Abstract

Background: Moyamoya disease (MMD) is a rare pathology caused by a progressive unilateral or bilateral stenosis of the terminal portion of the internal carotid artery, leading to the development of collateral vessels. Case Presentation: We report a rare case of a 46-year-old male, born in the city of São Paulo, Brazil, with sudden muscular strength deficit and right hemiparesis, associated with headache and emesis. A priori, the initial diagnosis was arteriovenous malformation (AVM) after performing a series of complementary tests during the patient’s follow-up, also considering the clinical picture similar to that of Moyamoya disease (MMD). The conclusive diagnosis of MMD was finally established when the magnetic resonance imaging (MRI) showed a network of tortuous and dilated collateral vessels, with a hazy “smoke cloud” aspect with stenosis of the M1 segment of the middle cerebral artery (MCA), branch of the artery internal carotid artery (ICA). Conclusion: The recommended treatment was surgical revascularization with extracranial-intracranial bypass, with a favorable prognosis to the patient.

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