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Katayoun Vahedi, MD; Hugues Chabriat, MD, PhD; Claude Levy, MD; Anne Joutel, MD, PhD; Elisabeth Tournier-Lasserve, MD; Marie-Germaine Bousser, MD

Arch Neurol. 2004;61:1237-1240.

Background  Migraine 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.

Objectives  To describe the patterns of MA in CADASIL and to compare brain magnetic resonance signal abnormalities between CADASIL patients with and without MA.

Design  Comparison of brain magnetic resonance signal abnormalities between cases and controls.

Setting  Patients with CADASIL seen at Lariboisière Hospital.

Patients  Forty-one CADASIL patients with MA and 31 age-matched CADASIL controls without MA.

Results  The 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.

Conclusions  In 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.

Author Affiliations: Service de Neurologie (Drs Vahedi, Chabriat, and Bousser) and Laboratoire de Cytogénétique (Drs Joutel and Tournier-Lasserve), Assistance Publique–Hôpitaux de Paris, Hôpital Lariboisière, Paris; Service de Radiologie, Institut Hospitalier Jacques Cartier (Dr Levy), Massy; and INSERM (Institut National de la Santé et de la recherche Médicale) Unit E365, Faculté de Médecine Lariboisière (Drs Joutel, Tournier-Lasserve, and Bousser), Paris, France.

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