Moyamoya disease

Česká verzia

Authors: V. Přibáň ¹; J. Dostál ¹ ; J. Mraček ¹; J. Baxa ²; P. Duras ²
Authors place of work: Neurochirurgická klinika, LF UK a FN Plzeň ¹; Klinika zobrazovacích metod, LF UK a FN Plzeň ²
Published in the journal: Cesk Slov Neurol N 2021; 84/117(2): 116-125
Category: Minimonografie
doi: https://doi.org/10.48095/cccsnn2021116

Summary

Moyamoya disease is bilateral progressive steno-occlusive impairment of the distal internal carotid artery accompanied by the formation of basal collaterals and finally by the exclusive collateralization from the territory of the external carotid artery. Suzuki angiographic classification describes progression of moyamoya disease. Aetiology is not known, but it is probably a combination of inherited and autoimmune factors. Asian population is mostly affected. Ischemic symptoms are typical in a pediatric population, and in adults, haemorrhage is a frequent symptom. Prognosis is poor. Therapy is exclusively surgical, either direct extra-intracranial bypass or indirect revascularization. Indirect techniques utilize potential of neoangiogenesis of vascularized tissue in the proximity of the brain. Combination of direct and indirect revascularization represents optimal treatment of symptomatic patients.

Keywords:

moyamoya disease – Etiology – revascularization techniques – extra-intracranial bypass

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