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CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2021; 40(03): e272-e276
DOI: 10.1055/s-0037-1602378
Case Report | Relato de Caso

Progressive Cerebral Arteriopathy – Moyamoya Disease: A Report of Two Cases with Different Clinical Presentation

Arteriopatia progressiva cerebral – Doença de moyamoya: Relato de dois casos com apresentações clínicas distintas
Marx Lima de Barros-Araújo
1   University Hospital, Universidade Federal do Piauí, Teresina, PI, Brazil
,
Tibério Silva Borges dos Santos
1   University Hospital, Universidade Federal do Piauí, Teresina, PI, Brazil
,
Irapuá Ferreira Ricarte
2   Institute of Neurosciences, Teresina, PI, Brazil
,
Guilherme Victor Sousa Medeiros
3   Federal University of Piauí, Teresina, PI, Brazil
,
Joemir Jabson da Conceição Brito
3   Federal University of Piauí, Teresina, PI, Brazil
,
Stephany Vargas Guindani
3   Federal University of Piauí, Teresina, PI, Brazil
,
Larissa Clementino Leite de Sá Carvalho
4   Diferencial Integral Faculty, Teresina, PI, Brazil
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Abstract

Moyamoya disease is a chronic and unusual cerebrovascular disorder characterized by progressive stenosis and occlusion of the distal portions of internal carotid arteries and its main branches within the circle of Willis. Posterior circulation (vertebral and basilar arteries) may also be affected; however, this presentation is uncommon. As well as stenosis of the terminal portion of intracranial arteries, it is seen the development of a network of collateral vessels abnormally dilated at the base of the brain with an aspect of a “puff of smoke,” whose term in Japanese is described as “moyamoya.” The present study aims to report two consecutive cases of patients who presented to our service with different clinical manifestations. Further investigation with digital subtraction angiography showed a moyamoya pattern.

Resumo

A doença de moyamoya (DMM) é uma desordem cerebrovascular crônica de rara incidência, caracterizada pela estenose progressiva das porções terminais das artérias carótidas internas, associada à proliferação de vasos colaterais anormalmente dilata dos na base do crânio, cujo aspecto se assemelha a uma “fumaça,” definido pelo termo em japonês “moyamoya.” A circulação posterior (artérias vertebrais e artéria basilar) também pode ser acometida, porém de forma menos frequente. A apresentação clínica é variada. O presente estudo objetiva relatar dois casos de pacientes que apresentaram diagnóstico angiográfico compatível com a DMM e manifestações clínicas distintas.

Keywords

cerebral arteriopathy - progressive vasculopathy - moyamoya

Palavras-chave

arteriopatia cerebral - vasculopatia progressiva - moyamoya


Publication History

Received: 10 February 2017

Accepted: 27 March 2017

Article published online:
28 April 2017

© 2017. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)

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