Visual Snow: a Potential Cortical Hyperexcitability Syndrome

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.

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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.

    CAS  Article  PubMed  Google Scholar 

  2. 2.

    Haas DC. Prolonged migraine aura status. Ann Neurol. 1982;11:197–9.

    CAS  Article  PubMed  Google Scholar 

  3. 3.

    Rothrock JF. Successful treatment of persistent migraine aura with divalproex sodium. Neurology. 1997;48:261–2.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    Article  PubMed  Google 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.

  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.

    Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    Article  Google 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.

  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.

    Article  Google 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.

    Article  PubMed  Google 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.

    Article  PubMed  Google Scholar 

  19. 19.

    Schankin CJ, Goadsby PJ. Visual snow—persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015;19:23.

    Article  PubMed  Google 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.

    Article  PubMed  Google Scholar 

  21. 21.

    Gersztenkorn D, Lee AG. Palinopsia revamped: a systematic review of the literature. Surv Ophthalmol. 2015;60:1–35.

    Article  PubMed  Google Scholar 

  22. 22.

    Goadsby PJ, Lipton RB, Ferrari MD. Migraine—current understanding and treatment. N Engl J Med. 2002;346:257–70.

    CAS  Article  PubMed  Google Scholar 

  23. 23.

    Zambrowski O, Ingster-Moati I, Vignal-Clermont C, Robert MP. The visual snow phenomenon. J Fr Ophtalmol. 2014;37:722–27.

    CAS  Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    CAS  PubMed  Google Scholar 

  26. 26.

    Roland PE, Gulyas B. Visual imagery and visual representation. Trends Neurosci. 1994;17:281–7. Discussion 94-7.

    CAS  Article  PubMed  Google Scholar 

  27. 27.

    Burstein R, Noseda R, Borsook D. Migraine: multiple processes, complex pathophysiology. J Neurosci. 2015;35:6619–29.

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  28. 28.

    Rozen TD. Treatment of a prolonged migrainous aura with intravenous furosemide. Neurology. 2000;55:732–3.

    CAS  Article  PubMed  Google Scholar 

  29. 29.

    Kilpatrick ZP, Bard EG. Hallucinogen persisting perception disorder in neuronal networks with adaptation. J Comput Neurosci. 2012;32:25–53.

    Article  PubMed  Google 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.

    CAS  Article  PubMed  PubMed Central  Google Scholar 

  31. 31.

    Nandakumar K, Leat SJ. Dyslexia: a review of two theories. Clin Exp Optom. 2008;91:333–40.

    Article  PubMed  Google 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.

  33. 33.

    Vincent M, Hadjikhani N. The cerebellum and migraine. Headache. 2007;47:820–33.

    Article  PubMed  PubMed Central  Google Scholar 

  34. 34.

    Haan J, Sluis P, Sluis LH, Ferrari MD. Acetazolamide treatment for migraine aura status. Neurology. 2000;55:1588–9.

    CAS  Article  PubMed  Google 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.

    Article  PubMed  Google Scholar 

  36. 36.

    Woodbury DM. Carbonic anhydrase inhibitors. Adv Neurol. 1980;27:617–33.

    CAS  PubMed  Google 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.

    Article  Google Scholar 

  38. 38.

    Malha L, Mann SJ. Loop diuretics in the treatment of hypertension. Curr Hypertens Rep. 2016;18:27.

    Article  PubMed  Google Scholar 

  39. 39.

    Podoll K, Dahlem M, Haas DC. Persistent migraine aura without infarction—a detailed description. Migraine Aura Foundation. 2005. http://www.migraine-aura.com/content/e25968/e26078/e26305/index_en.html. 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.

    Article  PubMed  Google Scholar 

  41. 41.

    D’Andrea G, Nordera GP, Allais G. Treatment of aura: solving the puzzle. Neurological Sci. 2006;27 Suppl 2:S96–9.

    Article  Google 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.

    Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    Article  Google 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.

    Article  Google 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.

    CAS  Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    CAS  Article  PubMed  PubMed Central  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google Scholar 

  52. 52.

    Welch KM, Barkley GL, Tepley N, Ramadan NM. Central neurogenic mechanisms of migraine. Neurology. 1993;43:S21–5.

    CAS  Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    CAS  Article  PubMed  Google Scholar 

  55. 55.

    Goldner JA, Levitt LP. Treatment of complicated migraine with sublingual nifedipine. Headache. 1987;27:484–6.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    Article  Google 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.

    CAS  Article  PubMed  Google Scholar 

  61. 61.

    Banerjee M, Findley LJ. Sumatriptan in the treatment of acute migraine with aura. Cephalalgia. 1992;12:39–44.

    CAS  Article  PubMed  Google Scholar 

  62. 62.

    Rozen TD. Aborting a prolonged migrainous aura with intravenous prochlorperazine and magnesium sulfate. Headache. 2003;43:901–3.

    Article  PubMed  Google 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.

    CAS  Article  PubMed  Google 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.

    Article  PubMed  Google 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)

  66. 66.

    Rozen T. Cessation of hemiplegic migraine auras with greater occipital nerve blockade. Headache. 2007;47:917–9.

    Article  PubMed  Google Scholar 

  67. 67.

    Kleen JK, Levin M. Injection therapy for headache and facial pain. Oral Maxillofac Surg Clin North Am. 2016;28:423–34.

    Article  PubMed  Google 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.

    Article  PubMed  Google 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.

    CAS  Article  PubMed  Google Scholar 

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Correspondence to Alaa Bou Ghannam MD.

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Bou Ghannam, A., Pelak, V.S. Visual Snow: a Potential Cortical Hyperexcitability Syndrome. Curr Treat Options Neurol 19, 9 (2017). https://doi.org/10.1007/s11940-017-0448-3

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Keywords

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