The outlook for adults with epileptic seizure(s) associated with cerebral cavernous malformations or arteriovenous malformations

Abstract

Cerebral cavernous malformations (CCMs) and arteriovenous malformations (AVMs) are common: their asymptomatic prevalence on brain magnetic resonance imaging (MRI) is 1 in 625 and 1 in 2,000, respectively. The risk of epileptic seizure(s) for people with AVMs and CCMs affects their domestic, social, and professional lives, and may influence their decisions about treatment. This article summarizes the seizure risks for people with AVMs and CCMs, gleaned from published original articles indexed in OVID Medline and Embase before 1 January 2012. In the absence of prior intracranial hemorrhage and nonhemorrhagic focal neurologic deficit, a population-based study in Scotland, United Kingdom, found that the 5-year risks of first seizure were 8% for AVM and 4% for CCM; presentation with intracranial hemorrhage or focal neurologic deficit raised this risk for AVM (23%) but not for CCM (6%). Features associated with the occurrence of epileptic seizures for CCM are lesion multiplicity and cortical CCM location, whereas for AVM the most consistently reported associations are younger age, temporal location, cortical involvement, and nidus diameter >3 cm. In the absence of prior intracranial hemorrhage and nonhemorrhagic focal neurologic deficit, the 5-year risk of developing epilepsy following a first seizure is 58% for AVM and 94% for CCM, though there is no difference in the chance of achieving 2-year seizure freedom in this time frame (45% AVM vs. 47% CCM). Observational case series describe encouraging differences in seizure frequency before and after AVM and CCM treatment, but the shortage of studies demonstrating dramatic effects in comparison to concurrent control groups justifies the need for more controlled studies, ideally with randomized treatment allocation when the benefits of AVM or CCM treatment are uncertain.