Semin Neurol 2015; 35(03): 223-234
DOI: 10.1055/s-0035-1552621
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Intracranial Vascular Malformations and Epilepsy

Colin B. Josephson
1  Department of Clinical Neurosciences, University of Calgary, Calgary, AB, Canada
Felix Rosenow
2  Epilepsy Center Frankfurt Rhine-Main, Department of Neurology, Center of Neurology and Neurosurgery, Goethe-University Frankfurt, Frankfurt a.M.; Germany
3  Epilepsy Center Hessen, Department of Neurology, Philipps-University Marburg, Marburg, Germany
Rustam Al-Shahi Salman
4  Division of Clinical Neurosciences, Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, United Kingdom
› Author Affiliations
Further Information

Publication History

Publication Date:
10 June 2015 (online)


Among the spectrum of intracranial vascular malformations (IVMs), arteriovenous malformations (AVMs), and cavernous malformations (CCMs) are of particular importance for epilepsy. Seizures are a common mode of presentation for both conditions. Seizures may occur de novo or secondary to intracerebral hemorrhage. Timely imaging is thus crucial for patients with seizures and AVMs or CCMs. Patients with a first-ever AVM- or CCM-related seizure can now be considered to have epilepsy according to the International League Against Epilepsy criteria. Observational studies and case series suggest that between 45 to 78% of patients with AVM-related epilepsy and 47 to 60% of patients with CCM-related epilepsy may achieve seizure freedom through antiepileptic drugs (AEDs) alone. Invasive procedures are available although current evidence suggests that epilepsy-specific preintervention evaluations are underused. Randomized controlled trials and population-based studies have demonstrated worse short-term functional outcomes after routine intervention on unruptured AVMs or CCMs when compared with conservative management. The role of invasive therapy for IVM-related epilepsy has yielded mixed results. Case series have reported high estimates of seizure freedom although these results have not been replicated in controlled observational studies. Randomized controlled trials of immediate invasive therapy versus conservative management, in addition to usual care with AEDs and of different types of treatment and their timing, are warranted for AVMs and CCM-related epilepsy.