Raffaele Ornello, MD

Koemans EA, Voigt S, Rasing I, van Etten ES, van Zweet EW, van Walderveen MAA, et al. Migraine With Aura as Early Disease Marker in Hereditary Dutch-Type Cerebral Amyloid Angiopathy. Stroke. 2020;51:1094–1099.

Migraine, especially with aura, is associated with monogenic cerebrovascular syndromes such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCLS). Dutch-type cerebral amyloid angiopathy (D-CAA) is a monogenic form of cerebral amyloid angiopathy (CAA). Migraine is common in patients with CAA; however, its characteristics have not been specifically assessed in those patients.

In their retrospective study, the authors assessed the prevalence and characteristics of migraine in 86 in- and outpatients with D-CAA visited in a specialized center over six years (2012-2018); the mean age of patients was 57 years, and 57% were women. The authors found a higher prevalence of migraine (55%) and especially of migraine with aura (all patients with migraine had aura attacks) in their sample compared with what expected in the general population; besides, patients with D-CAA tended to have a late onset of migraine, which is uncommon in migraine. Notably, in two-thirds of patients, migraine was the first symptom of D-CAA, and in more than half of cases, a migraine aura lasting more than 60 minutes signaled an intracerebral hemorrhage.

The data of the present study are limited by the fact that the transient focal neurological episodes typical of CAA can mimic migraine aura and lead to overestimation of migraine prevalence in patients with CAA. Nevertheless, the study expands the possible association between migraine with aura and cerebrovascular disease. Migraine leads to an increased risk of ischemic and hemorrhagic stroke for still unknown reasons. Discovering the possible mechanisms of association between migraine and monogenic cerebrovascular disease might provide interesting insights into the pathophysiology of migraine, help design new treatments, and possibly reduce the vascular risk of migraineurs.