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February 11, 2020; 94 (6) ArticleOpen Access

Visual snow syndrome

A clinical and phenotypical description of 1,100 cases

View ORCID ProfileFrancesca Puledda, View ORCID ProfileChristoph Schankin, View ORCID ProfilePeter J. Goadsby
First published January 15, 2020, DOI: https://doi.org/10.1212/WNL.0000000000008909
Francesca Puledda
From the Headache Group (F.P., P.J.G.), Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology & Neuroscience, King's College London; NIHR-Wellcome Trust King's Clinical Research Facility (F.P., P.J.G.), SLaM Biomedical Research Centre, King's College Hospital, London, UK; and Department of Neurology (C.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.
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  • ORCID record for Francesca Puledda
Christoph Schankin
From the Headache Group (F.P., P.J.G.), Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology & Neuroscience, King's College London; NIHR-Wellcome Trust King's Clinical Research Facility (F.P., P.J.G.), SLaM Biomedical Research Centre, King's College Hospital, London, UK; and Department of Neurology (C.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.
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Peter J. Goadsby
From the Headache Group (F.P., P.J.G.), Department of Basic and Clinical Neuroscience, Institute of Psychiatry, Psychology & Neuroscience, King's College London; NIHR-Wellcome Trust King's Clinical Research Facility (F.P., P.J.G.), SLaM Biomedical Research Centre, King's College Hospital, London, UK; and Department of Neurology (C.S.), Inselspital, Bern University Hospital, University of Bern, Switzerland.
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Visual snow syndrome
A clinical and phenotypical description of 1,100 cases
Francesca Puledda, Christoph Schankin, Peter J. Goadsby
Neurology Feb 2020, 94 (6) e564-e574; DOI: 10.1212/WNL.0000000000008909

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Abstract

Objective To validate the current criteria of visual snow and to describe its common phenotype using a substantial clinical database.

Methods We performed a web-based survey of patients with self-assessed visual snow (n = 1,104), with either the complete visual snow syndrome (n = 1,061) or visual snow without the syndrome (n = 43). We also describe a population of patients (n = 70) with possible hallucinogen persisting perception disorder who presented clinically with visual snow syndrome.

Results The visual snow population had an average age of 29 years and had no sex prevalence. The disorder usually started in early life, and ≈40% of patients had symptoms for as long as they could remember. The most commonly experienced static was black and white. Floaters, afterimages, and photophobia were the most reported additional visual symptoms. A latent class analysis showed that visual snow does not present with specific clinical endophenotypes. Severity can be classified by the amount of visual symptoms experienced. Migraine and tinnitus had a very high prevalence and were independently associated with a more severe presentation of the syndrome.

Conclusions Clinical characteristics of visual snow did not differ from the previous cohort in the literature, supporting validity of the current criteria. Visual snow likely represents a clinical continuum, with different degrees of severity. On the severe end of the spectrum, it is more likely to present with its common comorbid conditions, migraine and tinnitus. Visual snow does not depend on the effect of psychotropic substances on the brain.

Glossary

DSM-V=
Diagnostic and Statistical Manual of Mental Disorders, 5th edition;
HPPD=
hallucinogen persisting perception disorder;
VS=
visual snow;
VSS=
visual snow syndrome

Footnotes

  • Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.

  • The Article Processing Charge was funded by crowdfunding from the self-help group for visual snow Eye On Vision Foundation and funding from the Visual Snow Initiative; and from the SLaM Biomedical Research Centre.

  • Editorial, page 241

  • Podcast: NPub.org/fxcblh

  • CME Course: NPub.org/cmelist

  • Received February 11, 2019.
  • Accepted in final form August 28, 2019.
  • Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution License 4.0 (CC BY), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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