Moya Moya disease (meaning “smoke cloud” in Japanese) is a chronic cerebrovascular disease affecting the termination of the intracranial internal carotid arteries and the proximal part of the polygon of Willis (arteries at the base of the brain). It is caused by the development of an abnormal vascular network (arteries whose diameter narrows) at the base of the skull, whose angiogaphic “smoke cloud” appearance has given the disease its name.
Clinically, the disease manifests itself as repeated ischemic and hemorrhagic strokes due to impaired cerebral perfusion (vascularization).
Depending on the extent of the disease and its clinical impact, surgery may be required to revascularize a cerebral territory. It may therefore be necessary to propose an anastomosis between an artery of the external carotid system and an artery from the internal carotid system.
The management of Moya Moya disease requires close collaboration between neurologists, neurosurgeons and neuroradiologists.
Our neurosurgical team works in collaboration with the neuroradiology and neurology teams at the CERVCO (Centre de Référence pour les maladies rares des Vaisseaux du Cerveau et de l’Oeil). This reference center brings together departments at Hôpital Lariboisière specializing in the treatment of rare vascular pathologies of the retina, spinal cord and brain, including Moya Moya disease. The objectives of this center are to improve the treatment of these rare diseases on a national scale and in coordination with the family associations concerned, to set up protocols for the management of these conditions and to generally disseminate all useful information to patients, their families and all doctors or carers concerned, and finally to improve knowledge of the mechanisms of these conditions thanks to the research developed at the CERVCO. CERVCOwith the aim of developing new, effective therapies.
Monthly multi-disciplinary meetings are organized to discuss the problems posed by patients affected by Moya Moya disease and make collegial therapeutic decisions.
