Moya-Moya disease is a rare occlusive entity, progressive with time and of scarce prevalence or global distribution. It is due to stenosis of the intracraneal portion of the internal carotid artery, with establishment of compensatory secondary intracraneal anastamoses at different levels (leptomeningeal, basal ganglion, and transdurals). There is a higher incidence during the first five years of life (juvenile form) or in the third decade (adult form). Its course is cerebral hypoperfusion, which in clinical terms translates into recurrent intracraneal haemorrhages and transient ischaemic attacks. With reference to our speciality, an elevated risk of complications during pregnancy has been described, with caesarean section being recommended before term.
La enfermedad de Moya-Moya es una rara entidadoclusiva cerebrovascular de escasa prevalencia y distribución mundial, progresiva en el tiempo. Se debe auna estenosis de la porción intracraneal de la arteriacarótida interna, con establecimiento secundario deanastomosis intracraneales compensatorios en distintas localizaciones (leptomeninges, ganglios basales ytransdurales)1-8. Presenta una mayor incidencia en losprimeros 5 años de vida (forma juvenil) o en la tercera década (forma adulta)9. Cursa, por tanto, con hipoperfusión cerebral, que se traduce clínicamente enepisodios de hemorragias intracraneales recurrentes yataques isquémicos transitorios. En lo que se refiere anuestra especialidad, se ha descrito un riesgo elevadode complicaciones durante el embarazo, recomendándose la finalización del mismo mediante cesárea.
