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Pediatría (Asunción)

On-line version ISSN 1683-9803


ACUNA F, Ángel R  and  O. DE GODOY, Cristina. Moya-Moya Desease. Pediatr. (Asunción) [online]. 2010, vol.37, n.1, pp.42-47. ISSN 1683-9803.

Moya-Moya is a progressive steno-occlusive disease of the internal carotid arteries and its major branches (middle cerebral artery, and anterior cerebral artery) whose cause is unknown, and which is reported primarily in east Asia (Japan and Korea) but rare in Paraguay and the rest of the world. In children under age 10, it presents most commonly as a transient or permanent ischemic attack and in adults as a hemorrhagic stroke. Diagnosis is done by digital subtraction cerebral angiography, and treatment consists of cerebral revascularization to improve the neurological profile and quality of life. We present the case of a 3-year old female child with seizures and motor aphasia who was lucid, with left hemiparesis, and who after complete recovery presented again a week later with a more severe attack, with impaired consciousness and right hemiplegia. Radiological study revealed a typical image of swirls of 'cigarette smoke' at the base of the brain that are characteristic of Moya-Moya disease, and who was operated on using encephaloduroarteriosynangiosis (EDAS), with good results.

Keywords : Moya-Moya disease; cerebrovascular accident; cerebral revascularization; digital subtraction angiography.

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