Diagnosis, mechanisms, and management of visual snow syndrome: A narrative review

Abstract

Visual snow syndrome (VSS) is a chronic perceptual disorder defined by persistent pan-field visual static with ≥2 additional symptoms, namely palinopsia, photophobia, nyctalopia, or enhanced entoptic phenomena, lasting ≥3 months after exclusion of ophthalmic and structural neurological disease. Prevalence estimates range between 1.4–3.3% reported currently. Emerging evidence situates VSS along a rare-network spectrum characterized by thalamocortical dysrhythmia and cortical hyperexcitability, with convergent neuroimaging findings of hypermetabolism and altered connectivity in the lingual gyrus and motion area V5/MT, and microstructural white-matter changes linking visual and salience networks. Clinically, VSS frequently co-occurs with migraine and tinnitus, alongside anxiety and depression, amplifying disability. Differential diagnosis hinges on the disorder’s continuous course and normal ophthalmic testing, distinguishing it from migraine aura, retinal dystrophies, epilepsy, and drug-induced phenomena. Therapeutic evidence remains heterogeneous and largely class IV: lamotrigine yields partial improvement in a subset; benzodiazepines, topiramate, and acetazolamide show inconsistent benefits; migraine-specific agents are generally ineffective. Non-pharmacological adjuncts such as precision chromatic filters (e.g., FL-41), mindfulness-based cognitive therapy, and neuro-optometric rehabilitation, can mitigate symptom burden or functional impact; early neuromodulation trials (rTMS) report mixed results. Standardized outcome measures integrating patient-reported and objective neurophysiological/neuroimaging markers are urgently needed, as are multi-centre-controlled trials to resolve response heterogeneity and delineate VSS–migraine–tinnitus interfaces. This integrative synthesis consolidates clinical features, mechanistic models, diagnostic touchpoints, and current management strategies to inform rare-disease care pathways and future targeted interventions.

Keywords

• Vision Disorders
• Migraine Disorders
• Tinnitus
• Neuroimaging
• Lamotrigine

References

1. Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45(4):664–668. doi:10.1212/WNL.45.4.664.
2. Schankin CJ, Maniyar FH, Digre KB, Goadsby PJ. ‘Visual snow’—a disorder distinct from persistent migraine aura. Brain. 2014;137(5):1419–1428. doi:10.1093/brain/awu050.
3. Puledda F, Schankin C, Digre K, Goadsby PJ. Visual snow syndrome: what we know so far. Curr Opin Neurol. 2018;31(1):52–58. doi:10.1097/WCO.0000000000000511.
4. van Dongen RM, Waaijer LC, Onderwater GLJ, Ferrari MD, Terwindt GM. Treatment effects and comorbid diseases in 58 patients with visual snow. Neurology. 2019;93(4):e398–e403. doi:10.1212/WNL.0000000000007784.
5. Lauschke JL, Plant GT, Fraser CL. Visual snow: a thalamocortical dysrhythmia of the visual pathway? J Clin Neurosci. 2016;28:123–127. doi:10.1016/j.jocn.2015.11.036.
6. McKendrick AM, Chan YM, Tien M, Millist L, Clough M, Mack H, et al. Behavioral measures of cortical hyperexcitability assessed in people who experience visual snow. Neurology. 2017;88(14):1243–1249. doi:10.1212/WNL.0000000000003770.
7. Kondziella D, Olsen MH, Dreier JP. Prevalence of visual snow syndrome in the UK. Eur J Neurol. 2020;27(5):764–772. doi:10.1111/ene.14150.
8. Metzler AI, Robertson CE. Visual snow syndrome: proposed criteria, clinical implications, and pathophysiology. Curr Neurol Neurosci Rep. 2018;18(8):52. doi:10.1007/s11910-018-0861-4.

9. Fraser CE, Vaphiades MS, Van Stavern GP, Lee AG. Should visual snow and visual snow syndrome be evaluated outside of standard in-clinic ophthalmologic testing? J Neuroophthalmol. 2022;42(3):384–389. doi:10.1097/WNO.0000000000001675.
10. Chojdak-Łukasiewicz J, Dziadkowiak E, Rymaszewska J. Visual snow syndrome in a patient with migraine: case report and literature review. J Clin Med. 2024;13(5):1373. doi:10.3390/jcm13051373.
11. Visual Snow Initiative. Managing visual snow syndrome (VSS) [Internet]. [cited 2025 Sep 1]. Available from: https://www.visualsnowinitiative.org/managing-visual-snow-syndrome/EyeWiki. Visual snow [Internet]. American Academy of Ophthalmology; 2025 May 3 [cited 2025 Sep 1]. Available from: https://eyewiki.org/Visual_Snow
12. Ayesha A. Diagnostic and management strategies of visual snow syndrome: current perspectives. Eye Brain. 2025;17:—. doi:10.2147/EB.S418923.
13. Visual Snow Initiative. Q&A with Sierra Domb, Visual Snow Initiative Founder—Part 1 [Internet]. 2024 Jul 6 [cited 2025 Sep 1]. Available from: https://www.visualsnowinitiative.org/qa-with-sierra-domb-2024/
14. Puledda F, Schankin C, Goadsby PJ. Visual snow syndrome. Neurology. 2020;94(6):e564–e574. doi:10.1212/WNL.0000000000008909.
15. Klein A, Schankin CJ. Visual snow syndrome as a network disorder: a perspective. Front Neurol. 2021;12:724072. doi:10.3389/fneur.2021.724072.
16. Romozzi M, Frattale I, Altamura C, Passaseo I, Lo Buono V, Fofi L, et al. Prevalence and characteristics of visual snow syndrome in a sample of young Italian adults. Eur J Neurol. 2024;31(2):e16472. doi:10.1111/ene.16472.
17. Wood CL. Shedding new light on visual snow syndrome. Nat Rev Neurol. 2020;16(11):657–658. doi:10.1038/s41582- 020-00405-0.
18. Jones KJ, Youssef D, Chiang JP, Mack H, Robertson CE. Prevalence of visual snow in the pediatric population. Neurology. 2025;104(7 Suppl 1):—. (Meeting abstract; DOI not assigned.)
19. Unal-Cevik I, Yildiz FG. The relation between migraine, typical migraine aura and visual snow. Headache. 2014;54(7):1094–1104. doi:10.1111/head.12378.
20. Denuelle M, Boulloche N, Payoux P, Fabre N, Trotter Y, Géraud G. A PET study of photophobia during spontaneous migraine attacks. Neurology. 2011;76(3):213–218. doi:10.1212/WNL.0b013e3182074a57.
21. Puledda F, O’Daly O, Schankin C, Ffytche D, Williams SC, Goadsby PJ. Disrupted connectivity within visual, attentional and salience networks in the visual snow syndrome. Hum Brain Mapp. 2021;42(7):2032–2044. doi:10.1002/hbm.25343.
22. Van Laere K, Ceccarini J, Gebruers J, Goffin K, Boon P, Vandenberghe R, et al. Simultaneous 18F-FDG PET/MR metabolic and structural changes in visual snow syndrome and diagnostic use. EJNMMI Res. 2022;12(1):77. doi:10.1186/s13550-022-00949-0.
23. Visual Snow Syndrome—Ento Key [Internet]. [cited 2025 Sep 1]. Available from: https://entokey.com/visual-snow- syndrome/Fraser CL. How do I recognise and manage visual snow syndrome? Eye (Lond). 2024;38(10):—. doi:10.1038/s41433- 024-03059-4.
24. Barral M, Martins IP, Silva C, García-Azorín D, Viana M, Puledda F. Differential diagnosis of visual phenomena associated with migraine: a narrative review. Diagnostics (Basel). 2023;13(2):252. doi:10.3390/diagnostics13020252.
25. Yildiz FG, Turkyilmaz U, Unal-Cevik I. The clinical characteristics and neurophysiological assessments of the occipital cortex in visual snow syndrome with or without migraine. Headache. 2019;59(9):1457–1471. doi:10.1111/head.13494.
26. Hepschke JL, Seymour RA, He X, Etchell AC, Sowman PF, Fraser CL, et al. Cortical oscillatory dysrhythmias in visual snow syndrome: a magnetoencephalography study. Brain Commun. 2021;3(4):fcab296. doi:10.1093/braincomms/fcab296.
27. Ciuffreda KJ, Tannen B, Rutner D, Han MHE. Neuro-optometric treatment for visual snow syndrome: recent advances. Concussion. 2023;8(3):CNC110. doi:10.2217/cnc-2023-0006.
28. Aeschlimann M, Klein A, Schankin CJ. Visual snow syndrome (VSS)—recent advances in understanding the pathophysiology. Curr Opin Neurol. 2024;37(1):75–80. doi:10.1097/WCO.0000000000001276.
29. Garobbio M, Clough M, Solly EJ, Glarin T, D’Souza RD, White OB, et al. Brain network dynamics in people with visual snow syndrome. Hum Brain Mapp. 2023;44(16):5671–5685. doi:10.1002/hbm.26176.
30. Latini F, Fahlström M, Marklund N, Feresiadou A. White matter abnormalities in a patient with visual snow syndrome: new evidence from a diffusion tensor imaging study. Eur J Neurol. 2021;28(8):2789–2793. doi:10.1111/ene.14903.
31. Michels L, Stämpfli P, Aldusary N, Piccirelli M, Freund P, Weber KP, et al. Widespread white matter alterations in patients with visual snow syndrome. Front Neurol. 2021;12:723805. doi:10.3389/fneur.2021.723805.
32. Raviskanthan S, Ang EL, Suemoto CK, Klistorner A, Bell A, Nguyen BN, et al. Neuroimaging in visual snow: a review. Front Ophthalmol (Lausanne). 2022;1:49. doi:10.3389/fopht.2022.758963.
33. Chiong W, Wilson SM, D’Esposito M, Kayser AS, Grossman SN, Seeley WW, et al. The salience network causally influences default mode network activity during moral reasoning. Brain. 2013;136(Pt 6):1929–1941.
34. Peters SK, Dunlop K, Downar J. Cortico-striatal-thalamic loop circuits of the salience network: a central pathway in psychiatric disease and treatment. Front Syst Neurosci. 2016;10:104. doi:10.3389/fnsys.2016.00104.
35. Uddin LQ. Salience processing and insular cortical function and dysfunction. Nat Rev Neurosci. 2015;16(1):55–61. doi:10.1038/nrn3857.
36. Quentin R, Chanes L, Vernet M, Valero-Cabré A. Fronto-parietal anatomical connections influence the modulation of conscious visual perception by high-beta frontal oscillatory activity. Cereb Cortex. 2015;25(8):2095–2101. doi:10.1093/cercor/bhu079.
37. Puledda F, Ffytche D, O’Daley O, Goadsby PJ. Insular and occipital changes in visual snow syndrome: a BOLD fMRI and MRS study. Ann Clin Transl Neurol. 2020;7(3):296–306.
38. Vernet M, Quentin R, Chanes L, Mitsumasu A, Taylor PCJ, Valero-Cabré A. Frontal eye field, where art thou? Anatomy, function, and non-invasive manipulation of frontal regions involved in eye movements and associated cognitive operations. Front Integr Neurosci. 2014;8:66. doi:10.3389/fnint.2014.00066.
39. Schankin CJ, Maniyar FH, Chou DE, Puledda F, Goadsby PJ. Structural and functional footprint of visual snow syndrome. Brain. 2020;143(4):1106–1113. doi:10.1093/brain/awaa053.
40. Puledda F, Zelaya F, Schankin C, Goadsby PJ. Localised increase in regional cerebral perfusion in patients with visual snow syndrome: a pseudo-continuous arterial spin labelling study. Cephalalgia. 2018;38(Suppl 1):—.
41. Solly EJ, Clough M, McKendrick AM, Foletta P, White OB, Fielding J. The psychiatric symptomology of visual snow syndrome. Front Neurol. 2021;12:703006. doi:10.3389/fneur.2021.703006.
42. Grande RB, Solly EJ, Clough M, Fielding J, White OB. Quantitative and qualitative auditory dysfunction in visual snow syndrome. Front Neurol. 2021;12:724081. doi:10.3389/fneur.2021.724081.
43. Luna S, Lai D, Harris A, Rauschecker AM. Antagonistic relationship between VEP potentiation and gamma power in visual snow syndrome. Headache. 2018;58(1):138–144.
44. Aldusary N, Traber GL, Freund P, Fierz FC, Luechinger R, Weber KP, et al. Abnormal connectivity and brain structure in patients with visual snow. Front Hum Neurosci. 2020;14:582031. doi:10.3389/fnhum.2020.582031.
45. Omland PM, Nilsen KB, Uglem M, Gravdahl GB, Linde M, Hagen K, et al. Visual evoked potentials in interictal migraine: no confirmation of abnormal habituation. Headache. 2013;53(7):1071–1086.
46. Gersztenkorn D, Lee AG. Palinopsia revamped: a systematic review of the literature. Surv Ophthalmol. 2015;60(1):1– 35. doi:10.1016/j.survophthal.2014.11.003.
47. Halpern JH, Lerner AG, Passie T. A review of hallucinogen-induced persistent perception disorder (HPPD) and an exploratory study of subjects claiming symptoms of HPPD. Curr Top Behav Neurosci. 2018;36:333–360. doi:10.1007/7854_2016_57.
48. Di Carlo DT, Montemurro N, Gadda D, Benedetto N, Perrini P. Microsurgical anatomy of the sagittal stratum. Acta Neurochir (Wien). 2019;161(11):2319–2327. doi:10.1007/s00701-019-04051-3.
49. Silva EM, Puledda F. Visual snow syndrome and migraine: a review. Eye (Lond). 2023;37(12):2374–2378. doi:10.1038/s41433-022-02275-9.
50. Wong SH, Pontillo G, Kanber B, Prados F, Wingrove J, Yiannakas M, et al. Visual snow syndrome improves with modulation of resting-state functional MRI connectivity after mindfulness-based cognitive therapy: an open- label feasibility study. J Neuroophthalmol. 2024;44(1):112–118. doi:10.1097/WNO.0000000000002013.
51. Vaphiades MS, Wei EX, Sotoudeh H. Diagnostic evaluation of visual snow. Front Neurol. 2021;12:743608. doi:10.3389/fneur.2021.743608.
52. Yoo YJ, Park JK, Park HK, Baek IH, Choi JH, Lee JH. Visual snow syndrome: a potential clinical spectrum of posterior cortical dysfunction. J Clin Neurol. 2020;16(4):646–652. doi:10.3988/jcn.2020.16.4.646.
53. Eren O, Rauschecker AM, Giesemann AM, Schankin CJ. Evidence of dysfunction in the visual association cortex in visual snow syndrome. Ann Neurol. 2018;84(6):946–949. doi:10.1002/ana.25346.
54. Garobbio M, Solly EJ, Glarin T, Clough M, White OB, Fielding J, et al. Visual snow syndrome: symptom severity and quality of life are associated with dynamic functional connectivity changes in visual networks. Brain Commun. 2024;6(5):fcae341. doi:10.1093/braincomms/fcae341.
55. Solly EJ, Clough M, McKendrick AM, Foletta P, White OB, Fielding J. Ocular motor measures of visual processing changes in visual snow syndrome. Neurology. 2020;95(13):e1784–e1791. doi:10.1212/WNL.0000000000010372.
56. Hang L, Leishangthem L, Yan B. Visual snow: past, present, and future. Neuropsychiatr Dis Treat. 2021;17:3293–3300. doi:10.2147/NDT.S338111.
57. Han ME, Ciuffreda KJ, Rutner D. Visual snow: evolving understanding of the neural correlates and symptoms. Optom Vis Sci. 2023;100(5):328–333. doi:10.1097/OPX.0000000000002019.
58. Ciuffreda KJ, Tannen B, Rutner D, Han ME. Neuro-optometric treatment for visual snow syndrome: recent advances. Concussion. 2023;8(3):CNC110. doi:10.2217/cnc-2023-0006.
59. Visual Snow Initiative. First ever VSS RCT set to launch: MBCT-vision VSS RCT now recruiting [Internet]. 2025 [cited 2025 Sep 1]. Available from: https://www.visualsnowinitiative.org/research/how-mindfulness-based-therapy- may-help-people-with-visual-snow-syndrome-insights-from-a-new-research-perspective/
60. Ciuffreda KJ, Rutner D. Visual snow syndrome: therapeutic implications. J Clin Med. 2025;14(17):6070. doi:10.3390/jcm14176070.
61. Rusztyn P, Taylor M, White OB, Fielding J, van Gaal S, Solly EJ. Visual snow: a review on pathophysiology and treatment. J Clin Med. 2023;12(12):3868. doi:10.3390/jcm12123868.
62. Wong SH, Wingrove J. Mindfulness and MBCT-vision for visual snow syndrome: a therapeutic perspective. Front Neurol. 2025;16:1596642. doi:10.3389/fneur.2025.1596642.