Abstract
Purpose of Review
In this article, we review illustrative case descriptions of both primary and secondary visual snow from our clinic. We discuss recently proposed criteria for visual snow syndrome and offer a slight modification of these criteria. We also discuss the theories on the pathophysiological mechanisms of visual snow, as well as the current approach to treatment.
Recent Findings
Visual snow is a condition where patients see constant, innumerable flickering dots throughout the visual field, similar to “TV static.” Though visual snow was originally described in 1995, there were still fewer than 10 cases in the literature prior to 2014. In the last 4 years, this has grown to approximately 200 cases and there has been a concentrated effort to better understand and characterize this condition. It has become apparent that patients who see visual snow frequently have additional visual and non-visual symptoms, and the consistency of these symptoms has led to proposed criteria for visual snow syndrome.
Summary
When seeing a patient with visual snow, it is important to rule out a possible underlying secondary etiology. Patients with visual snow syndrome frequently have comorbid migraine, but visual snow appears to be a separate entity from persistent migraine aura. The pathophysiology of this syndrome is not clear, but recent neurophysiologic and neuroimaging studies have helped advance our understanding.
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References
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•• Schankin CJ, Maniyar FH, Digre KB, Goadsby PJ. ‘Visual snow’—a disorder distinct from persistent migraine aura. Brain. 2014a;137(Pt 5):1419–28. Visual snow syndrome is first proposed and defined in this article, while reviewing 78 cases of visual snow.
Santos-Bueso E, Sastre-Ibanez M, Saenz-Frances F, Porta-Etessam J, Garcia-Sanchez J. Visual snow. From a symptom to a syndrome? Arch Soc Esp Oftalmol. 2015;90(1):51–2.
• Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995;45(4):664–8. A case series of 10 patients with persistent positive visual phenomena and the first paper to describe visual snow and recognize its possible association with migraine.
• Lauschke JL, Plant GT, Fraser CL. Visual snow: a thalamocortical dysrhythmia of the visual pathway? J Clin Neurosci. 2016;28:123–7. The characteristics of a case cohort of 32 patients with visual snow is provided, with a discussion on the possible role of colored lenses as a treatment for visual snow.
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(9):1057–8.
Zambrowski O, Ingster-Moati I, Vignal-Clermont C, Robert MP. The visual snow phenomenon. J Fr Ophtalmol. 2014;37(9):722–7.
Beyer U, Gaul C. Visual snow. Nervenarzt. 2015;86(12):1561–5.
•• Puledda F, Schankin C, Digre K, Goadsby PJ. Visual snow syndrome: what we know so far. Curr Opin Neurol. 2018;31(1):52–8. This is a very well-written review of what we understand about the pathophysiology and characterization of visual snow.
Evans RW, Aurora SK. Migraine with persistent visual aura. Headache. 2012;52(3):494–501.
Koyama S, Kawamura M. Persistent visual aura following catheter ablation in a patient with WPW syndrome. Behav Neurol. 2007;18(3):187–92.
Tegetmeyer H. Visual snow syndrome: symptoms and ophthalmological findings. Klin Monatsbl Augenheilkd. 2017;234(5):713–8.
Luna S, Lai D, Harris A. Antagonistic relationship between VEP potentiation and gamma power in visual snow syndrome. Headache. 2018;58(1):138–44.
Schankin CJ, Goadsby PJ. Visual snow—persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015;19(6):23.
Sinclair SH, Azar-Cavanagh M, Soper KA, Tuma RF, Mayrovitz HN. Investigation of the source of the blue field entoptic phenomenon. Invest Ophthalmol Vis Sci. 1989;30(4):668–73.
•• Schankin CJ, Maniyar FH, Sprenger T, Chou DE, Eller M, Goadsby PJ. The relation between migraine, typical migraine aura and “visual snow”. Headache. 2014b;54(6):957–66. Neuroimaging study showing hypermetabolism of the lingual gyrus in patients with visual snow.
Tyler CW. Some new entoptic phenomena. Vis Res. 1978;18(12):1633–9.
Kanai R, Paulus W, Walsh V. Transcranial alternating current stimulation (tACS) modulates cortical excitability as assessed by TMS-induced phosphene thresholds. Clin Neurophysiol. 2010;121(9):1551–4.
Samaha J, Gosseries O, Postle BR. Distinct oscillatory frequencies underlie excitability of human occipital and parietal cortex. J Neurosci. 2017;37(11):2824–33.
Kolmel HW. Complex visual hallucinations in the hemianopic field. J Neurol Neurosurg Psychiatry. 1985;48(1):29–38.
Marshall CR. Entoptic phenomena associated with the retina. Br J Ophthalmol. 1935;19(4):177–201.
Bowen SF Jr. Retinal entoptic phenomena. Some diagnostic use. Arch Ophthalmol. 1963;69:551–5.
Abraham HD. Visual phenomenology of the LSD flashback. Arch Gen Psychiatry. 1983;40(8):884–9.
Pomeranz HD, Lessell S. Palinopsia and polyopia in the absence of drugs or cerebral disease. Neurology. 2000;54(4):855–9.
Kawasaki A, Purvin V. Persistent palinopsia following ingestion of lysergic acid diethylamide (LSD). Arch Ophthalmol. 1996;114(1):47–50.
Levi L, Miller NR. Visual illusions associated with previous drug abuse. J Clin Neuroophthalmol. 1990;10(2):103–10.
Martinotti G, Santacroce R, Pettorruso M, Montemitro C, Spano MC, Lorusso M, et al. Hallucinogen persisting perception disorder: etiology, clinical features, and therapeutic perspectives. Brain Sci. 2018;8(3)
Hughes MS, Lessell S. Trazodone-induced palinopsia. Arch Ophthalmol. 1990;108(3):399–400.
Ihde-Scholl T, Jefferson JW. Mitrazapine-associated palinopsia. J Clin Psychiatry. 2001;62(5):373.
Faber RA, Benzick JM. Nafazodone-induced palinopsia. J Clin Psychopharmacol. 2000;20(2):275–6.
Lauterbach EC, Abdelhamid A, Annandale JB. Posthallucinogen-like visual illusions (palinopsia) with risperidone in a patient without previous hallucinogen exposure: possible relation to serotonin 5HT2a receptor blockade. Pharmacopsychiatry. 2000;33(1):38–41.
Yun SH, Lavin PJ, Schatz MP, Lesser RL. Topiramate-induced palinopsia: a case series and review of the literature. J Neuroophthalmol. 2015;35(2):148–51.
Choi SY, Jeong SH, Kim JS. Clomiphene citrate associated with palinopsia. J Neuroophthalmol. 2017;37(2):220–1.
Purvin VA. Visual disturbance secondary to clomiphene citrate. Arch Ophthalmol. 1995;113(4):482–4.
Vaphiades MS, Celesia GG, Brigell MG. Positive spontaneous visual phenomena limited to the hemianopic field in lesions of central visual pathways. Neurology. 1996;47(2):408–17.
Gersztenkorn D, Lee AG. Palinopsia revamped: a systematic review of the literature. Surv Ophthalmol. 2015;60(1):1–35.
Santhouse AM, Howard RJ, Ffytche DH. Visual hallucinatory syndromes and the anatomy of the visual brain. Brain. 2000;123(Pt 10):2055–64.
Panayiotopoulos CP. Visual phenomena and headache in occipital epilepsy: a review, a systematic study and differentiation from migraine. Epileptic Disord. 1999;1(4):205–16.
Bien CG, Benninger FO, Urbach H, Schramm J, Kurthen M, Elger CE. Localizing value of epileptic visual auras. Brain. 2000;123(Pt 2):244–53.
Olesen J, Larsen B, Lauritzen M. Focal hyperemia followed by spreading oligemia and impaired activation of rCBF in classic migraine. Ann Neurol. 1981;9(4):344–52.
Andersen AR, Friberg L, Olsen TS, Olesen J. Delayed hyperemia following hypoperfusion in classic migraine. Single photon emission computed tomographic demonstration. Arch Neurol. 1988;45(2):154–9.
Cutrer FM, Sorensen AG, Weisskoff RM, Ostergaard L, Sanchez del Rio M, Lee EJ, et al. Perfusion-weighted imaging defects during spontaneous migrainous aura. Ann Neurol. 1998;43(1):25–31.
Hougaard A, Amin FM, Christensen CE, Younis S, Wolfram F, Cramer SP, et al. Increased brainstem perfusion, but no blood-brain barrier disruption, during attacks of migraine with aura. Brain. 2017;140(6):1633–42.
Olesen J, Friberg L, Olsen TS, Iversen HK, Lassen NA, Andersen AR, et al. Timing and topography of cerebral blood flow, aura, and headache during migraine attacks. Ann Neurol. 1990;28(6):791–8.
Jager HR, Giffin NJ, Goadsby PJ. Diffusion- and perfusion-weighted MR imaging in persistent migrainous visual disturbances. Cephalalgia. 2005;25(5):323–32.
Luda E, Bo E, Sicuro L, Comitangelo R, Campana M. Sustained visual aura: a totally new variation of migraine. Headache. 1991;31(9):582–3.
Chen WT, Fuh JL, Lu SR, Wang SJ. Persistent migrainous visual phenomena might be responsive to lamotrigine. Headache. 2001;41(8):823–5.
Relja G, Granato A, Ukmar M, Ferretti G, Antonello RM, Zorzon M. Persistent aura without infarction: decription of the first case studied with both brain SPECT and perfusion MRI. Cephalalgia. 2005;25(1):56–9.
Boulloche N, Denuelle M, Payoux P, Fabre N, Trotter Y, Geraud G. Photophobia in migraine: an interictal PET study of cortical hyperexcitability and its modulation by pain. J Neurol Neurosurg Psychiatry. 2010;81(9):978–84.
•• Denuelle M, Boulloche N, Payoux P, Fabre N, Trotter Y, Geraud G. A PET study of photophobia during spontaneous migraine attacks. Neurology. 2011;76(3):213–8. This study demonstrates possible involvement of the lingual gyrus in photophobia, raising the question of overlap with the lingual hypermetabolism on imaging for visual snow.
Schankin CJ, Viana M, Goadsby PJ. Persistent and repetitive visual disturbances in migraine: a review. Headache. 2017;57(1):1–16.
Bou Ghannam A, Pelak VS. Visual snow: a potential cortical hyperexcitability syndrome. Curr Treat Options Neurol. 2017;19(3):9.
•• Ffytche DH. The hodology of hallucinations. Cortex. 2008;44(8):1067–83. Excellent review of the structural and physiologic pathology that might lead to visual hallucinations, including information from fMRI, EEG, and tractography studies.
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(26):15222–7.
Unal-Cevik I, Yildiz FG. Visual snow in migraine with aura: further characterization by brain imaging, electrophysiology, and treatment—case report. Headache. 2015;55(10):1436–41.
Chen WT, Lin YY, Fuh JL, Hamalainen MS, Ko YC, Wang SJ. Sustained visual cortex hyperexcitability in migraine with persistent visual aura. Brain. 2011;134(Pt 8):2387–95.
Megela AL, Teyler TJ. Habituation and the human evoked potential. J Comp Physiol Psychol. 1979;93(6):1154–70.
Fantini J, Sartori A, Manganotti P. Can we speak of lack of habituation in visual snow? Headache. 2016;56(9):1517–8.
Afra J, Proietti Cecchini A, Sandor PS, Schoenen J. Comparison of visual and auditory evoked cortical potentials in migraine patients between attacks. Clin Neurophysiol. 2000;111(6):1124–9.
Coppola G, Pierelli F, Schoenen J. Is the cerebral cortex hyperexcitable or hyperresponsive in migraine? Cephalalgia. 2007;27(12):1427–39.
Lerner AG, Oyefe I, Isaacs G, Sigal M. Naltrexone treatment of hallucinogen persisting perception disorder. Am J Psychiatry. 1997;154(3):437.
Lugo E, Doti R, Faubert J. Ubiquitous crossmodal stochastic resonance in humans: auditory noise facilitates tactile, visual and proprioceptive sensations. PLoS One. 2008;3(8):e2860.
Trevino M, De la Torre-Valdovinos B, Manjarrez E. Noise improves visual motion discrimination via a stochastic resonance-like phenomenon. Front Hum Neurosci. 2016;10:572.
Harper DW. Signal detection analysis of effect of white noise intensity on sensitivity to visual flicker. Percept Mot Skills. 1979;48(3 Pt 1):791–8.
Manjarrez E, Mendez I, Martinez L, Flores A, Mirasso CR. Effects of auditory noise on the psychophysical detection of visual signals: cross-modal stochastic resonance. Neurosci Lett. 2007;415(3):231–6.
Collins JJ, Chow CC, Imhoff TT. Stochastic resonance without tuning. Nature. 1995;376(6537):236–8.
• Moss F, Ward LM, Sannita WG. Stochastic resonance and sensory information processing: a tutorial and review of application. Clin Neurophysiol. 2004;115(2):267–81. Excellent review of the possible role of stochastic resonance in sensory processing.
Drummond PD. Motion sickness and migraine: optokinetic stimulation increases scalp tenderness, pain sensitivity in the fingers and photophobia. Cephalalgia. 2002;22(2):117–24.
van der Groen O, Wenderoth N. Transcranial random noise stimulation of visual cortex: stochastic resonance enhances central mechanisms of perception. J Neurosci. 2016;36(19):5289–98.
Program Abstracts: American Headache Society(R) 58th Annual Scientific Meeting. Headache. 2016;56(Suppl 1):3–83.
Sastre-Ibanez M, Santos-Bueso E, Porta-Etessam J, Garcia-Feijoo J. Visual snow: report of three cases. J Fr Ophtalmol. 2015;38(7):e157–8.
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(12):1298–304.
Parra J, Lopes da Silva FH, Stroink H, Kalitzin S. Is colour modulation an independent factor in human visual photosensitivity? Brain. 2007;130(Pt 6):1679–89.
Huang J, Zong X, Wilkins A, Jenkins B, Bozoki A, Cao Y. fMRI evidence that precision ophthalmic tints reduce cortical hyperactivation in migraine. Cephalalgia. 2011;31(8):925–36.
Acknowledgements
Thank you to Mayo Clinic medical illustrator Bryce Bergene, for creating Fig. 1.
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Abby I. Metzler declares no conflict of interest.
Carrie E. Robertson has served on advisory boards for Amgen and Alder.
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Metzler, A.I., Robertson, C.E. Visual Snow Syndrome: Proposed Criteria, Clinical Implications, and Pathophysiology. Curr Neurol Neurosci Rep 18, 52 (2018). https://doi.org/10.1007/s11910-018-0854-2
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DOI: https://doi.org/10.1007/s11910-018-0854-2