Cerebral cavernous malformations (CCMs) are the most common vascular malformations in the brain, and occur at a rate of approximately 0.6 per 100.000 people. Recognized as familial or sporadic cases, CCMs are characterized as single or multiclusters of enlarged capillary-like channels with a single layer of endothelium and without intervening brain parenchyma. There are specific alterations in brain endothelial barrier components that ultimately lead to vascular hyperpermeability, extravasation of red cells and inflammatory response within brain parenchyma. Patients with CCMs may have seizures, focal deficits, or nonspecific headaches. The most common complication is hemorrhagic stroke.
In the recent years, significant progress was made in understanding the cellular mechanism of the cerebrovascular defect in CCMs. This paper reviews the recent findings about the mechanism of CCMs as well as the new therapeutic strategies in the treatment of sporadic and familiar cerebral cavernous malformations.
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