We report a case of moyamoya disease (MMD) associated with arterioveno
us malformation (AVM). The 30-year-old female patient presented with l
eft-hemispheric transient ischaemic attacks (TIAs) involving dysphasia
and right-sided hemiparesis. CT-scan acid lumbar puncture showed no e
vidence of intracranial haemorrhage. Cerebral angiography revealed typ
ical moyamoya vessels and occlusion of multiple cerebral arteries with
consecutive collateral blood supply. Moreover, a left-parietal AVM wi
th a diameter of approximately 2 cm was detected. An extra-intracrania
l arterial bypass (EIAB) connecting the left superficial temporal arte
ry (STA) with a cortical branch of the left middle cerebral artery (MC
A) was performed (STA-MCA anastomosis) and yielded subsequent resoluti
on of the neurological deficit. Nine months post-operatively neurologi
cal deficits similar to those of the initial presentation recurred. Re
peated angiography suggested comparatively increased AVM blood flow, a
nd successful extirpation of the AVM gradually re-established almost f
ull functional ability. However, deterioration of the neurological con
dition developed again. We herewith present the first European case of
moyamoya disease associated with arteriovenous malformation and repor
t the clinical course under an alternative neurosurgical treatment con
sisting of STA-MCA anastomosis and delayed extirpation of the AVM.