Migraine is a neurological disorder that frequently involves cortical paroxysmal dysfunction expressed as aura 1 . The most frequent type of aura is visual, sometimes experienced as fortification spectra, known as teichopsia. This typical zigzag, horseshoe-shaped expanding bright perception, usually perceived as complex interlacing lines named "chevaux de frises" by Gowers, followed by a bean-shaped loss of visual acuity, strongly suggests the involvement of visual processing areas. Clinical, experimental, and neuroimaging evidence demonstrates that cortical spreading depression (CSD) is the phenomenon underlying the pathophysiology of bizarre visual perceptions among migraineurs. Computerized models simulating a CSD wave passing through cortical area V1 convincingly reproduce the teichopsia pattern 2 .The cortical function is deeply dependent on neuronal input. Depriving the cortex of its natural input may influence the cerebral function and change the cortical maps both locally and distantly 3 . In blind individuals, the occipital cortex is critical for tactile Braille reading 4 . The visual cortex is pivotal in the pathophysiology of the migraine aura, the objective of the present study was to investigate whether the phenotype of migraine-related visual phenomena would change in amaurotic patients.
AbstrActMigraine is a central nervous system disorder frequently expressed with paroxysmal visual dysfunctions. Objective: To test the hypothesis that a normal visual input is vital for migrainous aura and photophobia. Methods: We studied the migraine-related visual disturbances in 8 sightless migraineurs identified among 200 visually impaired subjects. Results: The main findings were visual aura and photophobia disappearance along with the development of blindness, abnormal aura [too short, colorful (e.g., blue or fire-like), auditory in nature, or different in shape (round forms)], and the lack of photophobia. Conclusions: We propose that the aura duration should be accepted as shorter in visually impaired subjects. The changes in the aura phenotype observed in our patients may be a result of cerebral plasticity induced by visual impairment and/or the lack of a visual input per se. Integrity of visual pathways plays a key role in migraine visual aura and photophobia.Keywords: migraine with aura, subnormal vision, blindness, visual aura, photophobia resumo A enxaqueca é doença neurológica frequentemente associada a anormalidades visuais transitórias. Objetivo: Testar a hipótese de que a visão normal é importante para o fenótipo da aura e da fotofobia. Métodos: Estudamos 8 enxaquecosos deficientes visuais identificados em uma população de 200 indivíduos com visão subnormal. Resultados: Os principais achados foram: o desaparecimento da aura visual e da fotofobia com o início da cegueira; a ocorrência de aura atípica -muito curta, colorida (p. ex. azul, ou cor de fogo) auditiva ou diferente na forma (arredondadas); e a ausência de fotofobia. Conclusões: Propomos que a duração da aura possa ser admitida como mais cur...