Progression of arteriovenous malformation in moyamoya syndrome

We report a case of moyamoya disease (MMD) associated with arteriovenous malformation (AVM). The 30-year-old female patient presented with left-hemispheric transient ischaemic attacks (TIAs) involving dysphasia and right-sided hemiparesis. CT-scan acid lumbar puncture showed no evidence of intracranial haemorrhage. Cerebral angiography revealed typical moyamoya vessels and occlusion of multiple cerebral arteries with consecutive collateral blood supply. Moreover, a left-parietal AVM with a diameter of approximately 2 cm was detected. An extra-intracranial arterial bypass (EIAB) connecting the left superficial temporal artery (STA) with a cortical branch of the left middle cerebral artery (MCA) was performed (STA-MCA anastomosis) and yielded subsequent resolution of the neurological deficit. Nine months post-operatively neurological deficits similar to those of the initial presentation recurred. Repeated angiography suggested comparatively increased AVM blood flow, and successful extirpation of the AVM gradually re-established almost full functional ability. However, deterioration of the neurological condition developed again. We herewith present the first European case of moyamoya disease associated with arteriovenous malformation and report the clinical course under an alternative neurosurgical treatment consisting of STA-MCA anastomosis and delayed extirpation of the AVM.