INTRODUCTION Juvenile myasthenia gravis (JMG) is an infrequent autoimmune disease, the symptoms and therapeutic handling of which do not differ from those of the adult forms. Chronic treatment with corticoids very often causes side effects in childhood, which is why patients are being submitted to thymectomies at younger ages with better results. AIMS. To analyse the clinical and evolutionary profile of JMG treated by thymectomy in our centre.
PATIENTS AND METHODS We report the case of four girls aged between 5 and 13 who were diagnosed as suffering from generalised myasthenia gravis (MG) with bulbar affectation. One of them started with a myasthenic crisis. The four of them were submitted to Tensilon’s test, an electrophysiological study, determination of AChR, thoracic CT, and study of autoimmunity and thyroid functioning. After surgery the thymus was analysed histologically.
RESULTS They all gave positive in Tensilon’s test and were seropositive for AChR. They were treated with anticholinesterases, up to the maximum tolerated dose, and corticoids, without complete remission being accomplished and so they were submitted to a thymectomy in the first year of evolution. In three cases surgical approach was transsternal and in the other by means of a videothoracoscope. All the thymuses showed lymphoid hyperplasia. After a variable follow-up the girls are at present asymptomatic, although none of them has been able to completely give up the pharmacological treatment.
FULL TEXT(solo disponible en lengua castellana / Only available in Spanish)
Si ya es un usuario registrado en Neurologia, introduzca sus datos de inicio de sesión.
Rellene los campos para registrarse en Neurologia.com y acceder a todos nuestros artículos de forma gratuita
¿Olvidó su contraseña? Introduzca su correo electrónico y le haremos llegar una nueva
¡CONVIÉRTASE EN USUARIO PREMIUM DE NEUROLOGIA.COM!
Al hacerse premium, está apoyándonos para que Revista de Neurología
siga siendo uno de los referentes de habla hispana en la difusión del
conocimiento en neurociencias. ¡Gratuita tanto para autores como para
todos los usuarios de la web!
Además, por convertirte en usuario premium, recibirá las siguientes ventajas:
Plaza asegurada en todos nuestros Másteres (www.ineurocampus.com)
Descuento del 5% en los cursos de “Actualización en Neurología”, la FMC que estará disponible próximamente en la web.
Descarga gratuita en formato PDF dos de las obras con más éxito publicadas por Viguera Editores:
Oromotors Disorders in childhood (M. Roig-Quilis; L. Pennington)
Manual de Neuropsicología 2ª ed. (J. Tirapu-Ustárroz; M. Ríos-Lago; F. Maestú)
El precio para hacerse Premium durante el periodo de un año es de 5€, que podrá pagar a continuación a través de una pasarela de pago seguro con tarjeta de crédito, transferencia bancaria o PayPal:
OPEN ACCESS
