Subarachnoid Hemorrhage as the Presenting Feature of Moyamoya Disease: a case report

Hemorragia Subaracnoidea como Forma de Presentación de la Enfermedad de Moyamoya: caso clínico

• Facundo Rodríguez    
• Camila Brignoni    
• Agustín Carámbula    
• Matías Negrotto    
• Pedro Grille    
• Marcelo Barbato    
• Federico Verga    

• https://doi.org/10.22290/jbnc.2026.14051144
• J Bras Neurocirur 37 (3):431-436, 2026

Resumo

La enfermedad de Moyamoya es un trastorno cerebrovascular crónico caracterizado por estenosis progresiva y eventual oclusión de las arterias carótidas internas, como resultado de hiperplasia de las células del músculo liso con trombosis luminal superpuesta. Las manifestaciones clínicas más frecuentes incluyen eventos cerebrovasculares isquémicos o hemorrágicos, cuyo fenotipo predominante varía según la edad de presentación. En los adultos predominan los eventos hemorrágicos, particularmente los hematomas intraparenquimatosos y la hemorragia intraventricular, mientras que la hemorragia subaracnoidea constituye una forma de presentación inicial poco frecuente. Presentamos un caso de enfermedad de Moyamoya con una presentación fulminante caracterizada por hemorragia subaracnoidea, intraparenquimatosa e intraventricular concomitantes.

Palavras-chave

Enfermedad de Moyamoya; Hemorragia subaracnoidea; Neuroimagen; Ecografía Doppler dúplex.

Abstract

Moyamoya disease is a chronic cerebrovascular disorder characterized by progressive stenosis and eventual occlusion of the internal carotid arteries, resulting from smooth muscle cell hyperplasia with superimposed luminal thrombosis. Clinical manifestations most commonly include ischemic or hemorrhagic stroke, with the predominant phenotype varying according to age at presentation. In adults, hemorrhagic events predominate, particularly intraparenchymal hematomas and intraventricular hemorrhage, whereas subarachnoid hemorrhage is an uncommon initial manifestation. We report a case of Moyamoya disease with a fulminant presentation characterized by concurrent subarachnoid, intraparenchymal, and intraventricular hemorrhage.

Keywords

Moyamoya disease; Subarachnoid hemorrhage; Neuroimaging; Duplex Doppler Ultrasonography.

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1Intensive Care Unit - ICU, Maciel Hospital, Administración de los Servicios de Salud del Estado - ASSE, Montevideo, Uruguay.

2Maciel Hospital, Administración de los Servicios de Salud del Estado - ASSE, Montevideo, Uruguay.

 

Received Jan 27, 2026 

Accepted Mar 21, 2026