Dolichoectasia: a brain arterial disease with an elusive treatment

Dolichoectasia is a form of brain large artery disease associated with a high risk of mortality and morbidity. Progressive enlargement of arterial size is a predictor of mortality, but there are no specific treatments for arresting or slowing down dilatation. Additionally, dilated brain arteries can cause flow stagnation, which can trigger thrombosis and cause stroke. Pathology and genetic studies indicate a possible role for increased matrix metalloproteinase activation in arterial dilatation and thus in the pathophysiology of dolichoectasia. Therefore, therapeutic interventions aimed at slowing down arterial dilatation and preventing thrombosis could hypothetically play a role in treating patients with dolichoectasia.

We present four patients with dolichoectasia that exemplify therapeutic challenges worth discussing in the context of the current literature. Two patients were treated off-label with doxycycline (based on its antiMMP properties) and with apixaban, one patient was put on warfarin and later switched to aspirin, and the fourth patient underwent endovascular treatment.

We report four cases, all men 50 years or older. Of the two patients treated with doxycycline, we noted a slowdown of the basilar artery (BA) growth, but the BA continued to grow in the other patient. Of the two patients who received apixaban, none had a subsequent stroke in 5 and 4 years of follow-up, respectively. One patient was admitted with a fatal BA thrombosis and rupture, and pathological examination of the brain arteries demonstrated advanced arterial wall degeneration but no atherosclerosis.

These cases exemplify the challenges of treating people with dolichoectasia and highlight the need for better evidence regarding the best possible treatment for this population.