterotoxin (1024 ng/g) were produced by strain B4ac-1 in chicken gravy held at 30 and 40 degrees C. Strain F3802A/84 grew to populations of 7 log10 CFU/g in foods containing no added sodium chloride or 2% sodium chloride at 10 degrees C. Strain F3802A/84 produced the highest amount of enterotoxin (1024 ng/g) at 30 degrees C in chicken gravy containing 0.7 or 2% sodium chloride; however, little or low amounts of toxin (4-16 ng/g) were produced in chicken gravy at 10 degrees C. Compared to strain B4ac-1, cells of strain F3802A/84 subjected to a downward shift in incubation temperature (10 degrees C) grew more rapidly in chicken gravy. Strain B4ac-1 produced the highest amount of toxin (1024 ng/g) at 30 degrees C in gravy containing 4% sodium chloride and at 40 degrees C in gravy containing 0.7% sodium chloride. Toxin was not detected in inoculated mashed potatoes. Results of this study indicate that shifts in incubation
Xabier Adrián García de Albéñiz
Full Text Available Objetivo: Valorar la asociación de la situación laboral, el tipo de estudios y el estado civil con el consumo de tabaco en una cohorte de licenciados y diplomados universitarios españoles, donde predominan los profesionales sanitarios. Métodos: Se realizó un análisis transversal de los datos basales de los 7.508 primeros participantes del Proyecto SUN (Seguimiento Universidad de Navarra. Los sujetos fueron clasificados según su consumo de tabaco. Las variables independientes fueron: situación laboral, estado civil, nivel más alto de estudios completado, número de hijos, gramos de alcohol ingerido al día, índice de masa corporal y edad. En el análisis multivariante se ajustaron 3 grupos de modelos de regresión logística no condicional: a fumadores frente al conjunto formado por nunca fumadores más ex fumadores; b fumadores frente a nunca fumadores, y c fumadores de 15 o más cigarrillos al día frente al conjunto formado por fumadores de menos de 15 cigarrillos/día, nunca fumadores y ex fumadores. Resultados: En los varones no se encontró ninguna asociación entre la situación laboral y ser fumador en ninguna de las 3 comparaciones. Por el contrario, entre las mujeres se observó que haber cursado la diplomatura de enfermería se asociaba a una mayor prevalencia de tabaquismo, con una prevalencia del 48,5% de fumadoras actuales entre las enfermeras. Entre las mujeres, también era más frecuente el consumo de tabaco en las estudiantes. Para las estudiantes, la prevalencia de grandes fumadoras (≥ 15 cigarrilos/día mostraba una odds ratio de 1,81 (intervalo de confianza del 95%, 1,28-2,57. Haber realizado un doctorado se asociaba inversamente con el tabaquismo en ambos sexos. Conclusiones: Dentro de un gran colectivo español con niveles educativos superiores, la prevalencia de fumadoras actuales es mayor en las mujeres que han completado una diplomatura. Es preocupante, desde el punto de vista sociosanitario, que entre las
Full Text Available Resumen: Objetivo: Estimar la prevalencia de pacientes con terapia anticoagulante oral (TAO en la Comunitat Valenciana (CV y evaluar la calidad del seguimiento de la TAO con antagonistas de la vitaminaÂ K (AVK realizado en atenciÃ³n primaria. DiseÃ±o: Estudio observacional transversal realizado a travÃ©s de la Red Centinela Sanitaria de la Comunitat Valenciana (RCSCV, que incluye una encuesta y el anÃ¡lisis retrospectivo del control de la TAO. Emplazamiento: AtenciÃ³n primaria (AP, Comunitat Valenciana (CV, EspaÃ±a. Participantes: Todos los pacientes con â¥Â 18Â aÃ±os con TAO que consultaron durante el aÃ±o 2014. La poblaciÃ³n cubierta por los 59 mÃ©dicos de la RCSCV supone un 2,2% de la poblaciÃ³n adulta de la CV, y es representativa de esta. Mediciones principales: Datos demogrÃ¡ficos, socioeconÃ³micos, de salud y referentes a la TAO. La calidad del seguimiento de la TAO con AVK fue valorada mediante el porcentaje de tiempo dentro de rango terapÃ©utico (TRT, calculado mediante el mÃ©todo Rosendaal. Resultados: Se registraron 1.144 pacientes (edad media 74,5Â Â±Â 11Â aÃ±os; 49,7% mujeres. La prevalencia de TAO en la CV es de 1,3 casos por 100 habitantes. El perfil caracterÃstico de estos pacientes es una persona aÃ±osa, pluripatolÃ³gica, con bajo nivel educativo, que vive acompaÃ±ada. La fibrilaciÃ³n auricular es la indicaciÃ³n mÃ¡s habitual. El 82,8% de los pacientes con TAO con AVK llevaron seguimiento a travÃ©s de AP. El TRT medio fue del 65,0%, y el 53,9% de pacientes presentaron un TRT â¥Â 65%. El 74,4% de los pacientes con un control inadecuado fueron percibidos como bien controlados por el mÃ©dico de AP. Conclusiones: La prevalencia de la TAO es alta y se prevÃ© que siga aumentando. El grado de control alcanzado cumple con el estÃ¡ndar de calidad generalmente aceptado (TRT medio â¥Â 65% y es comparable al observado en otros estudios nacionales e
Oshima, Minako; Iida-Klein, Akiko; Maruta, Takahiro; Deitiker, Philip R; Atassi, M Zouhair
Experimental autoimmune myasthenia gravis (EAMG), an animal model of myasthenia gravis (MG), can be induced in C57BL/6 (B6, H-2 b ) mice by 2-3 injections with Torpedo californica AChR (tAChR) in complete Freund's adjuvant. Some EAMG mice exhibit weight loss with muscle weakness. The loss in body weight, which is closely associated with bone structure, is particularly evident in EAMG mice with severe muscle weakness. However, the relationship between muscle weakness and bone loss in EAMG has not been studied before. Recent investigations on bone have shed light on association of bone health and immunological states. It is possible that muscle weakness in EAMG developed by anti-tAChR immune responses might accompany bone loss. We determined whether reduced muscle strength associates with decreased bone mineral density (BMD) in EAMG mice. EAMG was induced by two injections at 4-week interval of tAChR and adjuvants in two different age groups. The first tAChR injection was either at age 8 weeks or at 15 weeks. We measured BMD at three skeletal sites, including femur, tibia, and lumbar vertebrae, using dual energy X-ray absorptiometry. Among these bone areas, femur of EAMG mice in both age groups showed a significant decrease in BMD compared to control adjuvant-injected and to non-immunized mice. Reduction in BMD in induced EAMG at a later-age appears to parallel the severity of the disease. The results indicate that anti-tAChR autoimmune response alone can reduce bone density in EAMG mice. BMD reduction was also observed in adjuvant-injected mice in comparison to normal un-injected mice, suggesting that BMD decrease can occur even when muscle activity is normal. Decreased BMD observed in both tAChR-injected and adjuvant-injected mice groups were discussed in relation to innate immunity and bone-related immunology involving activated T cells and tumour necrosis factor-related cytokines that trigger osteoclastogenesis and bone loss.
Garcés-Palacio, Isabel Cristina; Universidad de Antioquia,; Rubio-León, Diana Carolina; Ramos-Jaraba, Sara Milena
identificar barreras y facilitadores del seguimiento: diagnóstico y tratamiento de anormalidadescitológicas en mujeres de bajos ingresos usuarias de la red pública de servicios de salud de la ciudad deMedellín, Colombia. Métodos: se realizaron ocho grupos focales (62 mujeres) según grupos de edad (25-45y 46-69 años) y se hizo un análisis de contenido. Resultados: las barreras del sistema de salud reportadasfueron: a) barreras estructurales: fragmentación en la prestación del servicio, problem...
La productividad forrajera (PF)es una variable crítica para el manejo y la planificación de los sistemas de producción ganaderos. Sin embargo, las dificultades para cuantificarla a campo y la necesidad de manejar un marco conceptual robusto para utilizarla en la toma de decisiones hacen que habitualmente los sistemas de producción se manejen con una caracterización rudimentaria de esta variable. En esta tesis se desarrolló un sistema de seguimiento de la PF en tiempo real y a la escala de lot...
Solano Moreno,Héctor Alfonso; San Roman Vazquez,Alejandro
Las lesiones en la médula espinal representan un porcentaje importante como causa de discapacidad en México y en el mundo, siendo la tercera causa con un porcentaje superior a 25%. Este tipo de pacientes sufre múltiples complicaciones y una de ellas es la que corresponde al sistema urológico al que muchas veces no se le da un seguimiento de manera regular. Dentro de las lesiones de las vías neurológicas, que inervan a la vejiga, pueden afectar a uno o varios aspectos de la fisiología, ya sea ...
Rodríguez, Andrea; Zunino, Carlos; Speranza, Noelia; Fernández, Salomé; Varela, Adriana; Picón, Teresa; Giachetto, Gustavo
Resumen Introducción: un evento adverso supuestamente atribuible a la vacunación o inmunización es aquel evento negativo que ocurre luego de administrar una vacuna y que puede ser atribuido a la misma. El objetivo fue caracterizar y realizar un seguimiento de los eventos adversos graves notificados al Sistema Nacional de Farmacovigilancia del Ministerio de Salud Pública durante 2010-2014. Material y método: se realizó un estudio transversal de la evolución de los individuos afectados, medi...
Lavedán Santamaría, Ana
Objetivo: Estimar la evolución de las caídas en los adultos mayores de 75 y más años de edad que residen en la comunidad, en la ciudad de Lleida. Métodos: Estudio descriptivo, prospectivo y longitudinal (una primera fase transversal y una segunda fase longitudinal con una media de seguimiento de dos años). La muestra total fue 640 individuos de 75 y más años de edad, residentes en la comunidad en la ciudad Lleida. Las variables dependientes fueron la historia de caída en el últ...
A. Ruiz Lascano
Full Text Available Las lesiones cutáneas por citomegalovirus (CMV son infrecuentes y a menudo una manifestación tardía de una enfermedad sistémica, que generalmente anuncia un curso fatal. Comunicamos un caso de celulitis por CMV: una mujer de 70 años con trasplante renal efectuado 1 mes antes de la consulta, terapia inmunosupresora con ciclosporina A y metilprednisona. La paciente ingresó por fiebre, dolor e impotencia funcional en pierna derecha. Comprobamos la existencia de una placa de 8 por 4 cm eritematoedematosa. La tratamos con antibióticos sin mejoría, por lo que realizamos un estudio histopatológico de piel que mostró cambios citopáticos compatibles con infección por CMV. Los cultivos bacteriológicos y micológicos fueron negativos. La inmunohistoquímica específica para CMV y el estudio de reacción en cadena de la polimerasa (PCR de la biopsia de piel fueron positivas, al igual que la antigenemia. El tratamiento con ganciclovir produjo la mejoría del cuadro clínico. En la literatura revisada no hemos encontrado la celulitis como manifestación de enfermedad cutánea por CMV.
Stefanou, M I; Komorowski, L; Kade, S; Bornemann, A; Ziemann, U; Synofzik, M
Myasthenia gravis is an autoimmune neuromuscular disorder, which has only rarely been reported to co-manifest with myositis. The diagnosis of concomitant myositis in patients with myasthenia gravis is clinically challenging, and requires targeted investigations for the differential diagnosis, including EMG, autoantibody assays, muscle biopsy and, importantly, imaging of the mediastinum for thymoma screening. This report presents a case-vignette of a 72-year-old woman with progressive proximal muscle weakness and myalgias, diagnosed with thymoma-associated myasthenia and bioptically verified granulomatous myositis, with positive autoantibody status for ryanodine receptor and titin antibodies. The diagnosis of concurrent myositis and myasthenia gravis, especially in the presence of ryanodine receptor and titin antibodies, should lead neurologists to adopt different treatment strategies compared to those applied in myasthenia or myositis alone. Moreover, further evidence is warranted that titin and, particularly, ryanodine receptor antibodies may co-occur or be pathophysiologically involved in myasthenia-myositis cases.
Yan, Chong; Song, Jie; Pang, Song; Yi, Fangfang; Xi, Jianying; Zhou, Lei; Ding, Ding; Wang, Weifeng; Qiao, Kai; Zhao, Chongbo
Repetitive nerve stimulation (RNS) is a valuable diagnostic method for myasthenia gravis (MG). However, its association with clinical severity was scarcely studied. We reviewed medical records and retrospectively enrolled 121 generalized MG patients. Sensitivity of different muscles to RNS and clinical scoring systems was evaluated. RNS testing revealed facial muscles have the highest positive rate, followed by proximal muscles and distal muscles, with the palpebral portion of the orbicularis oculi muscle most sensitive. Amplitude decrement of compound muscle action potential (CMAP) in the palpebral portion of the orbicularis oculi muscle is related to quantitative myasthenia gravis (QMG) scores, MG-specific manual muscle testing (MMT) scores and myasthenia gravis-related activities of daily living (MG-ADL) scores. We suggest that RNS testing of the palpebral portion of the orbicularis oculi muscle is a potential assessment indicator in patients with generalized MG. Copyright © 2017 Elsevier Ltd. All rights reserved.
Rikihisa, Naoaki; Udagawa, Akikazu; Yoshimoto, Shinya; Ichinose, Masaharu; Kimura, Tomoe; Shimizu, Sara
To describe the clinical course and management of a patient with submucous cleft palate who developed myasthenia gravis (MG) as an adult and suffered recurrent hypernasality. Few reports have described MG patients undergoing pharyngeal flap surgery for velopharyngeal incompetence, and these have described only slight speech improvement in such patients. Case report. The patient underwent primary pushback palatoplasty and superiorly based pharyngeal flap surgery for submucous cleft and short palate at age 7. Hypernasality showed major improvement after initial surgery. At age 19, the patient developed MG that triggered the recurrence of velopharyngeal incompetence. After MG was treated, revision pushback palatoplasty was performed for velopharyngeal incompetence when the patient was 24 years old. Preoperatively and postoperatively, the patient was evaluated by the same speech-language-hearing therapists, each with at least 5 years of clinical experience in cleft palate speech. After the second pushback palatoplasty, hypernasality and audible nasal air emission during speech decreased to mild. Primary pushback palatoplasty and pharyngeal flap surgery were performed for the submucous cleft palate. Revision pushback palatoplasty improved velopharyngeal inadequacy induced by MG. Decreased perceived nasality positively influenced the patient's quality of life. Combined pushback palatoplasty and pharyngeal flap surgery is thus an option in surgical treatment for velopharyngeal inadequacy to close the cleft and the velopharyngeal orifice in cases of cleft palate and MG.
Pirronti, T.; Rinaldi, P.; Marano, P.; Batocchi, A.P.; Evoli, A.; Di Schino, C.
Purpose: To achieve a better understanding of the role of CT and MR imaging in the study of the mediastinum in patients with myasthenia gravis (MG). Material and Methods: Mediastinal CT and MR findings were correlated with the histopathological results in 104 thymectomized MG patients. Results: CT was performed in 104 patients; in 11 of them, MR was also carried out. 44 patients had hyperplasia at histology. On CT, thymic hyperplasia was confirmed in 16 cases, thymoma was diagnosed in 10 and a normal thymus in 18 (sensitivity 36%, specificity 95%). Of 52 patients with thymoma at histology, CT showed thymoma in 46, hyperplasia in 1, and normal thymus in 5. CT showed 88.5% sensitivity and 77% specificity for thymoma. In 10 patients with invasive thymoma, CT was indiscriminate, while invasiveness was detected in 7 cases at MR (70% sensitivity) and at CT in 1 case. Both CT and MR detected tumor recurrence in 5 cases, but the exact localization and degree of invasion were best defined by MR. Conclusion: In MG patients CT is a sensitive, specific and efficient modality for detecting thymoma, but is less so for detecting thymic hyperplasia. MR was shown to be accurate in detecting invasive thymoma both preoperatively and in postoperative follow-up
Florowski, Tomasz; Florowska, Anna; Chmiel, Marta; Adamczak, Lech; Pietrzak, Dorota; Ruchlicka, Magdalena
The objective of the study was to evaluate the use of PSE meat in the production of sterilized pork type canned meat in its own gravy. Canned meat products were produced with 50% of PSE meat as well as with 100% PSE meat, and compared with canned meat products of good quality (RFN). It was found that decreased quality of PSE meat had a small impact on the quality of canned meat products. Substitution of both 50% as well as the total quantity of RFN meat with PSE meat did not affect the course of the sterilization process, neither increase the quantity of excreted fat and jelly in canned meat. It also had no effect on the instrumentally-measured parameters of texture and neither did it affect different sensory quality features, including the overall desirability of the product. The PSE canned meat product were characterized by higher values of L* and b* color parameters. Copyright Â© 2016 Elsevier Ltd. All rights reserved.
Full Text Available The course of myasthenia gravis (MG is complicated by increased reports of cognitive defects in both human and animal models, which suggests potential central nervous system (CNS damage. We conducted a systematic review of the relationships between MG and cognitive function. This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA guidelines. Major databases were searched to examine the neuropsychological studies of adults with MG. Weighted effect sizes were pooled by cognitive domain. Eight studies representing 300 subjects were included. Eight cognitive domain categories were identified: (i Mini-Mental State Examination (MMSE, (ii language, (iii processing speed, (iv verbal learning and memory, (v visual learning and memory, (vi attention span, (vii response fluency, and (viii motor performance. Nine (cognitive domain categories, MMSE, language, processing speed, verbal learning and memory (except for delayed recall memory, and motor performance of 16 cognitive tasks revealed significant moderate effect sizes. Verbal logical-delayed memory, finger tapping with the preferred hand, and the Symbol Digit Modalities Test showed a greater magnitude relationship to cognitive function than did other specific cognitive domains. Verbal learning and memory seems to be the most significant affected according to cognitive domain categories. For MG, the ability of attention, response fluency, visual learning, and memory seems to be reserved. The MG patients seem to perform significantly worse than the non-MG controls in a range of cognitive domains. Our findings should be interpreted with caution because of the clinical and methodological heterogeneity of included studies.
Full Text Available Abstract Background Post-thymectomy pain in myasthenia gravis (MG patients can inhibit breathing and coughing. Inappropriate usage of analgesics may exacerbate respiratory inhibition and even cause myasthenic crisis. Flurbiprofen is a non-steroidal anti-inflammatory drug (NSAID that is commonly used to control moderate postoperative pain and is not associated with respiratory inhibition. We hypothesized that flurbiprofen may provide post-thymectomy pain relief without increasing the risk of complications in MG patients. Methods Two hundred MG patients underwent extended thymectomy from March 2006 to December 2010 and were randomly allocated to a flurbiprofen group (110 patients, 50 mg intravenous flurbiprofen axetil or a control group (90 patients, 100 mg intramuscular tramadol as postoperative analgesia. Visual analog scale (VAS pain score, heart rate, blood pressure, respiratory rate, pulse oximetry (SpO2, and adverse effects were recorded before and up to 24 h after drug administration. Results There were no significant differences in the preoperative clinical characteristics of the flurbiprofen and control (tramadol groups. Both flurbiprofen and tramadol significantly alleviated post-thymectomy pain (p p 2 in either group at all time points. Conclusions Post-thymectomy intravenous administration of flurbiprofen axetil provides safe and effective analgesia for MG patients.
Background Post-thymectomy pain in myasthenia gravis (MG) patients can inhibit breathing and coughing. Inappropriate usage of analgesics may exacerbate respiratory inhibition and even cause myasthenic crisis. Flurbiprofen is a non-steroidal anti-inflammatory drug (NSAID) that is commonly used to control moderate postoperative pain and is not associated with respiratory inhibition. We hypothesized that flurbiprofen may provide post-thymectomy pain relief without increasing the risk of complications in MG patients. Methods Two hundred MG patients underwent extended thymectomy from March 2006 to December 2010 and were randomly allocated to a flurbiprofen group (110 patients, 50 mg intravenous flurbiprofen axetil) or a control group (90 patients, 100 mg intramuscular tramadol) as postoperative analgesia. Visual analog scale (VAS) pain score, heart rate, blood pressure, respiratory rate, pulse oximetry (SpO2), and adverse effects were recorded before and up to 24 h after drug administration. Results There were no significant differences in the preoperative clinical characteristics of the flurbiprofen and control (tramadol) groups. Both flurbiprofen and tramadol significantly alleviated post-thymectomy pain (p flurbiprofen group had significantly lower VAS pain scores at 0.5 h, 2 h, 4 h, and 8 h after surgery (p flurbiprofen axetil provides safe and effective analgesia for MG patients. PMID:23020939
Roberto D. Rey
Full Text Available Durante la observacion de 50 pacientes con Myasthenia gravis entre los anos 1974 y 1987 se encontro un predominio del sexo femenino en la proporcion de 2,5 a 1 y mayor frecuencia de presentacion en Ias 3ª y 5ª décadas de la vida. Luego de la evaluacion clínica, el diagnóstico fué confirmado mediante: (1 prueba dei edrofonio, (2 estímulo nervioso repetitivo, (3 dosaje de anticuerpos antirreceptor colinérgico y (4 transferencia pasiva del suero al raton con ulterior niedicion de la amplitud de mepp's. La positividad diagnostica vario entre el 90 y el 100%, segun el tipo de prueba empleada. El estúdio radiológico del timo fué hecho con neumomediastinografía. obteniendose excelente correlación con la descripcion histológica de la glândula, y con tomografia computada, que demostro menor eficiência diagnostica. El tratamiento fué implementado en base a anticolinesterásicos, timectomía y corticoterapia inmunosupresora, evidenciandose con esta última mejores resultados. Nueve pacientes desarrollaron peoria transitória del cuadro muscular al iniciarse el tratamiento esteroideo, 6 de ellos exhibieron posteriormente evolucion desfavorable. Esta observacion aparenta tener valor pronóstico en la evolucion de la MG.
Salvado, Maria; Canela, Merce; Ponseti, Jose Maria; Lorenzo, Laura; Garcia, Cecilia; Cazorla, Sonia; Gili, Gisela; Raguer, Nuria; Gamez, Josep
Myasthenia gravis (MG) is an autoimmune disease caused by a failure of neuromuscular transmission. Familial clustering has been reported despiteMG usually manifesting as a sporadic condition presumed not to be inherited. Our study investigated the prevalence of FAMG in a Spanish cohort, characterizing their phenotype,antibody titres and thymus findings. We investigated the presence of familial cases in 462 MG patients, characterizing by age and MGFA class at debut, quantitative MG score, antibody titres, MGFA post-intervention status and thymus pathology. Sixteen cases from8 unrelated pedigrees were identified. The prevalence of FAMG caseswas 3.46%.Mean age at onset was 57.8 ± 17.4 years (range=23–82). Distribution at debut was: 6 ocular, 4 IIa, 4IIb, 1 IIIa and 1 IIIb. Thymoma was identified in two of the 7 thymectomized individuals. The prevalence of FAMG in Spain is similar to other populations. Post-intervention status did not differ from sporadic autoimmune MG. As in other neuromuscular disorders, phenotype and inheritance heterogeneity are present in FAMG. In addition to the interfamilial heterogeneity observed, members of the same family affected with FAMG may even present different ages of onset, severity and thymus involvement. Further studies are necessary to clarify the role of genetic risk factors in this form of autoimmune MG.
Csuka, Dorottya; Czirják, László; Hóbor, Renáta; Illes, Zsolt; Bánáti, Miklós; Rajczy, Katalin; Tordai, Attila; Füst, George
Controversy exists about the effectiveness of vaccine-induced immune response in patients with immunoregulatory disorders. Our aim was to determine the antibody titers to diphtheria and tetanus in patients with either of two autoimmune diseases. 279 patients with SLE (205 females, aged 45.0 ± 13.8 years), 158 patients with myasthenia gravis (MG) (101 females, aged 55 ± 18.7 years) and 208 healthy subjects (122 females, aged 48 ± 14.6 years) were enrolled. Serum concentrations of diphtheria-antitoxin-IgG (A-DIPHTH) and tetanus-antitoxoid-IgG (A-TET) were determined with ELISA. Equal proportions of healthy subjects, as well as patients with SLE or MG exhibited proper antibody responses and immune protection against diphtheria and tetanus. In all three test groups, serum concentration of A-DIPHTH decreased significantly (p60-years-old) subjects. There were no significant differences among the groups in the age-related changes of A-TET and A-DIPHTH except that in diphtheria and tetanus infections in patients with SLE or MG is comparable to the healthy population. Copyright © 2013 Elsevier Ltd. All rights reserved.
Full Text Available Previous studies addressing preoperative steroid treatment have revealed that control of myasthenia gravis (MG with steroids prior to surgery appeared to stabilize postoperative status. The purpose of our study was to clarify the clinical benefits of the preoperative programmed high-dose steroid treatment on the long-term outcomes of MG patients. We retrospectively reviewed the records of 171 MG patients who were followed up after undergoing thymectomy in our hospital between 1988 and 2006. One hundred and thirteen patients in the programmed treatment group had received preoperative steroid treatment, while 58 patients received no steroid treatment during the preoperative period. Clinical remission, which was defined as the achievement of the modified pharmacologic remission (PR for at least 1 year, and clinical benefits were compared between the two groups. With regard to the remission after thymectomy, Kaplan-Meier life-table curves for patients in the preoperative steroid treatment group versus those for patients in the no steroid preoperative treatment group revealed a significantly higher probability of the PR in the preoperative steroid treatment group (log-rank test, P<0.01. This study might be the first, as per our knowledge, to indicate that preoperative programmed high-dose steroid treatment has long-term beneficial effects for MG patients.
Higashi, Yasuto; Ono, Shimato; Yasuda, Takeshi; Morimoto, Kenji; Terao, Akira; Shirabe, Teruo; Yokobayashi, Tsuneo (Kawasaki Medical School, Kurashiki, Okayama (Japan))
We here report on skeletal-muscle CT at the thigh level as studied using a whole-body CT scanner, with special reference to polymyositis (PM) and myasthenia gravis (MG). Early diseased muscles appeared homogenous and were likely to be almost normal. The first sign of muscular atrophy was the appearance of small, patchy or linear, low-density tissues in several muscles. These low-density tissues gradually increased in number until finally the diseased muscles were totally replaced by low-density tissue. These pathological findings were more severe in PM than in MG. There was a maldistribution of low-density tissue in several cases of PM. According to these findings, skeletal-muscle CT was thought to be of great help for the recognition of the general condition of muscles and for the follow-up on the patients. We think skeletal-muscle CT has a very practical application for the better selection of suitable muscular biopsy and EMG sites and for the better clinical interpretation of these findings.
Bray, J.J.; Drachman, D.B.
Antibodies directed against acetylcholine (ACh) receptors are present in the sera of nearly 90% of patients with myasthenia gravis (MG), and are involved in the pathogenesis of this autoimmune disease. However, the antibody titers measured by the standard radioimmunoassay correspond poorly with the clinical severity of the disease. To determine whether this disparity could be accounted for by differences in the binding affinities of anti-ACh receptor antibodies in different patients, we have measured the binding affinities of these autoantibodies in 15 sera from MG patients. The affinity constants (K/sub o/), as determined by Scatchard analysis, were all in the range of 10/sup 10/ M/sup -1/, comparable to the highest values reported in immunized animals. The affinity constants were truly representative of the population of autoantibodies detected by the radioimmunoassay, as shown by the remarkable linearity of the Scatchard plots (r/sup 2/>0.90) and the close correlation between the antibody titers determined by extrapolation of the Scatchard plots and by saturation analysis (r = 0.99; p < 0.001). There was only a 6-fold variation in affinity constants measured in this series of patients despite widely differing antibody titers and severity of the disease. Factors other than the titer and affinity of anti-ACh receptor antibodies may correlate better with the clinical manifestations of MG.
Mori, Shuuichi; Motohashi, Norio; Takashima, Rumi; Kishi, Masahiko; Nishimune, Hiroshi; Shigemoto, Kazuhiro
Since the first report of experimental animal models of myasthenia gravis (MG) with autoantibodies against low-density lipoprotein receptor-related protein 4 (LRP4), there have not been any major reports replicating the pathogenicity of anti-LRP4 antibodies (Abs). Recent clinical studies have cast doubt on the specificity and pathogenicity of anti-LRP4 antibodies for MG, highlighting the need for further research. In this study, we purified antigens corresponding to the extracellular region of human LRP4 stably expressed with chaperones in 293 cells and used these antigens to immunize female A/J mice. Immunization with LRP4 protein caused mice to develop myasthenia having similar electrophysiological and histological features as are observed in MG patients with circulating Abs against muscle-specific kinase (MuSK). Our results clearly demonstrate that active immunization of mice with LRP4 proteins causes myasthenia similar to the MG induced by anti-MuSK Abs. Further experimental and clinical studies are required to prove the pathogenicity of anti-LRP4 Abs in MG patients. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.
Full Text Available Introduction. Cardiac involvement of myasthenia gravis (MG accompanies a poor prognosis. In the present study, we aimed to investigate the relationship between ECG abnormality and cardiac involvement. Methods. Of 178 patients diagnosed with MG between 2001 and 2013 at our hospital, we retrospectively analyzed consecutive 58 patients who underwent both ECG and echocardiography and without underlying cardiovascular disease. ECG abnormalities were defined by computer-assigned Minnesota-codes. Cardiac damage was defined as either (1 ejection fraction (EF 8 on echocardiography. Results. Thirty-three patients (56.8% had ECG abnormality. An elevated E/e′ was observed in patients with ECG abnormality compared to those without ECG abnormality (11.2±3.2, 8.7±2.2, resp., p=0.03. Among patients with ECG abnormality, 14 of 15 patients showed cardiac damage. Among patients without ECG abnormality, 6 of 33 patients showed cardiac damage (p=0.003. Reduced EF was observed in five patients (8.6% with ECG abnormality and none in patients without ECG abnormality. Conclusions. ECG may aid as the first step for the further examination of cardiac damage in patients with MG.
Qi, Guoyan; Gu, Shanshan; Liu, Peng; Yang, Hongxia; Dong, Huimin
Myasthenia gravis (MG) is a kind of autoimmune disease induced by transferring dysfunction of neuromuscular junction. In the present study, we developed an integrated therapy combined with traditional Chinese medicine and immuno suppressive agents to seek for an effective treatment of MG. 220 MG patients were randomly divided into two groups with different therapies. Plasma levels of acetylcholine receptors antibodies (AchRAb) and CD4+CD25+ regulatory T cells (CD4+CD25+Treg) were conducted through ELISA and flow cytometry. The amount of AchRAb (8.52±0.96 vs. 5.22±0.46) and CD4+CD25+Treg (1.94±1.21 vs. 3.21±0.96) in Group A receiving integrated therapy were significantly improved compared with Group B; the clinical performance of group treated with the integrated therapy was also much better. The integrated therapy in the present study could significantly improve the condition of MG with high recovery rate and low recurrence rate, which can be employed in future clinical treatment of MG. PMID:26770531
Noridomi, Kaori; Watanabe, Go; Hansen, Melissa N.; Han, Gye Won; Chen, Lin (USC)
The nicotinic acetylcholine receptor (nAChR) is a major target of autoantibodies in myasthenia gravis (MG), an autoimmune disease that causes neuromuscular transmission dysfunction. Despite decades of research, the molecular mechanisms underlying MG have not been fully elucidated. Here, we present the crystal structure of the nAChR α1 subunit bound by the Fab fragment of mAb35, a reference monoclonal antibody that causes experimental MG and competes with ~65% of antibodies from MG patients. Our structures reveal for the first time the detailed molecular interactions between MG antibodies and a core region on nAChR α1. These structures suggest a major nAChR-binding mechanism shared by a large number of MG antibodies and the possibility to treat MG by blocking this binding mechanism. Structure-based modeling also provides insights into antibody-mediated nAChR cross-linking known to cause receptor degradation. Our studies establish a structural basis for further mechanistic studies and therapeutic development of MG.
Full Text Available Biomarkers that assess treatment response for patients with the autoimmune disorder, myasthenia gravis (MG, have not been evaluated to a significant extent. We hypothesized the pro-inflammatory cytokine, osteopontin (OPN, may be associated with variability of response to glucocorticoids (GCs in patients with MG. A cohort of 250 MG patients treated with standardized protocol of GCs was recruited, and plasma OPN and polymorphisms of its gene, secreted phosphoprotein 1 (SPP1, were evaluated. Mean OPN levels were higher in patients compared to healthy controls. Carriers of rs11728697*T allele (allele definition: one of two or more alternative forms of a gene were more frequent in the poorly GC responsive group compared to the GC responsive group indicating an association of rs11728697*T allele with GC non-responsiveness. One risk haplotype (AGTACT was identified associated with GC non-responsiveness compared with GC responsive MG group. Genetic variations of SPP1 were found associated with the response to GC among MG patients.
Hokezu, Youichi; Kaseda, Syun; Arimura, Kimiyoshi; Osame, Mitsuhiro; Baba, Kuniaki (Kagoshima Univ. (Japan). Faculty of Medicine); Ohkubo, Koichi; Hagiwara, Hiroshi
Seven patients with myasthenia gravis (MG) were examined to find thymus or thymoma employing chest radiographs, computed tomography (CT), pneumomediastinography (PMG), computed tomography after pneumomediastinography (PMG-CT) and magnetic resonance imaging (MRI). X-ray CT examination could reveal thymus only in half out of 6 cases scanned. On the other hand, MRI confirmed thymus or thymoma in 6 out of 7 patients. PMG and PMG-CT confirmed thymus or thymoma clearly in all of the 4 cases studied. PMG and PMG-CT examinations revealed thymus or thymoma more clearly than MRI. MRI is, however, an examination causing no pain to the patients and also more superior to X-ray CT in distinguishing between a thymus and mediastinal fat or vascular structure. In addition, MRI could reveal even capsules in thymoma which were never revealed by X-ray CT. We concluded that MRI could be an alternative method to CT and PMG in detection of thymus or thymoma in MG. (author).
Higashi, Yasuto; Ono, Shimato; Yasuda, Takeshi; Morimoto, Kenji; Terao, Akira; Shirabe, Teruo; Yokobayashi, Tsuneo
We here report on skeletal-muscle CT at the thigh level as studied using a whole-body CT scanner, with special reference to polymyositis (PM) and myasthenia gravis (MG). Early diseased muscles appeared homogenous and were likely to be almost normal. The first sign of muscular atrophy was the apperance of small, patchy or linear, low-density tissues in several muscles. These low-density tissues gradually increased in number until finally the diseased muscles were totally replaced by low-density tissue. These pathological findings were more severe in PM than in MG. There was a maldistribution of low-density tissue in several cases of PM. According to these findings, skeletal-muscle CT was thought to be of great help for the recognition of the general condition of muscles and for the follow-up on the patients. We think skeletal-muscle CT has a very practical application for the better selection of suitable muscular biopsy and EMG sites and for the better clinical interpretation of these findings. (author)
Hou Xiuyu; Xu Yonggang; Gao Hong; Li Ming; Li Gaofeng; Liu Mingyuan
Objective: To evaluate the change of myasthenia gravis(MG) during radiotherapy for patients with malignant thymomas. Methods: Forty-five with malignant thymomas patients with were analyzed. The median total dose was DT54.2 Gy in 1.8-2.0 Gy/fraction, 5 clays a week. Anti-cholinesterase, such as pyridostigmine was used to control the MG symptoms. Results: Forty-five patients completed radiotherapy on schedule except one from whom the treatment was was with drawn because of respiratory muscle involvement. Among these 44 patients, myasthenic symptom was relieved in 4 to various degrees, 4 progressed, 34 no change and 2 developed cholinergic crisis. Myasthenic symptom was not changed in one patient for whom radiotherapy had been standed before operation nor during the course of postoperative radiotherapy. Conclusions: A course of radiotherapy of DT54.2 Gy, on fractionation of DT1.8-2.0 Gy modal would not aggravate myasthenia. However, proper use of anti-cholinesterase, careful observation and timely drug-adjustment are necessary. (authors)
Hokezu, Youichi; Kaseda, Syun; Arimura, Kimiyoshi; Osame, Mitsuhiro; Baba, Kuniaki; Ohkubo, Koichi; Hagiwara, Hiroshi.
Seven patients with myasthenia gravis (MG) were examined to find thymus or thymoma employing chest radiographs, computed tomography (CT), pneumomediastinography (PMG), computed tomography after pneumomediastinography (PMG-CT) and magnetic resonance imaging (MRI). X-ray CT examination could reveal thymus only in half out of 6 cases scanned. On the other hand, MRI confirmed thymus or thymoma in 6 out of 7 patients. PMG and PMG-CT confirmed thymus or thymoma clearly in all of the 4 cases studied. PMG and PMG-CT examinations revealed thymus or thymoma more clearly than MRI. MRI is, however, an examination causing no pain to the patients and also more superior to X-ray CT in distinguishing between a thymus and mediastinal fat or vascular structure. In addition, MRI could reveal even capsules in thymoma which were never revealed by X-ray CT. We concluded that MRI could be an alternative method to CT and PMG in detection of thymus or thymoma in MG. (author)
Priola, A.M.; Priola, S.M.
Myasthenia gravis (MG) is an autoimmune disorder often associated with thymic abnormalities. At onset, thymic lymphoid hyperplasia (TLH) and thymoma can be found in up to 65% and 15% of patients, respectively. Diagnostic imaging is crucial in this setting in order to detect the presence and type of the thymic abnormality and in the preoperative planning, when indicated. Chest radiography has a minor role due to its low accuracy. Computed tomography is the imaging modality of choice, although the differentiation between a small thymoma and TLH that appears as a focal soft-tissue mass may be not possible. Magnetic resonance imaging (MRI) is not usually employed, but it is useful in equivocal cases, especially in differentiating focal TLH from thymoma by using chemical-shift sequences for defining the proper management. In addition, diffusion-weighted (DW)-MRI can differentiate lipid-poor normal/hyperplastic thymus from thymoma and could be useful in differentiating non-advanced from advanced thymomas. Positron emission tomography (PET)-CT is not helpful in distinguishing early from advanced thymoma but can be used to differentiate thymic carcinoma from thymoma. Hereby, we discuss the imaging features of thymic abnormalities in MG, even focusing on novel aspects of chemical-shift and DW-MRI
Tanimura, Shigeo; Banba, Jiro; Masaki, Mikio; Irimoto, Masahiro; Matsushita, Akira
Comparison of radiographical findings of the thymus between pneumomediastinography and computed tomography were studied in 35 patients with myasthenia gravis. The patients consisted of 10 patients with thymoma and 25 without thymoma confirmed at the operations. Pneumomediastinography was very useful to discern whether the thymoma was invasive or noninvasive, but not contributory to know whether the thymus was composed of folicular lymphoid hyperplasia or normal thyimic tissues. Computed tomography was also useful to dertermine the localization and the invasiveness of the thymoma, but not helpful to know whether the thymus was of follicular lymphoid hyperplasia or normal tissues. However, the finding of ''reticular pattern''-many small nodules scattered resicularly in the thymus-in computed tomography could be regarded as a sign suggesting follicular lymphoid hyperplasia of the thymus. Therfore, both pneumomediastinography and computed tomography were very useful in the diagnosis of the localization and the invasiveness of the thymoma but not for the diagnosis of follicular lymphoid hyperplasia of the thymus. Neverethless the finding of ''reticular pattern'' computred tomography was helpful in the diagnosis of follicular lymphoid hyperplasia.
Mantegazza, Renato; Antozzi, Carlo
The prognosis for patients with myasthenia gravis (MG) has improved significantly over the past half century, including substantial reductions in mortality and morbidity. However, approximately 10% of patients fails to respond adequately to current therapies and are considered treatment refractory, or treatment intolerant, and up to 80% have disease that fails to achieve complete stable remission. Although patients with autoantibodies to muscle-specific tyrosine kinase (anti-MuSK positive) are more likely to become treatment refractory than those with autoantibodies to the acetylcholine receptor (anti-AChR positive), each of these serotypes is substantially represented in the refractory MG population. Other risk factors for becoming treatment refractory include history of thymoma or thymectomy and female sex. A modified treatment algorithm for MG is proposed: patients who have disease that fails to respond to the stepwise approach to therapy, are treatment intolerant, or who require chronic rescue measures despite ongoing therapy, should be considered treatment refractory and emerging therapies should be considered. Three emerging monoclonal antibody-based therapies are discussed: the anti-B-cell agent rituximab; the terminal complement activation inhibitor eculizumab; and belimumab, which targets B-cell activating factor. Increased understanding of molecular pathophysiology and accurate antibody subtyping in MG should lead to the use of new therapeutic agents and successful management of treatment-refractory patients. PMID:29403543
A Arturo eLeis
Full Text Available Eye trauma and blindness are common in the United States, with an incidence of over 2 million cases per year and 25 million blind adults, respectively. However, literature is surprisingly scarce on the potential confounding effect of eye trauma or blindness on the diagnosis of myasthenia gravis (MG, an autoimmune neuromuscular disease in which fluctuating ocular symptoms are the most distinguishing feature. We present the case of a 75 year-old man with eye enucleation referred for electrodiagnostic evaluation of the right upper limb after an accidental fall. Neurological examination showed proximal muscle weakness, but MG was not initially considered because the patient lacked the classic ocular symptoms of MG. The delay in diagnosis resulted in worsening of systemic MG symptoms, although in other patients it may have precipitated MG crisis or possibly death. Greater awareness that eye trauma or blindness can prevent expression of ocular symptoms in neuromuscular disorders is needed to avoid morbidity associated with an erroneous or delayed diagnosis.
Pirronti, T.; Rinaldi, P.; Marano, P. [Univ. Cattolica del S. Cuore, Roma (Italy). Inst. of Radiology; Batocchi, A.P.; Evoli, A.; Di Schino, C. [Univ. Cattolica del S. Cuore, Roma (Italy). Inst. of Neurology
Purpose: To achieve a better understanding of the role of CT and MR imaging in the study of the mediastinum in patients with myasthenia gravis (MG). Material and Methods: Mediastinal CT and MR findings were correlated with the histopathological results in 104 thymectomized MG patients. Results: CT was performed in 104 patients; in 11 of them, MR was also carried out. 44 patients had hyperplasia at histology. On CT, thymic hyperplasia was confirmed in 16 cases, thymoma was diagnosed in 10 and a normal thymus in 18 (sensitivity 36%, specificity 95%). Of 52 patients with thymoma at histology, CT showed thymoma in 46, hyperplasia in 1, and normal thymus in 5. CT showed 88.5% sensitivity and 77% specificity for thymoma. In 10 patients with invasive thymoma, CT was indiscriminate, while invasiveness was detected in 7 cases at MR (70% sensitivity) and at CT in 1 case. Both CT and MR detected tumor recurrence in 5 cases, but the exact localization and degree of invasion were best defined by MR. Conclusion: In MG patients CT is a sensitive, specific and efficient modality for detecting thymoma, but is less so for detecting thymic hyperplasia. MR was shown to be accurate in detecting invasive thymoma both preoperatively and in postoperative follow-up.
Komatsu, Midori; Tanaka, Makoto; Morimatsu, Mitsunori; Hirai, Shunsaku; Heshiki, Atsuko (Gunma Univ., Maebashi (Japan). School of Medicine)
We investigated whether CT had any advantage over pneumomediastinography (PMG) for the visualization of thymus in twenty-one patients with myasthenia gravis (MG). In two cases of thymoma which had been suspected with chest radiography, CT demonstrated an anterior mediastinal tumor distinguishable from other mediastinal organs, and for these cases PMG was not performed. Excluding three patients who were submitted to PMG only, CT of the anterior mediastinum was carried out in sixteen patients of MG, revealing thymic shadows in seven (44%). PMG followed by conventional tomography was done subsequently in three of these seven cases, for whom thymectomy was indicated because of uncontrollable myasthenic symptoms, and in all patients finger-like thymic shadows were disclosed. The vertical extension of thymus was more easily demonstrable by PMG than CT. PMG was carried out in six of nine patients in whom CT was negative, and in all cases thymic shadows were obvious with subsequent conventional tomography. Consequently, false negative rate of CT was at least 38% (6/16) with regard to the visualization of the nontumorous thymus. Although CT of the anterior mediastinum is useful as a screening method because of its non-invasiveness, its negative result does not rule out an absence of the pathologic thymus in view of its high false negative rate. In this regard, PMG is still necessary for the final determination of the thymic configuration in the MG patients.
Oksenberg, J.R.; Cavalli-Sforza, L.L.; Steinman, L.; Sherritt, M.; Bernard, C.C.; Begovich, A.B.; Erlich, H.A.
Polymorphic markers in genes encoding the α chain of the human T-cell receptor (TcR) have been detected by Southern blot analysis in Pss I digests. Polymorphic bands were observed at 6.3 and 2.0 kilobases (kb) with frequencies of 0.30 and 0.44, respectively, in the general population. Using the polymerase chain reaction (PCR) method, the authors amplified selected sequences derived from the full-length TcR α cDNA probe. These PcR products were used as specific probes to demonstrate that the 6.3-kb polymorphic fragment hybridizes to the variable (V)-region probe and the 2.0-kb fragment hybridizes to the constant (C)-region probe. Segregation of the polymorphic bands was analyzed in family studies. To look for associations between these markers and autoimmune diseases, the authors have studied the restriction fragment length polymorphism distribution of the Pss I markers in patients with multiple sclerosis, myasthenia gravis, and Graves disease. Significant differences in the frequency of the polymorphic V α and C α markers were identified between patients and healthy individuals
Full Text Available Somsak Tiamkao,1,2 Sineenard Pranboon,3 Kaewjai Thepsuthammarat,4 Kittisak Sawanyawisuth1,5,6 1Department of Medicine, Faculty of Medicine, 2The Neuroscience Research and Development Group, 3Nursing Division, Srinagarind Hospital, 4Clinical Epidemiology Unit, Faculty of Medicine, 5Research Center in Back, Neck, Other Joint Pain and Human Performance (BNOJPH, 6Ambulatory Medicine Research Group, Khon Kaen University, Khon Kaen, Thailand Background: Myasthenia gravis (MG in elderly populations is increasing. This study aimed to evaluate predictors for treatment outcomes in elderly hospitalized MG patients using the national database. Methods: We collected data of elderly hospitalized MG patients from the National Health Security Office from October 2009 to September 2010. Predictors for treatment outcomes were examined. Results: During the study period, 1,948 identified MG patients were admitted to hospitals throughout Thailand. Of those, 441 patients (22.64% were aged ≥ 60 years. There were 66 patients (14.97% who had poor outcomes. There were only three significant factors in the final model. Presence of pneumonia, use of mechanical ventilators, and septicemia had adjusted odds ratios (95% confidence interval of 2.83 (1.03, 7.75, 5.33 (2.24, 12.72, and 4.47 (1.86, 10.75, respectively. Conclusion: Pneumonia, being on a mechanical ventilator, and septicemia were independent factors associated with poor treatment outcomes in elderly hospitalized MG patients according to national data. Keywords: pneumonia, ventilator, mortality, predictor
Full Text Available Abstract Introduction The association of thymoma with myasthenia gravis (MG is well known. Thymoma with sarcoidosis however, is very rare. We presented an interesting case with coexisting thymoma, MG and sarcoidosis. Case presentation A 59-year-old female patient with a history of sarcoidosis was admitted to the hospital with a one-day history of sudden onset of right-sided partial ptosis and diplopia. Neurosarcoidosis with cranial nerve involvement was considered, but was ruled out by the clinical findings, and MG was confirmed by the positive tensilon test, electrophysiological findings and positive acetylcholine receptor binding antibodies. On further evaluation, a CT chest scan showed a left anterior mediastinal mass and bilateral lymphadenopathy. Post surgical diagnosis confirmed the thymoma and sarcoidosis in the lymph nodes. Conclusion When two or more diseases of undetermined origin are found together, several interesting questions are raised. It is important to first confirm the diagnoses individually. Immunologic mechanisms triggering the occurrence of these diagnoses together, are difficult to address. Although the coexistence of thymoma, MG and sarcoidosis may be coincidental, it is noteworthy to report this case because of the multiple interesting features observed as well as the rarity of occurrence.
Full Text Available Introduction: There are many adverse effects that have been described for isotretinoin. To the best of our knowledge, this is the first report of a possible association of oral isotretinoin intake with autoimmune thyroiditis and ocular myasthenia gravis (OMG. Case Presentation: A 19-year-old Caucasian male, who had used oral isotretinoin for severe acne disease for the previous six months, was referred to our clinic. He had a three-week history of diplopia and variable bilateral ptosis. Physical examination showed moderate periorbital edema and limitations of up- and down-gaze in the left eye. Laboratory findings and thyroid ultrasound were consistent with autoimmune thyroiditis. Antithyroid therapy did not relieve the clinical symptoms. Concomitant OMG was suspected. Variable ptosis and a positive response to oral prednisolone of 40 mg/day and pyridostigmine of 360 mg/day supported the diagnosis of concomitant autoimmune thyroiditis and OMG. Conclusion: Autoimmune disorders may be triggered by oral isotretinoin treatment. Clinicians prescribing isotretinoin should be aware of the possible association between isotretinoin intake and concomitant autoimmune thyroiditis and OMG.
Aline de Almeida Xavier Aguiar
Full Text Available 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% were females and 37 (30.3% 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% (n=6 persisted as ocular myasthenia. Thymectomy was performed in 42.6% (n=52 of myasthenic patients. A thymoma was present in 10 patients. Serum acetylcholine receptor (AChR antibodies were present in 80% (n=20 of specimens tested. The data presented are similar to those of studies performed in other countries.
Análida Elizabeth Pinilla Roa
Full Text Available Se presenta un caso de un hombre de 81 años natural de zona rural, agricultor, recolector de café 50 años atrás y jardinero por 22 años en Bogotá. Consultó por dolor abdominal de 10 días de evolución, localizado hacia mesogastrio y epigastrio. Además, disnea, tos productiva. Antecedentes: enfermedad pulmonar obstructiva crónica, cor pulmonale, fumador de 25 paquetes /año, había recibido prednisona oral y beclometasona inhalada en diversas ocasiones incluso en la última hospitalización. En el examen físico presentó signos de desnutrición, taquicardia, taquipnea; estertores en base del hemitórax derecho; abdomen: blando, depresible con dolor a la palpación profunda en epigastrio e hipocondrio derecho. Ingresó con síndrome de respuesta inflamatoria sistémica por taquicardia, taquipnea, leucocitosis 37.600 y eosinofilia 52-60%, Ig E: 180 UI/ ml. Se evidenciaron larvas rabditiformes de Strongyloides stercoralis en esputo y materia fecal. La radiografía de tórax mostró derrame pleural derecho, la ecografía hepática fue normal, la endoscopia de vías digestivas altas evidenció gastritis y duodenitis. Se inició tratamiento con ivermectina 200 mg/Kg/dosis, con seguimiento clínico y de laboratorio con evolución clínica satisfactoria.
Grant, I.R.; Nixon, C.R.; Patterson, M.F.
Specific growth rates of two strains of Listeria monocytogenes in unirradiated and irradiated (2 kGy) roast beef and gravy stored at 5° and 10°C were found to be similar. However, exponential growth of L. monocytogenes after irradiation was preceded by an extended lag period of 6–9 d at 5°C and 3–4 d at 10°C, compared with lag periods of 1–2 d and <0.1 d in unirradiated beef and gravy stored similarly
Komasawa, Nobuyasu; Ueki, Ryusuke; Tomita, Yukihiko; Kaminoh, Yoshiroh; Tashiro, Chikara
We report a case of awake intubation utilizing Pentax-AWS Airwayscope in semi-sitting position. A 74-year-old man with myasthenia gravis and cervical disc hernia was scheduled for distal gastrectomy under general anesthesia. He could not move his head due to severe cervical disc hernia and also could not sufficiently breathe due to the fatigue of respiratory muscles by myasthenia gravis in supine position. With fentanyl bolus administration and lidocaine spray for laryngotracheal anesthesia, we performed awake intubation in semi-sitting position with AWS from cranial side. The patient did not buck during intubation and no hemodynamic change was observed.
Full Text Available El seguimiento preciso de la boca de una persona, cuando está hablando, es un desafío importante en varias aplicaciones, como la identificación de la cara o la interacción con el computador. La complejidad de forma, textura y color de la boca, y los cambios de iluminación y fondos de los posibles escenarios hacen que este sea aún un problema abierto. En este artículo se propone un algoritmo para el seguimiento del contorno externo de la boca, sin utilizar marcadores o alguna clase de maquillaje para resaltar los labios, basado en apariencia y en restricciones morfológicas definidas en el estándar MPEG-4. El algoritmo es robusto ante la presencia de barba, tono de piel y calidad de la imagen.An accurate tracking of a person's mouth when he/she is speaking is an important challenge in several applications such as face identification or interaction with computer. Complexity of shape, texture, and color of the mouth, as well as changes in lighting and backgrounds of possible scenarios makes of it an open problem yet. This article proposed an algorithm for a tracking of the mouth external contour without using markers or any kind of make-up for highlighting lips, based on appearance and morphological restrictions defined by the MPEG-4 Standard. Algorithm is robust before the presence of beard, skin tone, and image quality.
Iván Andrés Sánchez Ortiz; Tsunao Matsumoto
El objetivo principal de esta investigación fue evaluar el desempeño de la Planta de Tratamiento de Aguas Residuales de Ilha Solteira-SP durante las diferentes épocas climáticas del año. Se realizó un levantamiento batimétrico de las lagunas facultativas primarias, un monitoreo de 24 horas y el seguimiento de su desempeño en 3 etapas de colecta de 3 meses de duración cada una. La planta tuvo cerca del 40% de su volumen efectivo ocupado por lodos; el efluente final registró una eficiencia medi...
Ruiz Luna, David; Farran, L.; Ramos, E.; Biondo, Sebastián; Moreno Llorente, Pablo; Bettónica, C.; Jorba, R.; Borobia, F. G.; Jaurrieta Mas, Eduardo
Introducción: el tratamiento de la hemorragia digestiva alta por rotura de varices esofágicas y/o gástricas en pacientes con cirrosis hepática debe estar dirigido al control inicial de la hemorragia sin alterar más una función hepática ya deteriorada , y a la prevención de la recidiva hemorrágica precoz. Métodos endoscópicos, farmacológicos y quirúrgicos forman el conjunto de alternativas terapéuticas. Material y métodos: estudio prospectivo de los resultados obtenidos tras el seguimiento de...
Manoj K Sanwal
Full Text Available Myasthenia Gravis (MG is an acquired, autoimmune disorder affecting neuromuscular junction presenting with easy fatigability, progressive weakness, diplopia, difficulty in speaking and swallowing and even ventilatory failure in severe cases. During pregnancy the disease may go into remission or may exacerbate at any time during first, second and third trimesters or postpartum period. We are reporting the case of a 28 year old primigravida, known case of MG, who underwent caesarean section and developed muscular weakness on third postoperative day. Her neonate also had tachypnoea and hypotonia, Both, the mother and the baby were managed aggressively and responded well to therapy.
Pawan Kumar Jain
Full Text Available Myasthenia gravis is a chronic autoimmune disease affecting voluntary skeletal muscles. The altered sensitivity of acetylcholine receptors to muscle relaxants and concomitant treatment with anticholinesterase in these patients affect their anaesthetic management. Patients who have undergone bowel anastomosis and are on regular anticholinesterase treatment are susceptible to anastomotic leaks. We report successful anaesthetic management of class I myasthenic patient with coexisting small bowel intussusception operated for jejuno-ileal anastomoses using regional, inhalational and intravenous (i.v anaesthesia based on train of four responses, and avoiding the use of reversal (anticholinesterase.
Gardenia Edith Cedeño Marcillo
Full Text Available La evaluación de la pertinencia formativa de las instituciones de educación superior, a través de sus graduados, requiere de la aplicación de modelos y métodos que garanticen la mayor información posible, no sólo de orden cuantitativo, sino también de insumos que permitan un análisis crítico del contexto, las condiciones, circunstancias, aspiraciones y necesidades que tanto los egresados universitarios como el contexto laboral y social, presentan a las instituciones de educación superior. En este sentido, se persigue como uno de los propósitos de este trabajo, presentar algunos aportes de los principales autores que han direccionado los procesos de evaluación de la educación, desde los enfoques cualitativo y cuantitativo de la evaluación, destacando las utilidad de ambos paradigmas, en una indagación de carácter social, como son los estudios de egresados; lo que invita a reflexionar sobre la pertinencia y utilidad de una metodología sistemática, descriptiva, critica o valorativa, pero sobre, todo susceptible al análisis e inferencia de la realidad social en la que se desempeñan los graduados universitarios. La indagación de evidencias que permitan conocer la calidad de la formación recibida, en su relación con las necesidades del contexto social y laboral, son la base fundamental para la consolidación de estrategias, de las instituciones de educación superior, para aportar a la construcción de una sociedad más justa y equitativa para todos y todas.PALABRAS CLAVES: perspectiva social; evaluación; seguimiento a graduados; modelos cuantitativo y cualitativo.SOCIAL ASSESSMENT PERSPECTIVE. ITS EXPRESSION IN GRADUATE FOLLOW-UPABSTRACTThe formative evaluation of the relevance of higher education institutions, through its graduates, requires the application of models and methods to ensure the best possible information, not only quantitative, but also inputs to a critical analysis of context, conditions, circumstances
Full Text Available Introducción: Las heridas producidas por armas de fuego que comprometen la región cervical son materia de controversia en cuanto a cuál es el mejor método para tratarlas. La resolución endovascular de este tipo de patología ha cobrado gran relevancia en los últimos años debido al desarrollo de nuevos dispositivos y, consecuentemente, a la menor incidencia de complicaciones inherentes al procedimiento. Objetivo: Determinar el rol del tratamiento endovascular en la resolución de las lesiones cervicales por arma de fuego. Material y métodos: Se incluyeron 76 pacientes que sufrieron lesiones cervicales por arma de fuego, a los cuales se les realizó angiografía de los vasos supraaórticos para decidir la conducta a seguir. Resultados: Del total de 76 pacientes, 47 (61,8% presentaron una angiografía patológica; de estos, en 26 se decidió la resolución endovascular de la lesión, implantándose 1,1 stents por paciente. La estadía intrahospitalaria fue en promedio de 10,4 días. A los 30 días de seguimiento, 2 pacientes presentaron secuela neurológica y uno evolucionó con sepsis. En el seguimiento alejado con un promedio de 4 años se registró una muerte a los 117 días de realizado el procedimiento. Conclusiones: La intervención endovascular ha ganado mayor protagonismo con el desarrollo de nuevos dispositivos que permiten el tratamiento de patologías cada vez más complejas y reducir el tiempo de internación intrahospitalaria, como también las complicaciones relacionadas con el procedimiento.
Tinoco Hernandez, Rosanna; Paredes Bejarano, Margarita; Romero Chaglia, Norman; Yapur Auad, Miguel Eduardo
El presente trabajo trata sobrees el diseño y la implementación de un graficador de señales cardiacas por computadora, para lo cual diseñamos un circuito electrónico capaz de recibir la senal analógica proveniente de la actividad electrica del corazón , amplificarla, y luego convertirla en una señal digital para ser procesada por software y finalmente ser graficada, siendo posible así observar la señal cardiaca en el monitor de un computador como un tren de pulsos. Cabe destacar que par...
Masías-Arias, Lubeth; Fiestas-Teque, Luis
Se presenta el caso de un adolescente de 14 años con diagnóstico de trastorno de déficit de atención e hiperactividad que empezó a presentar síntomas psicóticos al tercer día de tratamiento con metilfenidato, los cuales no remitieron a pesar del retiro de dicho estimulante. Se inició tratamiento antipsicótico, al sexto día del retiro, por la intensidad y persistencia de los síntomas. Los síntomas psicóticos persistieron por más de 8 meses de seguimiento del paciente por lo cual se le hizo dia...
Satomi, M; Kimura, B; Hayashi, M; Okuzumi, M; Fujii, T
A novel species that belongs to the genus Marinospirillum is described on the basis of phenotypic characteristics, phylogenetic analysis of 16S rRNA and gyrB gene sequences and DNA-DNA hybridization. Four strains of helical, halophilic, Gram-negative, heterotrophic bacteria were isolated from kusaya gravy, which is fermented brine that is used for the production of traditional dried fish in the Izu Islands of Japan. All of the new isolates were motile by means of bipolar tuft flagella, of small cell size, coccoid-body-forming and aerophilic; it was concluded that they belong to the same bacterial species, based on DNA-DNA hybridization values (>70% DNA relatedness). DNA G+C contents of the new strains were 42-43 mol% and they had isoprenoid quinone Q-8 as the major component. Phylogenetic analysis of 16S rRNA gene sequences indicated that the new isolates were members of the genus Marinospirillum; sequence similarity of the new isolates to Marinospirillum minutulum, Marinospirillum megaterium and Marinospirillum alkaliphilum was 98.5, 98.2 and 95.2%, respectively. Phylogenetic analysis based on the gyrB gene indicated that the new isolates had enough phylogenetic distance from M. minutulum and M. megaterium to be regarded as different species, with 84.7 and 78.7% sequence similarity, respectively. DNA-DNA hybridization showed that the new isolates had <36% DNA relatedness to M. minutulum and M. megaterium, supporting the phylogenetic conclusion. Thus, a novel species is proposed: Marinospirillum insulare sp. nov. (type strain, KT=LMG 21802T=NBRC 100033T).
Ou, C Y; Ran, H; Qiu, L; Huang, Z D; Lin, Z Z; Deng, J; Liu, W B
Objective: To investigate the clinical features of the Pre-Crisis State and analyze the correlated risk factors of Pre-Crisis State of myasthenia crisis. Methods: We included 93 patients with myasthenia gravis (MG) who experienced 127 times Pre-Crisis State between October 2007 and July 2016. Those patients were hospitalized in the MG specialize center, Department of Neurological Science, first Affiliated Hospital of Sun Yat-sen University. The information of the general situation, the clinical manifestations and the blood gas analysis in those patients were collected using our innovated clinical research form. Statistic methods were applied including descriptive analysis, univariate logistic analysis, multivariate correlation logistic analysis, etc. Results: (1)The typical features of MG Pre-Crisis State included: dyspnea (127 times, 100% not requiring intubation or non-invasive ventilation), bulbar-muscle weakness (121 times, 95.28%), the increased blood partial pressure of carbon dioxide (PCO(2)) (94 times, 85.45%), expectoration weakness (99 times, 77.95%), sleep disorders (107 times, 84.25%) and the infection (99 times, 77.95%). The occurrence of dyspnea in combination with bulbar-muscle weakness ( P =0.002) or the increased blood PCO(2) ( P =0.042) often indicated the tendency of crisis. (2) The MG symptoms which were proportion to the occurrence of crisis includes: bulbar-muscle weakness ( P =0.028), fever ( P =0.028), malnutrition ( P =0.066), complications ( P =0.071), excess oropharyngeal secretions ( P =0.005) and the increased blood PCO(2) ( P =0.007). The perioperative period of thymectomy would not increase the risk of crisis. Conclusions: Dyspnea indicates the occurrence of the Pre-Crisis State of MG. In order to significantly reduce the morbidity of myasthenia crisis, the bulbar-muscle weakness, the increased blood PCO(2), expectoration weakness, sleep disorders, infection & fever and excess oropharyngeal secretions should be treated timely.
Braz, Nayara Felicidade Tomaz; Rocha, Natalia Pessoa; Vieira, Érica Leandro Marciano; Gomez, Rodrigo Santiago; Barbosa, Izabela Guimarães; Malheiro, Olívio Brito; Kakehasi, Adriana Maria; Teixeira, Antonio Lucio
This current study aimed to evaluate the frequency of low bone mass, osteopenia, and osteoporosis in patients with myasthenia gravis (MG) and to investigate the possible association between bone mineral density (BMD) and plasma levels of bone metabolism markers. Eighty patients with MG and 62 controls BMD were measured in the right femoral neck and lumbar spine by dual-energy X-ray absorptiometry. Plasma concentrations of osteocalcin, osteopontin, osteoprotegerin, tumor necrosis factor (TNF-α), interleukin (IL)-1β, IL-6, dickkopf (DKK-1), sclerostin, insulin, leptin, adrenocorticotropic hormone, parathyroid hormone, and fibroblast growth factor (FGF-23) were analyzed by Luminex®. The mean age of patients was 41.9 years, with 13.5 years of length of illness, and a mean cumulative dose of glucocorticoids 38,123 mg. Patients had significant reduction in BMD of the lumbar, the femoral neck, and in the whole body when compared with controls. Fourteen percent MG patients had osteoporosis at the lumbar spine and 2.5% at the femoral neck. In comparison with controls, patients with MG presented lower levels of osteocalcin, adrenocorticotropic hormone, parathyroid hormone, sclerostin, TNF-α, and DKK-1 and higher levels of FGF-23, leptin, and IL-6. There was a significant negative correlation between cumulative glucocorticoid dose and serum calcium, lumbar spine T-score, femoral neck BMD, T-score, and Z-score. After multivariate analysis, higher TNF-α levels increased the likelihood of presenting low bone mass by 2.62. MG patients under corticotherapy presented low BMD and altered levels of bone markers.
Suzuki, Shigeaki; Ishikawa, Nobuhisa; Konoeda, Fumie; Seki, Nobuhiko; Fukushima, Satoshi; Takahashi, Kikuko; Uhara, Hisashi; Hasegawa, Yoshikazu; Inomata, Shinichiro; Otani, Yasushi; Yokota, Kenji; Hirose, Takashi; Tanaka, Ryo; Suzuki, Norihiro; Matsui, Makoto
To report the clinical features of myasthenia gravis (MG) induced by treatment with immune checkpoint inhibitors using 2-year safety databases based on postmarketing surveys in Japan. We studied 10,277 patients with cancer who had received monotherapy with either nivolumab or ipilimumab between September 2014 and August 2016. As the control group, 105 patients with idiopathic MG were used. There were 12 MG cases (0.12%) among 9,869 patients with cancer who had been treated with nivolumab, but none among 408 patients treated with ipilimumab. These 12 patients included 6 men and 6 women with a mean age of 73.5 ± 6.3 years. MG onset occurred in the early phase after nivolumab treatment and rapidly deteriorated. Nivolumab-related MG (nivoMG) included 4 patients with mild involvement and 8 patients with severe involvement. Bulbar symptoms and myasthenic crisis were observed more frequently in nivoMG than idiopathic MG. Ten patients were positive for anti-acetylcholine receptor antibodies. Serum creatine kinase levels were markedly elevated to an average level of 4,799 IU/L. Among the 12 patients with nivoMG, 4 had myositis and 3 had myocarditis, with 1 of these patients having both. Immunosuppressive therapy was effective. Postintervention status showed that pharmacologic remission or minimal manifestations were obtained in 4 patients; however, 2 patients died. Immune-related adverse events triggered by nivolumab impaired the patients' daily living activity. The prompt and correct recognition of MG following treatment with immune checkpoint inhibitors in patients with cancer is important. © 2017 American Academy of Neurology.
Qi-Guang Shi; Zhi-Hong Wang; Xiao-Wei Ma; Da-Qi Zhang; Chun-Sheng Yang; Fu-Dong Shi; Li Yang
Objective To evaluate the frequency,distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor (AChR) in patients with myasthenia gravis (MG).Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG (OMG) (n =90) and generalized MG (GMG) patients (n =110).The fetaltype (2α∶ β∶ γ∶ δ) and adult-type (2α∶ β∶ ε∶ δ) AChR were used as antigens,and their relevance to disease presentation was assessed.Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined,with 14 having serum specific to the AChR-γ subunit,and 22 to the AChR-ε subunit.The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only (P =0.015).Compared with OMG patients,the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG.Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio (OR),1.03;95％ confidence interval (CI),1.01-1.06 and OR,5.09;95％ CI,2.23-11.62].Conclusion Using both fetal-and adulttype AChRs as the antigens may be more sensitive than using either subtype.Patients with serum specific to both isoforms are at a greater risk of progressing to GMG.Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.
Peltier, Amanda C.; Black, Bonnie K.; Raj, Satish R.; Donofrio, Peter; Robertson, David; Biaggioni, Italo
We report a case of a 55 year old man with non-small cell lung cancer who underwent radiation, chemotherapy with carbotaxol and paclitaxel, and left upper lobe removal two years prior to evaluation. He was referred for disabling orthostatic hypotension (113/69 supine, 66/47 mmHg standing after 10 minutes without a compensatory heart rate increase (57 to 59 bpm), fatigue, and constipation with episodes of ileus. Clinical examination showed mild ptosis bilaterally, fatiguable neck flexor weakness and hip flexor weakness. Blood pressure response to Valsalva maneuver was abnormal with absence of phase 4 overshoot and a Valsalva heart rate ratio of 1.04, The plasma norepinephrine level was low (79 pg/ml supine to 330 pg/ml standing). Single fiber EMG of the right extensor digitorum communis revealed normal mean MCD (jitter) but several pairs exceeded a jitter of 100 µs. Antibodies against muscle acetylcholine receptor [(AChR) 0.66 nmol/L, normal <0.02] and ganglionic AChR (0.34 nmol/L, normal <0.02) were present. Treatment with plasma exchange normalized responses to standing posture (105/68 supine to 118/82 mmHg standing, 66 to 79 bpm), to Valsalva (normal blood pressure overshoot, HR ratio 1.47), norepinephrine (194 pg/ml supine, 763 standing), and jitter measurements. We conclude that autoimmune autonomic ganglionopathy and myasthenia gravis can coexist and suggest that the latter should be excluded in patients with autoimmune autonomic ganglionopathy who complain of fatigue that is improved with non-supine rest. PMID:19882640
Guidetti, D; Sabadini, R; Bondavalli, M; Cavalletti, S; Lodesani, M; Mantegazza, R; Cosi, V; Solime, F
We carried out a retrospective incidence, prevalence and mortality survey of myasthenia gravis in the province of Reggio Emilia in Northern Italy. Based on 49 patients, the mean incidence per year for the period 1980 through 1994 was 7.8 per 1,000,000. On 31 December 1994 the prevalence rate was 117.5 per 1,000,000 for all patients, either active or recovered (50 cases in a population of 427,493) and 103.4 per 1,000,000 for the active disease. In the 15-year period 1980-1994 the average mortality rate was 1.0 per 1,000,000 per year. The average age at onset was 44.6 +/- 21.0, and the average age at the time of prevalence determination was 51.1 +/- 19.6 for the active disease. At the time of diagnosis, 21 patients (36.8%) were classed in group I according to Osserman's criteria, 31 in group II (54.4%), (19 in group II-A and 12 in group II-B), and the other 5 (8.8%) in group III. Of all the prevalence cases, 6 (12%) were in remission without therapy and 6 with therapy, while most of the others 16 (32%) were classed in group I, 15 (30%) in group II, and 1 (2%) in group III. Thymectomy was performed in 20 patients (35.1%), 12 (21%) had thymoma (malignant in 4 cases), 6 had thymic hyperplasia while in two patients thymic histology was normal. The relation the grade of Osserman's scale at the time of incidence and the presence of thymoma were significant. Higher grades of Osserman's scale were associated were malignant thymoma. Furthermore the relationship between thymectomy and the grade of Osserman's scale at the date of prevalence was significant for the presence of lower grades of Osserman's scale in the patients submitted to thymectomy.
Chen, Xin-lin; Liu, Feng-bin; Guo, Li; Liu, Xiao-bin
To investigate the scientificity of patient-reported outcome (PRO) scale for myasthenia gravis (MG), which was used to evaluate the clinical effects of traditional Chinese and Western medicine treatment on MG patients. Psychometric performance of the MG-PRO scale was also expected to be evaluated in this study. A total of 100 MG patients and 100 healthy people were face-to-face interviewed by well-trained investigators, and the data of MG-PRO scale were collected. The classical theory test (CTT) and item response theory (IRT) methods were used to analyze the psychometric performance such as validity, reliability, person separation index (PSI) and differential item functioning (DIF) in the MG-PRO scale. The results of CTT analysis showed that the split-half reliabilities of the MG-PRO scale and each dimension were greater than 0.7. In the analysis of internal consistency of each dimension, the Cronbach's alpha was greater than 0.8. Each facet had greater correlation with its dimension than the other dimensions. Four principal components were extracted by exploratory factor analysis, which represented all dimensions of the scale, and the cumulative variance was 55.54%. The scores of each of the 8 facets between MG patients and healthy people were different (Pdefinition and connotation of quality of life and contains special issues of MG patients as well, and shows good reliability (split-half reliability, Cronbach's alpha), validity (content validity, construct validity, discriminate validity) from the results of CTT, and good psychometric performance from the results of IRT.
Apinyawasisuk, Supanut; Zhou, Xinkai; Tian, Jack J; Garcia, Giancarlo A; Karanjia, Rustum; Sadun, Alfredo A
Forced eyelid closure test (FECT) is a clinical screening test developed from the original Cogan lid twitch (CLT) sign to assist in the diagnosis of ocular myasthenia gravis (OMG), We evaluated the sensitivity and specificity of FECT compared with CLT and benchmarked to standard diagnostic tests. This study was a retrospective chart review of 48 patients using electronic medical records of those that presented with ptosis and/or diplopia at Doheny Eye Institute, University of California, Los Angeles between February 2015 and April 2016. Patients without FECT testing were excluded. FECT and CLT results, and final diagnosis were recorded. To perform FECT, the patient was asked to squeeze his or her eyelids shut for 5-10 seconds then open quickly and fixate in primary position. The excessive upward overshoot of eyelids movement indicated a positive FECT. The test was performed by a neuro-ophthalmologist before establishing the diagnosis. Patients who had equivocal test results and/or inconclusive final diagnosis were excluded. Of the 48 patients studied, 18 patients (37.5%) had positive FECT; 15 of whom had a final diagnosis of OMG (83.3%). Of the 30 patients with negative FECT, 1 had OMG (3.3%). Of the 48 patients, 35 patients also had a documented CLT result (72.9%). CLT was positive in 11 of these 35 patients (31.4%), and 9 of these 11 had OMG (81.8%). Of the 24 patients with negative CLT, 2 of them had OMG (8.3%). Sensitivity and specificity of FECT were 94% and 91% (joint 95% confidence region: sensitivity × specificity = [0.70, 1] × [0.75, 1]). The relative true-positive fraction (rTPF) between FECT and CLT was 1.15; the relative false-positive fraction was 1.31. FECT is a simple clinical screening test with good sensitivity and specificity for OMG.
Yi, JS; Guidon, A; Sparks, S; Osborne, R; Juel, VC; Massey, JM; Sanders, DB; Weinhold, KJ; Guptill, JT
Muscle specific tyrosine kinase myasthenia gravis (MuSK MG) is a form of autoimmune MG that predominantly affects women and has unique clinical features, including prominent bulbar weakness, muscle atrophy, and excellent response to therapeutic plasma exchange. Patients with MuSK MG have predominantly IgG4 autoantibodies directed against MuSK on the postsynaptic muscle membrane. Lymphocyte functionality has not been reported in this condition. The goal of this study was to characterize T-cell responses in patients with MuSK MG. Intracellular production of IFN-gamma, TNF-alpha, IL-2, IL-17, and IL-21 by CD4+ and CD8+ T-cells was measured by polychromatic flow cytometry in peripheral blood samples from 11 Musk MG patients and 10 healthy controls. Only one MuSK MG patient was not receiving immunosuppressive therapy. Regulatory T-cells (Treg) were also included in our analysis to determine if changes in T cell function were due to altered Treg frequencies. CD8+ T-cells from MuSK MG patients had higher frequencies of polyfunctional responses than controls, and CD4+ T-cells had higher IL-2, TNF-alpha, and IL-17. MuSK MG patients had a higher percentage of CD4+ T-cells producing combinations of IFN-gamma/IL-2/TNF-gamma, TNF-alpha/IL-2, and IFN-gamma/TNF-alpha. Interestingly, Treg numbers and CD39 expression were not different from control values. MuSK MG patients had increased frequencies of Th1 and Th17 cytokines and were primed for polyfunctional proinflammatory responses that cannot be explained by a defect in Treg function or number. PMID:24378287
Full Text Available Clinical practice guidelines (CPGs are systematically developed statements to assist practitioners in making decisions about appropriate healthcare in specific clinical circumstances. The methodological quality of CPGs for myasthenia gravis (MG are unclear.To critically evaluate the methodological quality of CPGs for MG using AGREE II instrument.A systematical search strategy on PubMed, EMBASE, DynaMed, the National Guideline Clearinghouse (NGC and the Chinese Biomedical Literature database (CBM was performed on September 20th 2013. All guidelines related to MG were evaluated with AGREE II. The software used for analysis was SPSS 17.0.A total of 15 CPGs for MG met the inclusion criteria (12 CPGs in English, 3 CPGs in Chinese. The overall agreement among reviews was moderate or high (ICC >0.70. The mean scores (mean ± SD for al six domains were presented as follows: scope and purpose (60.93% ± 16.62%, stakeholder involvement (40.93% ± 20.04%, rigor of development (37.22% ± 30.46%, clarity of presentation (64.26% ± 16.36%, applicability (28.19% ± 20.56% and editorial independence (27.78% ± 28.28%. Compared with non-evidence-based CPGs, evidence-based CPGs had statistically significant higher quality scores for all AGREE II domains (P0.05. The quality scores of CPGs developed by NGC/AAN were higher than the quality scores of CPGs developed by other organizations for all domains. The difference was statistically significant for all domains with the exception of clarity of presentation (P = 0.07.The qualities of CPGs on MG were generally acceptable with several flaws. The AGREE II instrument should be adopted by guideline developers, particularly in China.
Ramos-Fransi, A; Rojas-García, R; Segovia, S; Márquez-Infante, C; Pardo, J; Coll-Cantí, J; Jericó, I; Illa, I
Myasthenia gravis (MG) may become life-threatening if patients have respiratory insufficiency or dysphagia. This study aimed to determine the incidence, demographic characteristics, risk factors, response to treatment and outcome of these life-threatening events (LTEs) in a recent, population-based sample of MG patients. A retrospective analysis of MG patients who presented with an LTE between 2000 and 2013 was performed. Participants were identified from a neuromuscular diseases registry in Spain that includes 648 patients with MG (NMD-ES). Sixty-two (9.56%) patients had an LTE. Thirty-two were classified as class V according to the MG Foundation of America, and 30 as class IVB. Fifty per cent were previously diagnosed with MG and median duration of the disease before the LTE was 24 months (3-406). The most common related factor was infection (n = 18). All patients received intravenous human immunoglobulin; 11 had a second infusion and six had plasma exchange. Median time to feeding tube removal was 13 days (1-434). Median time to weaning from ventilation was 12 days (3-176), and it was significantly shorter in late onset MG (≥50 years) (P = 0.019). LTEs improved <2 weeks in 55.8% but did not improve until after 1 month in 20% of patients. Four patients died. No other factors influenced mortality or duration of LTEs. The percentage of LTEs in MG patients was low, particularly amongst those previously diagnosed and treated for the disease. The significant percentage of treatment-resistant LTEs indicates that more effective treatment approaches are needed for this vulnerable sub-population. © 2015 EAN.
Full Text Available El presente trabajo muestra los resultados de un seguimiento educativo a personas con diabetes mellitus tipo 2 y sobrepeso corporal u obesidad, realizado como continuidad del estudio latinoamericano PEDNID-LA, con el objetivo de evaluar, en forma prospectiva, los cambios clínicos, bioquímicos y terapéuticos obtenidos después de participar en un programa de educación grupal con metodología interactiva. El estudio incluyó 40 pacientes no insulino dependientes, obesos o en sobrepeso y preferentemente de reciente inicio de la enfermedad, seleccionados en orden cronológico de asistencia a la consulta ambulatoria. Se estableció una consulta interactiva trimestral, donde cuidados y educación se desarrollaban como una unidad, y el contenido educativo iba surgiendo de las necesidades reales y sentidas del grupo, lo que se centró en promover la participación activa del paciente y aumentar la adhesión al tratamiento, con prioridad del plan de alimentación, la actividad física, la reducción del peso corporal y el automonitoreo de glucosa en orina, tal como se había hecho con otros grupos de personas con diabetes. Los conocimientos sobre diabetes se midieron en cuestionarios idénticos diseñados en el protocolo general multicéntrico del estudio PEDNID-LA y aplicados al inicio y final de dicho estudio y a los 4 años del seguimiento interactivo. El resto de las variables fueron valoradas antes, después del año del estudio PEDNID-LA y a los 4 años del seguimiento interactivo. Los datos fueron procesados mediante el uso del programa Epi-Info. Las diferencias estadísticas se determinaron por prueba de Chi cuadrado, y se aceptó p The present paper showed the results of educational follow-up of persons with type 2 diabetes mellitus and overweight/obesity carried out as part of the Latin American study PEDNID-LA, with the objective of prospectively evaluating the clinical, biological and therapeutic changes after their participation in a
Full Text Available Myasthenia gravis is an autoimmune disorder in which antibodies have been shown to form against the nicotinic acetylcholine nicotinic postsynaptic receptors located at the neuromuscular junction. "Warming yang and invigorating qi" acupuncture treatment has been shown to reduce serum inflammatory cytokine expression and increase transforming growth factor beta expression in rats with experimental autoimmune myasthenia gravis. However, few studies have addressed the effects of this type of acupuncture on the acetylcholine receptors at the neuromuscular junction. Here, we used confocal laser scanning microscopy to examine the area and density of immunoreactivity for an antibody to the nicotinic acetylcholine receptor at the neuromuscular junction in the phrenic nerve of rats with experimental autoimmune myasthenia gravis following "warming yang and invigorating qi" acupuncture therapy. Needles were inserted at acupressure points Shousanli (LI10, Zusanli (ST36, Pishu (BL20, and Shenshu (BL23 once daily for 7 consecutive days. The treatment was repeated after 1 day of rest. We found that area and the integrated optical density of the immunoreactivity for the acetylcholine receptor at the neuromuscular junction of the phrenic nerve was significantly increased following acupuncture treatment. This outcome of the acupuncture therapy was similar to that of the cholinesterase inhibitor pyridostigmine bromide. These findings suggest that "warming yang and invigorating qi" acupuncture treatment increases acetylcholine receptor expression at the neuromuscular junction in a rat model of autoimmune myasthenia gravis.