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• W2940400218 abstract "Resumen La miastenia gravis (MG) es la enfermedad de la union neuromuscular mas frecuente. Se trata de un trastorno adquirido de base autoinmune, en el que se detectan diferentes anticuerpos contra elementos del receptor muscular postsinaptico. Los sintomas iniciales suelen aparecer en un grupo muscular aislado, preferentemente en la musculatura ocular con aparicion de ptosis y diplopia. Posteriormente, los sintomas progresan con afectacion muscular generalizada, apareciendo una afectacion bulbar con sintomas de disfagia y disartria, debilidad de la musculatura cervical y a nivel proximal de las extremidades. Si la progresion clinica de la MG continua, los pacientes desarrollan una crisis miastenica, asociandose fallo respiratorio. El rasgo clinico mas caracteristico de la MG es la variabilidad de la debilidad, con fluctuaciones clinicas dentro del mismo dia, en relacion con el ejercicio o factores agravantes, alcanzando en ocasiones una remision espontanea. La sospecha clinica es clave tras reconocer este patron clinico y confirmar el diagnostico, principalmente con la determinacion en sangre de los anticuerpos caracteristicos de la MG acompanada del estudio neurofisiologico. El avance en el conocimiento de la inmunopatologia subyacente ha permitido la optimizacion de las estrategias terapeuticas, combinando inhibidores de la acetilcolinesterasa, inmunosupresores y timectomia, con la consiguiente disminucion en su morbimortalidad. Myasthenia gravis (MG) is the most common disease of the neuromuscular junction. It is an acquired autoimmune disease, in which several antibodies against postsynaptic receptors are detected. Initial symptoms often manifest focally, mainly ocular weakness resulting in ptosis and diplopia. Subsequently, symptomatology evolves to generalized muscular involvement and bulbar symptoms: dysphagia and dysarthria, cervical musculature and proximal limb weakness. Clinical progression results on myasthenic crisis and respiratory failure. The clinical hallmark of MG is a fluctuating pronounced muscular weakness along the same day and related with physical activity or aggravating factors. Occasionally, spontaneous remissions have been reported. Diagnosis key is clinical suspicion achieving by clinical pattern recognition. Diagnostic confirmation is obtained by serological (MG antibodies) and neurophysiological tests. Thanks to advances in the knowledge about underlying immunopathology, the range of therapeutic strategies has grown (combining acetylcholinesterase inhibitors with immunosuppressant drugs and thymectomy), decreasing its morbidity and mortality." @default.
• W2940400218 created "2019-04-25" @default.
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• W2940400218 date "2019-04-01" @default.
• W2940400218 modified "2023-09-27" @default.
• W2940400218 title "Miastenia gravis y trastornos relacionados con la unión neuromuscular" @default.
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• W2940400218 doi "https://doi.org/10.1016/j.med.2019.04.001" @default.
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