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Liem, M.K.; Oberstein, S.A.J.L.; Grond, J. van der; Ferrari, M.D.; Haan, J. (2010)

CADASIL and migraine: A narrative review

Article / Letter to editor
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene and is clinically characterized by recurrent stroke, cognitive decline, psychiatric disturbances and migraine. The prevalence of migraine in CADASIL is slightly higher than in the general population, and the proportion of migraine with aura is much higher. The pathophysiological mechanism that leads to increased aura prevalence in CADASIL is unknown. Possible mechanisms of the excess of migraine with aura are an increased susceptibility to cortical spreading depression (CSD) or a different expression of CSD. It is also possible that the brainstem migraine area is involved in CADASIL. Last, it is possible that the NOTCH3 mutation acts as a migraine aura susceptibility gene by itself. In this narrative review we summarize the literature about migraine in CADASIL, with a special focus on what CADASIL might teach us about the pathophysiology of...Show moreCerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is caused by mutations in the NOTCH3 gene and is clinically characterized by recurrent stroke, cognitive decline, psychiatric disturbances and migraine. The prevalence of migraine in CADASIL is slightly higher than in the general population, and the proportion of migraine with aura is much higher. The pathophysiological mechanism that leads to increased aura prevalence in CADASIL is unknown. Possible mechanisms of the excess of migraine with aura are an increased susceptibility to cortical spreading depression (CSD) or a different expression of CSD. It is also possible that the brainstem migraine area is involved in CADASIL. Last, it is possible that the NOTCH3 mutation acts as a migraine aura susceptibility gene by itself. In this narrative review we summarize the literature about migraine in CADASIL, with a special focus on what CADASIL might teach us about the pathophysiology of migraine.Show less
Migraine CADASIL aura spreading depression NOTCH3 autosomal-dominant arteriopathy cortical spreading depression brain-stem activation subcortical infarcts blood-flow white-matter vascular malformation cerebral hemodynamics notch3 mutation functional mri
All authors
Liem, M.K.; Oberstein, S.A.J.L.; Grond, J. van der; Ferrari, M.D.; Haan, J.
Date
2010-11-30
Journal
Cephalalgia
Volume
30
Issue
11
Pages
1284 - 1289
DOI
doi:10.1177/0333102410370870