Visual Snow: a Potential Cortical Hyperexcitability Syndrome

  • Alaa Bou GhannamEmail author
  • Victoria S. Pelak
Neurologic Ophthalmology and Otology (RK Shin and DR Gold, Section Editors)
Part of the following topical collections:
  1. Topical Collection on Neurologic Ophthalmology and Otology

Opinion statement

The purpose of this review is to provide an overview of visual snow (VS) and provide information regarding current treatment options for VS. Visual snow (VS) is a rare disorder manifesting with a persistent visual phenomenon of seeing numerous tiny snow-like dots throughout the visual field, and it can cause debilitating visual and psychological consequences. It is emerging as a disorder separate from, but associated with, migraine visual aura, and neuronal cortical hyperexcitability is being considered as a theoretical mechanism for the persistent-positive visual symptoms. There are few studies that have investigated the treatment of VS, but as our understanding of this entity begins to change, we expect that new treatment approaches and treatment trials will emerge in the next decade. Currently, our approach is to consider pharmacologic treatment for all patients with VS who report decreased quality of life as a result of VS. Resolution of the disorder is difficult to accomplish with treatment, but in our experience, even when symptom intensity is simply reduced, many patients find that there is an improvement in their quality of life that is beneficial. Our preferred treatment options include: (1) oral lamotrigine with a slow increase from 25 mg daily to a maintenance dose of 200–300 mg daily in divided doses as tolerated, and this is typically achieved by advancing the dose in increments of 25–50 mg weekly following the first 2 weeks of therapy; (2) oral acetazolamide with an initial dose of 250 mg daily followed by a slow increase over 1–2 weeks to a total of 1000 mg daily in divided doses, and higher doses can be tolerated by some without increasing the risk-benefit ratio; or (3) oral verapamil long-acting at 120–240 mg daily, and if side effects limit the dose the can be initiated, then lower doses with short-acting verapamil two or three times daily can be substituted until higher doses with the long-acting formula can be tolerated. By initiating drug treatments with low doses and slowly increasing over 1 to 4 weeks, tolerability and compliance improves and allows patients to realize the full benefits of treatment. The proposed mechanisms of microstructural cortical abnormalities and hyperexcitability as a cause of VS may lead to new treatment approaches in the future. Until such a time, medications reported to relieve persistent visual phenomena of migraine and visual aura of migraine are treatment options worth considering and these are reviewed for that purpose. Although clinical trials for the treatment of visual snow are lacking due to the rarity of the disorder, medications reviewed here should be considered for use in patients with VS who experience an impact on their quality of life. Theoretical mechanisms that lead to cortical hyperexcitability are being investigated and could lead to new treatment options. In the meantime, medications may provide benefits in this disabling condition.


Visual snow Cortical hyperexcitability Migraine visual aura Persistent visual phenomena of migraine 


Compliance with Ethical Standards

Conflict of Interest

The authors declare that they have no conflict of interest.

Human and Animal Rights and Informed Consent

This article does not contain any studies with human or animal subjects performed by any of the authors.

References and Recommended Reading

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  1. 1.
    Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45:664–8.CrossRefPubMedGoogle Scholar
  2. 2.
    Haas DC. Prolonged migraine aura status. Ann Neurol. 1982;11:197–9.CrossRefPubMedGoogle Scholar
  3. 3.
    Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261–2.CrossRefPubMedGoogle Scholar
  4. 4.
    Chen WT, Fuh JL, Lu SR, Wang SJ. Persistent migrainous visual phenomena might be responsive to lamotrigine. Headache. 2001;41:823–5.CrossRefPubMedGoogle Scholar
  5. 5.
    Jager HR, Giffin NJ, Goadsby PJ. Diffusion- and perfusion-weighted MR imaging in persistent migrainous visual disturbances. Cephalalgia. 2005;25:323–32.CrossRefPubMedGoogle Scholar
  6. 6.
    Relja G, Granato A, Ukmar M, Ferretti G, Antonello RM, Zorzon M. Persistent aura without infarction: description of the first case studied with both brain SPECT and perfusion MRI. Cephalalgia. 2005;25:56–9.CrossRefPubMedGoogle Scholar
  7. 7.
    San-Juan OD, Zermeno PF. Migraine with persistent aura in a Mexican patient: case report and review of the literature. Cephalalgia. 2007;27:456–60.CrossRefPubMedGoogle Scholar
  8. 8.
    Wang YF, Fuh JL, Chen WT, Wang SJ. The visual aura rating scale as an outcome predictor for persistent visual aura without infarction. Cephalalgia. 2008;28:1298–304.CrossRefPubMedGoogle Scholar
  9. 9.
    Belvis R, Ramos R, Villa C, Segura C, Pagonabarraga J, Ormazabal I, et al. Brain apparent water diffusion coefficient magnetic resonance image during a prolonged visual aura. Headache. 2010;50:1045–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Rastogi RG, VanderPluym J, Lewis KS. Migrainous aura, visual snow, and “Alice in Wonderland” syndrome in childhood. Semin Pediatr Neurol. 2016;23:14–7.CrossRefPubMedGoogle Scholar
  11. 11.
    •• Schankin CJ, Viana M, Goadsby PJ. Persistent and repetitive visual disturbances in migraine: a review. Headache. 2017;57(1):1–16. This article is a review of all visual disturbances that are associated with migraine including visual snow.Google Scholar
  12. 12.
    • Simpson JC, Goadsby PJ, Prabhakar P. Positive persistent visual symptoms (visual snow) presenting as a migraine variant in a 12-year-old girl. Pediatr Neurol. 2013;49:361–3. This is a case report of a girl with visual snow. It focuses on different pharmaceutical treatments that could be used in visual snow.CrossRefPubMedGoogle Scholar
  13. 13.
    •• Lauschke JL, Plant GT, Fraser CL. Visual snow: a thalamocortical dysrhythmia of the visual pathway? J Clin Neurosci. 2016;28:123–7. One of the larger cohorts that studied different characteristics in patients with visual snow. It also introduced a new different type of treatment visual snow through color filtered lenses.CrossRefPubMedGoogle Scholar
  14. 14.
    Schankin C, Maniyar F, Hoffman J, Chou D. Visual snow: a new disease entity distinct from migraine aura. Neurology. 2012;78:S36.006.CrossRefGoogle Scholar
  15. 15.
    Schankin CJ, Maniyar F, Hoffmann J, Chou D, Goadsby PJ. Field-testing the criteria for “visual snow” (positive persistent visual disturbance). J Headache Pain. 2013;14(Suppl 1):P132.Google Scholar
  16. 16.
    Schankin CJ, Maniyar F, Hoffman J, Chou D, Goadsby PJ. Clinical characterization of “visual snow” (positive persistent visual disturbance). J Headache Pain. 2013;14:132.CrossRefGoogle Scholar
  17. 17.
    • Schankin CJ, Maniyar FH, Digre KB, Goadsby PJ. ‘Visual snow’—a disorder distinct from persistent migraine aura. Brain. 2014;137:1419–28. This is the largest review article of patients diagnosed with visual snow. It also separates visual snow as a disease entity on its own.CrossRefPubMedGoogle Scholar
  18. 18.
    • Schankin CJ, Maniyar FH, Sprenger T, Chou DE, Eller M, Goadsby PJ. The relation between migraine, typical migraine aura and “visual snow”. Headache. 2014;54:957–66. This article assesses the relationship between “visual snow”, comorbid migraine, and typical migraine aura.CrossRefPubMedGoogle Scholar
  19. 19.
    Schankin CJ, Goadsby PJ. Visual snow—persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015;19:23.CrossRefPubMedGoogle Scholar
  20. 20.
    Bessero AC, Plant GT. Should ‘visual snow’ and persistence of after-images be recognised as a new visual syndrome? J Neurol Neurosurg Psychiatry. 2014;85:1057–8.CrossRefPubMedGoogle Scholar
  21. 21.
    Gersztenkorn D, Lee AG. Palinopsia revamped: a systematic review of the literature. Surv Ophthalmol. 2015;60:1–35.CrossRefPubMedGoogle Scholar
  22. 22.
    Goadsby PJ, Lipton RB, Ferrari MD. Migraine—current understanding and treatment. N Engl J Med. 2002;346:257–70.CrossRefPubMedGoogle Scholar
  23. 23.
    Zambrowski O, Ingster-Moati I, Vignal-Clermont C, Robert MP. The visual snow phenomenon. J Fr Ophtalmol. 2014;37:722–27.CrossRefPubMedGoogle Scholar
  24. 24.
    Unal-Cevik I, Yildiz FG. Visual snow in migraine with aura: further characterization by brain imaging, electrophysiology, and treatment—case report. Headache. 2015;55:1436–41.CrossRefPubMedGoogle Scholar
  25. 25.
    Zeki S, Watson JD, Lueck CJ, Friston KJ, Kennard C, Frackowiak RS. A direct demonstration of functional specialization in human visual cortex. J Neurosci. 1991;11:641–9.PubMedGoogle Scholar
  26. 26.
    Roland PE, Gulyas B. Visual imagery and visual representation. Trends Neurosci. 1994;17:281–7. Discussion 94-7.CrossRefPubMedGoogle Scholar
  27. 27.
    Burstein R, Noseda R, Borsook D. Migraine: multiple processes, complex pathophysiology. J Neurosci. 2015;35:6619–29.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Rozen TD. Treatment of a prolonged migrainous aura with intravenous furosemide. Neurology. 2000;55:732–3.CrossRefPubMedGoogle Scholar
  29. 29.
    Kilpatrick ZP, Bard EG. Hallucinogen persisting perception disorder in neuronal networks with adaptation. J Comput Neurosci. 2012;32:25–53.CrossRefPubMedGoogle Scholar
  30. 30.
    Llinas RR, Ribary U, Jeanmonod D, Kronberg E, Mitra PP. Thalamocortical dysrhythmia: a neurological and neuropsychiatric syndrome characterized by magnetoencephalography. Proc Natl Acad Sci U S A. 1999;96:15222–7.CrossRefPubMedPubMedCentralGoogle Scholar
  31. 31.
    Nandakumar K, Leat SJ. Dyslexia: a review of two theories. Clin Exp Optom. 2008;91:333–40.CrossRefPubMedGoogle Scholar
  32. 32.
    Gronseth GS, Woodroffe LM, Getchius TSD. Clinical practice guideline process manual. In: American Academy of Neurology, editor. St. Paul, MN, 2011. p. 1-57.Google Scholar
  33. 33.
    Vincent M, Hadjikhani N. The cerebellum and migraine. Headache. 2007;47:820–33.CrossRefPubMedPubMedCentralGoogle Scholar
  34. 34.
    Haan J, Sluis P, Sluis LH, Ferrari MD. Acetazolamide treatment for migraine aura status. Neurology. 2000;55:1588–9.CrossRefPubMedGoogle Scholar
  35. 35.
    De Simone R, Marano E, Di Stasio E, Bonuso S, Fiorillo C, Bonavita V. Acetazolamide efficacy and tolerability in migraine with aura: a pilot study. Headache. 2005;45:385–6.CrossRefPubMedGoogle Scholar
  36. 36.
    Woodbury DM. Carbonic anhydrase inhibitors. Adv Neurol. 1980;27:617–33.PubMedGoogle Scholar
  37. 37.
    de Almeida RF, Leao IA, Gomes JB, da Silva Jr AA, Teixeira AL. Migraine with persistent visual aura: response to furosemide. Clinics (Sao Paulo). 2009;64:375–6.CrossRefGoogle Scholar
  38. 38.
    Malha L, Mann SJ. Loop diuretics in the treatment of hypertension. Curr Hypertens Rep. 2016;18:27.CrossRefPubMedGoogle Scholar
  39. 39.
    Podoll K, Dahlem M, Haas DC. Persistent migraine aura without infarction—a detailed description. Migraine Aura Foundation. 2005. Accessed 1 Oct 2016.
  40. 40.
    Lampl C, Bonelli S, Ransmayr G. Efficacy of topiramate in migraine aura prophylaxis: preliminary results of 12 patients. Headache. 2004;44:174–6.CrossRefPubMedGoogle Scholar
  41. 41.
    D’Andrea G, Nordera GP, Allais G. Treatment of aura: solving the puzzle. Neurological Sci. 2006;27 Suppl 2:S96–9.CrossRefGoogle Scholar
  42. 42.
    Thissen S, Vos IG, Schreuder TH, Schreurs WM, Postma LA, Koehler PJ. Persistent migraine aura: new cases, a literature review, and ideas about pathophysiology. Headache. 2014;54:1290–309.CrossRefPubMedGoogle Scholar
  43. 43.
    Beltramone M, Donnet A. Status migrainous and migraine aura status in a French tertiary-care center: an 11-year retrospective analysis. Cephalalgia. 2014;34:633–7.CrossRefPubMedGoogle Scholar
  44. 44.
    D’Andrea G, Colavito D, Dalle Carbonare M, Leon A. Migraine with aura: conventional and non-conventional treatments. Neurological Sci. 2011;32 Suppl 1:S121–9.CrossRefGoogle Scholar
  45. 45.
    D’Andrea G, Allais G, Grazzi L, Fumagalli L. Migraine with aura from pathophysiology to treatment: therapeutic strategies. Neurological Sci. 2005;26 Suppl 2:s104–7.CrossRefGoogle Scholar
  46. 46.
    Lampl C, Buzath A, Klinger D, Neumann K. Lamotrigine in the prophylactic treatment of migraine aura—a pilot study. Cephalalgia. 1999;19:58–63.CrossRefPubMedGoogle Scholar
  47. 47.
    Pascual J, Caminero AB, Mateos V, Roig C, Leira R, Garcia-Monco C, et al. Preventing disturbing migraine aura with lamotrigine: an open study. Headache. 2004;44:1024–8.CrossRefPubMedGoogle Scholar
  48. 48.
    Lampl C, Katsarava Z, Diener HC, Limmroth V. Lamotrigine reduces migraine aura and migraine attacks in patients with migraine with aura. J Neurol Neurosurg Psychiatry. 2005;76:1730–2.CrossRefPubMedPubMedCentralGoogle Scholar
  49. 49.
    Steiner TJ, Findley LJ, Yuen AW. Lamotrigine versus placebo in the prophylaxis of migraine with and without aura. Cephalalgia. 1997;17:109–12.CrossRefPubMedGoogle Scholar
  50. 50.
    Dooley J, Camfield P, Gordon K, Camfield C, Wirrell Z, Smith E. Lamotrigine-induced rash in children. Neurology. 1996;46:240–2.CrossRefPubMedGoogle Scholar
  51. 51.
    Racagni G, Apud JA, Cocchi D, Locatelli V, Muller EE. GABAergic control of anterior pituitary hormone secretion. Life Sci. 1982;31:823–38.CrossRefPubMedGoogle Scholar
  52. 52.
    Welch KM, Barkley GL, Tepley N, Ramadan NM. Central neurogenic mechanisms of migraine. Neurology. 1993;43:S21–5.CrossRefPubMedGoogle Scholar
  53. 53.
    Jensen K, Tfelt-Hansen P, Lauritzen M, Olesen J. Clinical trial of nimodipine for single attacks of classic migraine. Cephalalgia. 1985;5:125–31.CrossRefPubMedGoogle Scholar
  54. 54.
    Hoffert MJ, Scholz MJ, Kanter R. A double-blind controlled study of nifedipine as an abortive treatment in acute attacks of migraine with aura. Cephalalgia. 1992;12:323–4.CrossRefPubMedGoogle Scholar
  55. 55.
    Goldner JA, Levitt LP. Treatment of complicated migraine with sublingual nifedipine. Headache. 1987;27:484–6.CrossRefPubMedGoogle Scholar
  56. 56.
    Hedman C, Andersen AR, Andersson PG, Gilhus NE, Kangasniemi P, Olsson JE, et al. Symptoms of classic migraine attacks: modifications brought about by metoprolol. Cephalalgia. 1988;8:279–84.CrossRefPubMedGoogle Scholar
  57. 57.
    Kaube H, Herzog J, Kaufer T, Dichgans M, Diener HC. Aura in some patients with familial hemiplegic migraine can be stopped by intranasal ketamine. Neurology. 2000;55:139–41.CrossRefPubMedGoogle Scholar
  58. 58.
    Afridi SK, Giffin NJ, Kaube H, Goadsby PJ. A randomized controlled trial of intranasal ketamine in migraine with prolonged aura. Neurology. 2013;80:642–7.CrossRefPubMedGoogle Scholar
  59. 59.
    Allais G, D’Andrea G, Airola G, De Lorenzo C, Mana O, Benedetto C. Picotamide in migraine aura prevention: a pilot study. Neurological Sci. 2004;25 Suppl 3:S267–9.CrossRefGoogle Scholar
  60. 60.
    Anderson IM, Ferrier IN, Baldwin RC, Cowen PJ, Howard L, Lewis G, et al. Evidence-based guidelines for treating depressive disorders with antidepressants: a revision of the 2000 British Association for Psychopharmacology guidelines. J Psychopharmacol. 2008;22:343–96.CrossRefPubMedGoogle Scholar
  61. 61.
    Banerjee M, Findley LJ. Sumatriptan in the treatment of acute migraine with aura. Cephalalgia. 1992;12:39–44.CrossRefPubMedGoogle Scholar
  62. 62.
    Rozen TD. Aborting a prolonged migrainous aura with intravenous prochlorperazine and magnesium sulfate. Headache. 2003;43:901–3.CrossRefPubMedGoogle Scholar
  63. 63.
    Peroutka SJ, Wilhoit T, Jones K. Clinical susceptibility to migraine with aura is modified by dopamine D2 receptor (DRD2) NcoI alleles. Neurology. 1997;49:201–6.CrossRefPubMedGoogle Scholar
  64. 64.
    Lau Moon Lin M, Robinson PD, Flank J, Sung L, Dupuis LL. The safety of prochlorperazine in children: a systematic review and meta-analysis. Drug Saf. 2016;39:509–16.CrossRefPubMedGoogle Scholar
  65. 65.
    Cuadrado ML, Aledo-Serrano A, Lopez-Ruiz P, Gutierrez-Viedma A, Fernandez C, Orviz A et al. Greater occipital nerve block for the acute treatment of prolonged or persistent migraine aura. Cephalalgia. 2016. June 10. (Epub ahead of publishing)Google Scholar
  66. 66.
    Rozen T. Cessation of hemiplegic migraine auras with greater occipital nerve blockade. Headache. 2007;47:917–9.CrossRefPubMedGoogle Scholar
  67. 67.
    Kleen JK, Levin M. Injection therapy for headache and facial pain. Oral Maxillofac Surg Clin North Am. 2016;28:423–34.CrossRefPubMedGoogle Scholar
  68. 68.
    Barrington MJ, Watts SA, Gledhill SR, Thomas RD, Said SA, Snyder GL, et al. Preliminary results of the Australasian Regional Anaesthesia Collaboration: a prospective audit of more than 7000 peripheral nerve and plexus blocks for neurologic and other complications. Reg Anesth Pain Med. 2009;34:534–41.CrossRefPubMedGoogle Scholar
  69. 69.
    Bates D, Ashford E, Dawson R, Ensink FB, Gilhus NE, Olesen J, et al. Subcutaneous sumatriptan during the migraine aura. Sumatriptan Aura Study Group. Neurology. 1994;44:1587–92.CrossRefPubMedGoogle Scholar

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© Springer Science+Business Media New York 2017

Authors and Affiliations

  1. 1.Departments of Ophthalmology and NeurologyUniversity of Colorado School of MedicineAuroraUSA

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