Introduction: Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. Development: VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. Conclusions: VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms. (c) 2021 Sociedad Espaniola de Neurologi ' a. Published by Elsevier Espania, S.L.U. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/ 4.0/).
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Kings Coll London, Inst Psychiat Psychol & Neurosci, Wolfson SPaRRC, Headache Grp, London, England
Kings Coll London, Kings Clin Res Facil, Natl Inst Hlth Res NIHR, London, England
Univ Calif Los Angeles, Dept Neurol, Los Angeles, CA USAKings Coll London, London SE5 9PJ, England
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Kings Coll London, NIHR Wellcome Trust Clin Res Facil, Headache Grp, London WC2R 2LS, England
Univ Munich Hosp Grosshadern, Dept Neurol, Munich, GermanyUniv Calif San Francisco, Dept Neurol, San Francisco, CA USA
Schankin, Christoph J.
Maniyar, Farooq H.
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Barts & London NHS Trust, Royal London Hosp, Dept Neurol, London, England
Basildon & Thurrock Univ Hosp NHS Fdn Trust, Nethermayne, EnglandUniv Calif San Francisco, Dept Neurol, San Francisco, CA USA
Maniyar, Farooq H.
Sprenger, Till
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Univ Basel Hosp, Div Neuroradiol, CH-4031 Basel, SwitzerlandUniv Calif San Francisco, Dept Neurol, San Francisco, CA USA
Sprenger, Till
Chou, Denise E.
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Eller, Michael
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Goadsby, Peter J.
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Kings Coll London, NIHR Wellcome Trust Clin Res Facil, Headache Grp, London WC2R 2LS, EnglandUniv Calif San Francisco, Dept Neurol, San Francisco, CA USA
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Univ Calif San Francisco, Dept Neurol, UCSF Headache Ctr, San Francisco, CA USAUniv Calif San Francisco, Dept Neurol, UCSF Headache Ctr, San Francisco, CA USA
Schankin, C.
Maniyar, F.
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Maniyar, F.
Sprenger, T.
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Chou, D. E.
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Eller, M.
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Eller, M.
Goadsby, P. J.
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