• Katayoun Vahedi, Hugues Chabriat, Claude Levy, Anne Joutel, Elisabeth Tournier-Lasserve, and Marie-Germaine Bousser.
• Service de Neurologie, Assistance Publique--Hôpitaux de Paris, Hôpital Lariboisière, Paris, France. katayoun.vahedi@lrb.ap-hop-paris.fr
• Arch Neurol Chicago. 2004 Aug 1;61(8):1237-40.

BackgroundMigraine with aura (MA) is one of the clinical hallmarks of CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy), a small vessel disease of the brain caused by mutations in the NOTCH3 gene, but its exact mechanisms are unknown.ObjectivesTo describe the patterns of MA in CADASIL and to compare brain magnetic resonance signal abnormalities between CADASIL patients with and without MA.DesignComparison of brain magnetic resonance signal abnormalities between cases and controls.SettingPatients with CADASIL seen at Lariboisière Hospital.PatientsForty-one CADASIL patients with MA and 31 age-matched CADASIL controls without MA.ResultsThe mean age at onset of MA was significantly younger in women compared with men and occurred a mean of 15 years prior to stroke onset. A majority of patients (56%) reported at least 1 migraine attack with atypical aura. All CADASIL patients either with or without MA had white matter signal abnormalities on T2-weighted imaging. There was no difference in the frequency and distribution of brain signal abnormalities between CADASIL patients with and without MA.ConclusionsIn CADASIL, MA is characterized by an unusually high frequency of attacks of migraine with atypical aura. The distribution and extent of magnetic resonance signal abnormalities did not differ according to migraine phenotype.

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