From Cavern-Dwellers to Cavernoma Science

Abstract

See related article, pages 3222–3230. Since the introduction of noninvasive imaging tools roughly 30 years ago, vascular malformations of the brain have attracted increasing interest. Among them, cerebral cavernous malformations (CCM) appear to be the most frequently diagnosed entity with current prevalence estimates ranging from 100 to 500 cases per 100000 in the general population.1 They may come to clinical attention after epileptic seizures, progressive neurological syndromes, or symptomatic hemorrhage, but overall, asymptomatic lesions seem to be most frequently encountered since the introduction of highly sensitive gradient echo (T2*) weighted sequences in routine MR imaging protocols.2 Despite increasing detection rates and growing insight into underlying molecular and genetic mechanisms, many practical issues remain as yet unsolved, particularly when it comes to actual patient care and counseling: First, no standardized imaging protocols exist. Brain MRI with the aforementioned T2*-weighted images are now considered the diagnostic standard, but the absence of contrast-enhanced T1 sequences may miss an associated developmental venous anomaly and vice versa, which may for example impact on the strategy of any neurosurgical intervention (Figure 1). Figure 1. Brain MRI study of a 37-year-old woman presenting with left internuclear ophtalmoplegia, ataxia, and a dissociated sensory deficit attributable to a left posterior pontine hematoma on FLAIR-weighted images (left plane). Three-month follow-up …