Adult-Onset Hemorrhagic Quasi-Moyamoya Disease with Unilateral Steno-occlusive Lesion in a Patient with Neurofibromatosis Type 1.

BACKGROUND

Quasi-moyamoya disease is a condition that occurs in association with a specific underlying condition or disease such as atherosclerotic disease or neurofibromatosis type 1 (NF1). Pediatric cases are frequently reported, and an ischemic and bilateral presentation is more common than a hemorrhagic and unilateral presentation.

METHODS

A 39-year-old woman previously diagnosed with NF1 presented to our department with nausea and left hemiparesis. She was diagnosed with right temporal intracerebral hemorrhage by initial computed tomography. Subsequent angiography showed an occlusion of the terminal portion of the right internal carotid artery, and magnetic resonance imaging showed multiple flow voids in the right basal ganglia, suggesting quasi-moyamoya disease. The hematoma was surgically removed, and her neurological condition improved after the operation.

CONCLUSIONS

This is the first reported case of quasi-moyamoya disease with a rare combination of characteristics, including an adult-onset, hemorrhagic presentation and a unilateral lesion in a patient previously diagnosed with NF1.