Moyamoya disease ("puff of smoke" in Japanese) is a chronic cerebrovascular disease affecting the terminal portion of the intracranial internal carotid arteries and the proximal part of the circle of Willis (arteries at the base of the brain). It leads to the development of an abnormal vascular network, whose angiographic appearance as a "puff of smoke" gave the disease its name. Clinically, it presents with recurrent ischemic and hemorrhagic strokes related to impaired cerebral perfusion. Description of the disease
The disease causes progressive narrowing of the arteries at the base of the skull, leading to the development of an abnormal collateral vascular network.
Clinically, the disease manifests as recurrent ischemic and hemorrhagic strokes due to impaired cerebral perfusion (vascularization).
Surgical management
Depending on the extent of the disease and its clinical impact, cerebral revascularization surgery may be required. It consists of an anastomosis (temporo-sylvian bypass) between an artery from the external carotid system and an artery from the internal carotid system, in order to restore sufficient perfusion to the affected cerebral territory.
Management involves close collaboration between neurologists, neurosurgeons, and neuroradiologists. The referring neurologist (Prof. Dominique HERVÉ) assesses and follows patients, then presents their case at a monthly multidisciplinary meeting to discuss, collectively, the indication for surgery if required.
CERVCO Reference Center
Our team works in collaboration with the neuroradiology and neurology departments within CERVCO — the Reference Center for rare diseases of the Brain and Eye Vessels. This center brings together the Lariboisière Hospital departments specializing in rare vascular disorders of the retina, spinal cord, and brain.
CERVCO missions
- Improve treatment nationwide
- Implement care protocols
- Disseminate information to patients, families, and caregivers
- Develop research for new therapies
Coordination
CERVCO coordinates its work in collaboration with the relevant family associations and all medical teams involved in the management of these rare diseases nationwide.
Useful links
Guide
Your stay is our priority, and we have established a patient pathway program for your surgical schedule, admission and intervention, through to your discharge. Please do not hesitate to contact us if you have any questions.