[An association between moya-moya disease and morning glory anomaly]. / Asociación entre enfermedad de moya moya y anomalía de morning glory.

INTRODUCTION: The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. CASE REPORT: A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imaging were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a puff of smoke . This is a typical imaging for Moyamoya disease. CONCLUSIONS: In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease.

Asociación entre la enfermedad de moya-moya y la anomalía de morning glory / An association between moya-moya disease and morning glory anomaly

Introducción. La anomalía de morning glory (AMG) es una alteración congénita de la papila óptica poco frecuente, que se asocia a una disgenesia vascular retiniana. Se han descrito varios pacientes que presentan esta entidad y alteraciones cerebrovasculares compatibles con la enfermedad de moya-moya (EMM). Presentamos el caso de un niño con esta asociación. Caso clínico. Niño de 5 años, que consultó por haber presentado varios episodios de desconexión con el medio, en algunos casos precedidos de cefalea intensa y asociados a lenguaje incoherente, y, en otras ocasiones, a hipotonía y risa inmotivada, de duración variable. La exploración física y la neurológica fueron normales, salvo la papila del ojo derecho, que era muy grande y excavada, compatible con AMG. La tomografía computarizada craneal y el EEG fueron normales. En la resonancia magnética (RM) se evidenciaron múltiples imágenes puntiformes de vacío de señal en los núcleos de la base sugerentes de aumento de la vascularización y la angio-RM mostró estenosis en la porción distal de ambas carótidas internas, así como en los segmentos proximales de la arteria cerebral anterior y la arteria cerebral media izquierda, con un aumento de la circulación colateral en la zona de los núcleos de la base, compatible con la típica imagen en `volutas de humo', que confirmó el diagnóstico de EMM. Conclusión. En todo paciente diagnosticado de AMG, sobre todo si presenta sintomatología neurológica, se deberá valorar la realización de estudios de angio-RM, para descartar la existencia de EMM (AU)

Introduction. The morning glory disc anomaly is a not very frequent congenital alteration of the optic papilla associated with a retinal vascular dysgenesia. Several cases of association have been described between this disc anomaly and the presence of Moyamoya vessels. We report the case of a boy with this association. Case report. A 5 year-old boy was evaluated for several episodes of disconnection, some of them preceded by intense headaches and associated to incoherent language, and in other occasions with hypotonia and unmotivated laugh of variable duration. The physical and neurological exploration was normal, except for the optic disc papilla of the right eye that was very big and dug, compatible with Morning glory disc anomaly. No alterations were found at cranial CT and EEG. In the magnetic resonance imagine were visualized multiple small round low intensity areas in the basal ganglia, because of the high number of collateral vessels. The angioRM showed stenosis of the supraclinoid portions of both internal carotids as well as the proximal segments of the anterior cerebral artery and the left middle cerebral artery with abnormal network of collateral vessels at the base of the brain like a ‘puff of smoke’. This is a typical imagine for Moyamoya disease. Conclusions. In all patient with Morning glory disc anomaly, mainly if they presents neurological clinical features, the realization of angioRM studies will be evaluated, to discard the existence of Moyamoya disease (AU)