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.