Brazilian Neurosurgery (Apr 2017)

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

  • Marx Lima de Barros-Araújo,
  • Tibério Silva Borges dos Santos,
  • Irapuá Ferreira Ricarte,
  • Guilherme Victor Sousa Medeiros,
  • Joemir Jabson da Conceição Brito,
  • Stephany Vargas Guindani,
  • Larissa Clementino Leite de Sá Carvalho

DOI
https://doi.org/10.1055/s-0037-1602378
Journal volume & issue
Vol. 40, no. 03
pp. e272 – e276

Abstract

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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.

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