Ces radiol. 2020, 74(4):246-253 | DOI: 10.55095/CesRadiol2020/036

Moyamoya - radiodiagnostic and clinical characteristics of the rare diseaseReview article

Jiří Dostál¹, Radek Tupý², Jan Mraček¹, Filip Heidenreich², Vladimír Přibáň¹

¹ Neurochirurgická klinika LF UK a FN, Plzeň

² Klinika zobrazovacích metod LF UK a FN, Plzeň

Moyamoya is a rare disease which causes progressive stenosis of the terminal parts of both internal carotid arteries and their branches. During the course of the disease, a net of fragile collateralizing vessels forms in the territory of the middle cerebral artery. In the terminal stage, the brain blood supply from the internal carotid arteries is replaced by an exclusive blood supply from the transdural anastomoses from the external carotid arteries. The name is a Japanese expression for a "puff of smoke", which is resembled by the fine basal collateralizing vessels on the angiogram.
The occurrence of the disease is typical for Asian countries and it is rare in Europe. High morbidity and mortality is caused by ischaemic or hemorrhagic complications. In early diagnosed patients, severe course of the disease can be reversed .The treatment is exclusively surgical - it is based on various techniques of brain revascularization surgeries without damaging existing collateralizing vessels.
The gold standard of the diagnostics is the DSA. Based on the DSA, the stage and form of the disease can be assesed. New findings have strenghtened the role of the magnetic resonance imaging in the diagnostics of the disease and brought a new insight into the pathophysiology. The results of the brain functional studies are indispensable factors in the surgery indications. Non-invasive angiographic techniques on modern devices bring information about the extent of the disease and help with the pre-operative planning.
With regard to the numbers of published cases and claimed incidence, the authors believe, that part of the moyamoya patients can go unnoticed. The aim of this communication is to introduce imaging and clinical features of the rare moyamoya disease and contribute to detection of more cases in our population.

Keywords: basal collaterals, EC-IC bypass, ivy sign, revascularization, Suzuki classification

Grants and funding:

Práce byla podpořena MZ ČR - RVO (Fakultní nemocnice Plzeň - FNPl, 00669806).

Accepted: December 15, 2020; Published: December 1, 2020  Show citation