Cerebral arteriovenous malformations (AVMs) come to clinical attention mainly in young adults in their mid-thirties. The chief symptoms and signs are focal or generalized seizures, episodic or chronic headaches, progressive neurological deficits and AVM rupture with intracerebral, intraventricular and/or subarachnoid haemorrhage.

There are few data in the literature about the natural history of the disease. No controlled studies are available ascertaining the risks of invasive treatment (endovascular, surgical or radiotherapeutic).

This review focuses on the clinical features of AVMs and their management. It discusses possible predictors of adverse events in the natural history and the benefits and risks of invasive treatment.

AVM management is ideally based on an inter-disciplinary approach with joint teams of vascular neurologists, neuroradiologists, neurosurgeons and radiotherapists. A new diagnosis of an AVM is not necessarily an a priori indication for interventional treatment. The decision in favor or against a particular mode of therapy rests mainly on clinical criteria: presence or absence of a history of spontaneous rupture, the neurological examination, the patient’s age and any associated co-morbidity, etc. A critical feature is the angio-architecture of the malformation itself.

The international multicentric study ARUBA (A Randomized Trial of Unruptured Brain AVMs) is the first randomized clinical trial comparing invasive treatment with non- invasive management.