Glover, Patrick

Objective: To characterize migraine in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Background: CADASIL serves as a key model of a monoallelic stroke disease. A portion of patients have coexistent migraine with aura, yet the relationship between migraine and the pathophysiology of CADASIL is poorly understood. Design/Methods: We performed a prospective study of adults with a confirmed genetic diagnosis of CADASIL, age-matched controls with migraine with aura (MA), and headache-free controls. The Migraine Disability Assessment Questionnaire (MIDAS) and Headache Impact Test (HIT-6) were used to quantify migraine-related disability. Subjects were followed for one year after enrollment. Results: We enrolled 88 subjects, which included 33 subjects with CADASIL, 32 subjects without CADASIL but with MA, and 23 control subjects without CADASIL or MA. Mean age was 53.6 (range, 22-86), 64.8% (57/88) were females, and 83% (73/88) were white. Of the CADASIL subjects, 61% (20/33) suffered from MA (CAD/+MA). Aura semiology amongst the CAD/+MA and MA patients were as follows: visual (80% vs 81%), sensory (75% vs 81%), speech (75% vs 50%), motor (30% vs 50%), brain stem (60% vs 81%) and retinal (30% vs 25%). Mean MIDAS scores at enrollment were comparable between the CAD/+MA and MA populations (32.3 vs 33.5), as were mean HIT-6 scores (56.3 vs 56.9). Discussion: These preliminary results do not show a significant difference in migraine disability between CADASIL migraineurs and chronic migraineurs. Minor differences were observed in aura distribution between the two groups, although further investigations are needed to better understand the underlying pathology of CADASIL and migraine.

Objectives: Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is inherited microangiopathy characterized by recurrent subcortical infarcts. A majority of those with CADASIL report coexistent migraine with aura. The authors aim to quantitatively describe migraine-related disability within a CADASIL cohort. Methods: A cross-sectional analysis was performed in a cohort of CADASIL. The Migraine Disability Assessment (MIDAS) quantified migraine-related disability. Further metrics obtained include first and last migraine, aura semiology, and therapeutic measures. Results: Twenty-four individuals were included [63% (15/24) female individuals; mean age, 56 y; range, 34 to 81 y]. Fifty-four percent (13/24) reported migraine, whereas 46% (11/24) reporting varying degrees of migraine-related disability. MIDAS Questionnaire scores appeared bimodal: 58% (14/24) scored 0 to 5, 7% (1/24) scored 6 to 10, 7% (1/24) scored 11 to 20 and 33% (8/24) scored over 20. Severe disability was associated with the multiplicity of aura semiologies and poor response to pharmacologic prophylaxis. Conclusions: A bimodal distribution of migraine-related disability was observed. A third of individuals had a severe disability and appeared medically refractory to medical migraine prophylactic measures. This study may serve as a reference point for future trials quantitatively gauging response of novel migraine treatment strategies within this unique population.