Moyamoya syndrome, from image to diagnosis. Presentation of a case

Abstract

Moyamoya disease and Moyamoya syndrome refer to a progressive steno-occlusive vasculopathy in the terminal portions of the bilateral internal carotid arteries and/or the proximal parts of the middle and anterior cerebral arteries, with prominent formation of collateral arteries. Being able to generate irreversible damage to cerebral hemodynamics due to the progressive nature of the affected cerebral vessels, the pathological examination does not show atherosclerotic or inflammatory lesions and the cause of the stenosis is the overgrowth of the smooth muscle layer, with thrombotic changes.

The disease leads not only to a different degree of stenosis and occlusions of the great arteries in the anterior part of the circle of Willis, but also to the development of collateral vasculature that produces a typical angiographic image, termed 'puffs of smoke' or 'puff'. of cigarette smoke'.

We present the case of a 28-year-old female patient who presents with insidious symptoms and a previous wrong clinical and imaging diagnosis, in whom a follow-up magnetic resonance of the brain was performed, evidencing findings by imaging and angioresonance compatible with Moyamoya disease; the imaging findings of the syndrome in the different modalities of radiological studies are analyzed.