Eficacia y seguridad de la toxina botulínica en el tratamiento de la sialorrea en niños con diversas alteraciones neurológicas / Efficacy and safety of botulinum toxin in the treatment of sialorrhea in children with neurological disorders

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Síndrome de Marin-Amat y Marcus-Gunn invertido. Presentación de 2 casos de sincinesia facial / Marin-Amat and inverted Marcus-Gunn syndrome. Two case report

El síndrome de Marin-Amat es una sincinesia trigémino-facial generalmente adquirida, debida a una regeneración aberrante del nervio facial que produce una contracción simultánea de orbicular de los párpados y los pterigoideos externos. Clínicamente, se manifiesta con blefarospasmo al abrir la boca o sonreír. Existen casos congénitos conocidos como fenómeno de Marcus-Gunn invertido por tener clínica inversa, ptosis palpebral con la apertura bucal, pero con la misma sincinesia (III-VII). Presentamos 2 casos clínicos, uno adquirido secundario a cirugía de una adenitis cervical tuberculosa y otro congénito, de forma gráfica y haciendo referencia a la forma de presentación, evolución y el tratamiento. Concluimos que es una entidad infrecuente en pediatría, con pocos casos descritos, aunque probablemente infradiagnosticada por su desconocimiento. El diagnóstico es esencialmente clínico y no precisa pruebas complementarias para su confirmación, si bien, la electromiografía puede ser de gran utilidad para demostrar la sincinesia (AU)

Marin-Amat’s syndrome is a rare associated movement, wherein contraction of or bicularisoculi is brought about by opening of the jaw in association with aberrant regeneration of facial nerve. This is the opposite of the Marcus-Gunn phenomenon; hence it has also been called Inverted Marcus-Gunn Phenomenon. Moreover in this case the opened the mouth or moved the jaw, the eye would close. This phenomenon is congenital and the closure of the eye is due to inhibition of the levator palpebrae superior is. We present two cases, one acquired after the surgery of tuberculosus cervical adenitis and other congenital with reference to the presentation, course and treatment. This entity is rare in children, with few reported cases, although probably will be found more frequently if looked for carefully. The diagnosis is clinical and does not require additional tests, although EMG may be useful to demonstrate the synkinesis (AU)

[Marin-Amat and inverted Marcus-Gunn syndrome. Two case reports]. / Síndrome de Marin-Amat y Marcus-Gunn invertido. Presentación de 2 casos de sincinesia facial.

Marin-Amat's syndrome is a rare associated movement, wherein contraction of orbicularis oculi is brought about by opening of the jaw in association with aberrant regeneration of facial nerve. This is the opposite of the Marcus-Gunn phenomenon; hence it has also been called Inverted Marcus-Gunn Phenomenon. Moreover in this case the opened the mouth or moved the jaw, the eye would close. This phenomenon is congenital and the closure of the eye is due to inhibition of the levator palpebrae superioris. We present two cases, one acquired after the surgery of tuberculosus cervical adenitis and other congenital with reference to the presentation, course and treatment. This entity is rare in children, with few reported cases, although probably will be found more frequently if looked for carefully. The diagnosis is clinical and does not require additional tests, although EMG may be useful to demonstrate the synkinesis.