Pediatric Visual Introduction Snow Syndrome (VSS): The clinical condition of “ syndrome” (VSS) is reported to be a relatively A Case Series rare and unique, cortically-based entity pri­ Kenneth J. Ciuffreda, OD, PhD1 marily described in the medical literature.1-15 Its M.H. Esther Han, OD hallmark characterization is the of Barry Tannen, OD “visual snow” (VS) appearing in a single plane in front of and throughout the entire visual field 1 SUNY/College of Optometry (Figure 1). VS has been described by some Brain Injury Clinical Research Unit as appearing like the electronic “snow” on a New York, New York television set when the primary video signal is either reduced or absent, as well as pixelated ABSTRACT fuzz and bubbles.1-15

ARTICLE Visual snow syndrome (VSS) is a relatively rare, unusual, and disturbing abnormal visual condition. The individual perceives “visual snow” (VS) throughout the entire visual field, as well as other abnormal visual phenomena (e.g., ). Only relatively recently has treatment been proposed (e.g., chromatic filters) in adults with VSS, but rarely in the pediatric VSS population (i.e., medications). In this paper, we present three well-documented cases of VSS in children, including their successful neuro-optometric therapeutic interventions (i.e., chromatic filters and saccadic-based vision ). Figure 1: Cat with overlay of visual snow as depicted by a patient.

Correspondence regarding this article should be emailed to Kenneth J. Ciuffreda, OD, PhD, at kciuffreda@sunyopt. edu. All statements­ are the authors’ personal opinions In addition to the presence of VS, and based and may not reflect the opinions of the College of on currently evolving criteria,2-15 the individual Optometrists in Vision Development, Vision Development must also report two or more of the following & Rehabilitation or any institu­tion or organization to which the authors may be affiliated. Permission to to be formally diagnosed with VSS: palinopsia, use reprints of this article must be obtained from the enhanced entoptic imagery, “nyctalopia”, editor. Copyright 2019 College of Optometrists in and photophobia/photosensitivity. These Vision Development. VDR is indexed in the Directory of Open Access Journals. Online access is available at individuals also frequently report having covd.org. https://doi.org/10.31707/VDR2019.5.4.p249. , photopsia, phonophobia, hyper­ Ciuffreda KJ, Han MH, Tannen B. Pediatric Visual Snow acusis, cutaneous allodynia, tinnitus, balance Syndrome (VSS): A case series. Vision Dev & Rehab problems, and tremor.2-15 2019;5(4):249-54. Due to its presumed extremely low prevalence, there is a relative paucity of reports in the literature on VSS in adults Keywords: neuro-optometry, vision, visual dating back to 1944,1-12 and only three case intervention, , visual snow, reports in children and adolescents ages visual snow syndrome 11-14 years over the past few years.13-15 In

249 Vision Development & Rehabilitation Volume 5, Issue 4 • December 2019 Table 1. Case Summaries Case 1 Case 2 Case 3 DEMOGRAPHICS Current age (in years) 10 10 15 Gender (M,F) M F F Age first noticed snow (in years) 10 8 11 Is visual snow constant (C) or transient (T)? C C T Is visual snow chromatic (C) or monochromatic (M)? C, M C, M C Provocative environments? Close objects None Bright lights; Computers Possible etiology? Pneumonia Brain cyst removal Migraine Possible co-morbid conditions? None None Migraine Treatment(s)? Tint Tint Tint & VT Medications? None Nortriptyline None

PRIMARY VISUAL SYMPTOMS Palinopsia √ √ Enhanced entoptic imagery √ √ Photosensitivity √ √ √ "Nyctalopia" (impaired night vision)

SECONDARY VISUAL/NONPVISUAL SYMPTOMS Photopsia √ √ Migraine √ Phonophobia Hyperacusis Cutaneous allodynia √ Tinnitus Balance problems √ √ Tremor these three cases, all conventional medical nocu­lar, and ocular health status. The case diagnostic testing was negative (e.g., brain descriptions will be restricted to the relevant imaging15), and all attempted drug treatments VSS details, as well as the other primary visual (e.g., sumatriptan15) were unsuccessful. None diagnoses. The key VSS findings are presented of the reports in the pediatric literature noted in Table 1 for each patient.12 any successful treatments such as chromatic filters or vision therapy. Neither were there any Case One successful medications directed solely at VS, The patient was a 10-year-old male who but rather only medications toward migraine first noticed VS soon after an extended bout symptoms or other coincident symptoms. (~4 weeks) of pneumonia for which he was Thus, we present a neuro-optometrically- successfully treated five months prior to his based, pediatric case series (ages 10-15 years) sensorimotor evaluation. The perception of of three patients with well-documented VSS. VS was constant and typically monochromatic This report includes both valuable diagnostic in nature. His primary VSS-related symptoms and therapeutic aspects for the clinician. included photosensitivity and enhanced entoptic imagery, whereas his secondary VSS- Case Presentations related symptoms included balance problems All received a comprehensive optometric and cutaneous allodynia. In addition, his other vision examination, including refractive, bi­ symptoms included intermittent diplopia

250 Vision Development & Rehabilitation Volume 5, Issue 4 • December 2019 at near, dizziness, positional vertigo, and was taking nortriptyline (25 mg, 3 times per gen­eral fatigue. Interestingly, all of these day) for depression and leg . symptoms worsened following remission of The patient was referred by her neuro- the pneumonia, and thus he was referred for ophthalmologist to the SUNY/University Eye additional medical care. The MRI and ENT Center, Vision Rehabilitation Service specifically results were normal. Moreover, the Lyme titer for a Cerium colorimetric evaluation. The was clear. There was no history of head trauma. basic vision examination revealed esophoria He was not taking any medications. at distance, low hyperopia, and saccadic The patient was then referred by a local dysfunction, for which she received successful optometrist to the SUNY/University Eye oculomotor-based vision therapy; that is, Center for a vision therapy evaluation. He symptoms reduced, and signs improved. was diagnosed with low hyperopia, binocular No refractive correction was prescribed at instability, and a receded near point of that time. Then, the Cerium-based (Intuitive convergence, and he is scheduled for vision Colorimeter),16 colorimetric testing was therapy in the near future. At the same time, performed. She required two different he was referred for a Cerium-based (Intuitive spectacle tints. One was for lessening the VS, Colorimeter), colorimetric examination.16 He which reduced it by approximately 75% (pink- preferred a 40% BPI-IR blue tint, which was purple, hue = 320, saturation = 25, Purple prescribed in spectacle form (CR-39). This A6, Purple Rose A6, Rose C4, 45% overall tint reduced most of his visual symptoms, transmission). The other was for reducing especially the VS and photosensitivity, thus the palinopsia duration when reading, which resulting in overall “improved visual comfort”. decreased the visual persistence from 17 Lastly, he had a neutral response to a session seconds to 5 seconds, and thus it improved her of optometric phototherapy (i.e., syntonics),17 reading comfort (turquoise/green, hue=180, so it was not pursued at this time. saturation=25, turquoise C3, green C3, 47% overall transmission). Case Two The patient was a 10-year-old female who Case Three first noticed VS within a day following surgical The patient was a 15-year-old female who removal of a brain cyst two years prior to first noticed VS at the age of 11 years, after her optometric evaluation. The cyst was first experiencing her first ocular migraine attack. discovered at age 3 years. The perception of VS The perception of VS was transient and was constant and typically monochromatic, but chromatic. It could be precipitated by bright also purple with a green background at times lights, fatigue, and computer screen viewing. (e.g., after yawning). Her primary VSS-related Her primary VSS-related symptoms included visual symptoms included photosensitivity palinopsia with trailing a few times per day, and palinopsia (especially when reading), enhanced chromatic entoptic imagery once whereas her secondary VSS-related symptoms per day, and constant photosensitivity, whereas included photopsia and balance problems. her VSS-related secondary symptom was In addition, her other symptoms included intermittent photopsia. The VS and palinopsia blurred vision, headaches, and loss of place, occurred independently. She also reported two all when reading, as well as the sensation of other interesting perceptual phenomena. First, dizziness and not feeling “grounded”. Prior at times, hallways were perceived to be longer, visual fields and dilated fundus examinations and objects appeared to be either closer (i.e., by two ophthalmologists were normal. She pelopsia) or farther (i.e., teleopsia) than their

251 Vision Development & Rehabilitation Volume 5, Issue 4 • December 2019 physical presence, with such size and distance tint prescribing. In addition, an ever increasing distortions suggesting Alice in Wonderland number of optometrists have knowledge Syndrome.18 Second, at times, she noticed a and experience in specialized colorimetric strobe/pulse effect surrounding letters during testing (i.e., Cerium Intuitive Colorimeter)16 reading. She had no history of concussion and to prescribe the optimal filter combination was not taking any medications. for maximum visual comfort and benefit (e.g., The patient was self-referred to a reading efficiency) in the patient with VSS. This private practice, neuro-optometrist for a would especially include the visual symptoms comprehensive vision evaluation. She was of VS, palinopsia, and photosensitivity/ diagnosed with accommodative insufficiency, photophobia. Lastly, others may be assistive convergence excess, saccadic oculomotor in the process, such as the occupational dysfunction, visual-directional problems, and therapist and ophthalmologist, to assess vision visual-memory deficits. She was successfully and related gains with the therapeutic lenses treated with oculomotor-based vision therapy within the context of their own discipline. and visual perceptual therapy; that is, symptoms Treatment for VSS is important for all reduced, and signs improved. The saccadic individuals, but perhaps even more so in young therapy reduced the palinopsia persistence children/adolescents, for several reasons. and related trailing,12,19 presumably by First, there is the possible psychological reestablishing a normal saccadic suppression effect of having such unusual and abnormal, inhibitory level.12 In addition, an FL-41 tint was undiagnosed, visual percepts. Some adult prescribed in spectacle form, which reduced patients with VSS indicated their initial the perception of VS. childhood reservation about telling this to a friend or family member, for fear of disbelief Discussion and even possible ridicule. Therefore, a VSS represents a new and exciting challenge detailed, probing case history may be to the neuro-optometrist, and others (e.g., the required to elicit the presence of VS in some ophthalmologist, neurologist). This syndrome patients, especially in those conditions with a has been a long misunderstood,9,18 somewhat relatively high probability of occurrence such perplexing, unique, and even questioned as concussion, brain , and taking a (especially in young children) diagnosis among new medication.12 Second, those manifesting the medical community, at least since 1944 palinopsia, which seems to be the majority,12 when visual snow was reported by some adult frequently find presence of their sustained patients taking digitalis for heart problems.1 and superimposed , especially with The neuro-optometrist has expertise in trailing, to be both distracting and disruptive general vision function/dysfunction, with when reading, as expected, thus likely leading specialty knowledge in optics (e.g., tint to reduced reading rate, reduced visual spectra, lens design), human visual perception comfort, and poorer comprehension. And (e.g., motion, saccadic suppression, reading), lastly, as they become of driving age, the and vision therapy/neurorehabilitation (e.g., presence of VSS, palinopsia, and/or enhanced perceptual and motor learning, oculomotor entoptic imagery, especially at night, may control). Thus, the neuro-optometrist can impact adversely on their driving ability and provide the comprehensive, basic vision safety due to impaired visual perception examination (i.e., refractive binocular, and and attentional distraction. If so, they should ocular health), as well as the vision therapy, be advised to obtain counselling from a and also both the general (e.g., neutral gray) professional driving trainer with expertise in and more advanced (e.g., FL-41, BPI Omega) those having visual impairment.20

252 Vision Development & Rehabilitation Volume 5, Issue 4 • December 2019 In an earlier paper on this topic,12 we 6. Santos-Bueso E, Sastre-Ibanez M, Saenz-Frances F et al. Visual snow: from a symptom to a syndrome? Arch Soc had proposed a “unifying hypothesis”, or Esp Oftalmol 2015; 9:47-52. mechanism, to understand many/all aspects 7. Fraser CL, White OB. There’s something in the air. Sur of VSS, namely neural “disinhibition”. Ophthal 2018; 63: 1-5. Others had proposed the mechanism to 8. Ghannam B, Pelak VS. Visual snow: a potential cortical be a “hypersensitivity phenomenon”.8 At hyperexcitability syndrome. Curr Treat Options Neurol 2017; 19: 8-20. first blush, this appeared to be descriptive. 9. Schankin CJ, Maniyar FH, Sprenger T, et al. The relation While not necessarily incorrect, it seems to between migraine, typical migraine aura, and “visual emphasize the wrong aspect. These patients snow”. Headache 2014; 54: 957-966. are not hypersensitive per se, but rather 10. Laushke JL, Plant GL, Fraser CL. Visual snow: a thalamocortical dysrhythmia of the visual pathway? J Clin appear to exhibit abnormally-reduced levels of Neurosci 2016; 28: 123-127. naturally-occurring neural inhibition/have less 11. Metzler AI, Robertson CE. Visual snow syndrome: proposed suppression. This idea is supported by recent criteria, clinical implications, and pathophysiology. Curr 21,22 Neurol Neurosci Rep 2018; 18:52. https://doi.org/10.1007/ laboratory findings. Thus, the patient with s11910-018-0854-2 VSS sees/experiences what others do not 12. Ciuffreda KJ, Tannen B, Han ME. Visual snow syndrome see (e.g., palinopsia) or feel (e.g., cutaneous (VSS): an evolving neuro-optometric perspective. Vis Dev allodynia). Rehab 2019; 5: 75-82. 13. Bruen R, Peng SL, Perreault S, Major P, Ospina LH. In the future, a randomized clinical trial Persistent migraine aura in an adolescent girl. J AAPOS (RCT) is warranted to assess of the 2013; 17: 426-427. two current neuro-optometric, therapeutic 14. Santos-Bueso E, Munoz-Hernandez N, Avalos-Franco N, interventions, namely tinted spectacles and Garcia-Saenz S, Saenz-Frances F. Porta-Etessom J. Visual snow in a pediatric patient. Arch Soc Esp Oftalmol. 2017; oculomotor-based vision therapy, separately 92: 602-604. and combined, in conjunction with a placebo 15. Simpson JC, Goadsby PJ, Prabhakar P. Positive, persistent condition.23 However, a cross-over, interventional visual symptoms (visual snow) presenting as a migraine experimental design would also be both variant in a 12-year-old girl. Pediatr Neurol 2013; 49:361-363. 21 16. Willeford KT, Fimreite V, Ciuffreda KJ. The effect of spectral acceptable and more efficient. If proven to filters on VEP and alpha-wave responses. J Optom 2016; provide symptomatic relief (e.g., per the VSS 9:110-117. questionnaire)12 and/or increased visual/reading 17. Collier S. In syntony. Herstellung and Verlag, Norderstedr, efficiency (e.g., per the objective Visagraph Germany, 2011. 24 18. Rastogi RG, VanderPluym J, Lewis KS. Migrainous aura, reading testing), additional studies would be visual snow, and “Alice in Wonderland” Syndrome in helpful to determine retention of the long-term childhood. Sem Pediatr Neurol 2016; 23:14-17. benefits and any visual adaptation effects. 19. Ciuffreda KJ, Ludlam DP, Kapoor N. Clinical oculomotor training in traumatic brain injury. Optom Vis Dev 2009; 40: 16-23. References 20. Gianutsos R, Campbell A, Beattie A, Mandriota F. The 1. Carroll F. Visual symptoms caused by digitalis. Trans Amer driving advisement system: a computer augmented quasi- Ophthal Soc. 1944; 42:243-249. simulation of the cognitive prerequisites for resumption 2. Puledda F, Schankin C, Digre K, Goadsby PJ. Visual snow of driving after brain injury. Assistive Tech. 1992; 4: 70-86. syndrome: what we know so far. Curr Opin Neurol 2018; 21. Mc Kendrick AM, Chan YM, Tien M, et al. Behavioral 31: 52-58. measures of cortical hyperexcitability assessed in people 3. White OB, Clough M, McKendrick AM, Fielding J. Visual who experience visual snow. Neurol 2017; 88; 1243-1249. snow: visual misperception. J Neuro-Ophthalmol 2018; 22. Yildiz FG, Turkyilmaz U, Unal-Cevik I. The clinical 38: 514-521. characteristics and neurophysiological assessments of the 4. Schankin C, Maniyar FH, Digre KB, Goadsly PJ. “Visual occipital cortex in visual snow syndrome with or without snow”- a disorder distinct from persistent migraine aura. migraine. Headache 2019;59(4):484-494. Brain 2014; 137: 1419-1428. 23. Hatch SW. Ophthalmic research and epidemiology. 5. Liu GT, Svatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Butterworth-Heinemann, Boston MA, 1998, pp. 61-110. Komorsky GS. Persistent positive visual phenomena in 24. Ciuffreda KJ, Tannen B. basics for the migraine. Neurol 1995; 45: 664-668. clinician. St. Louis: Mosby Year Book, 1995.

253 Vision Development & Rehabilitation Volume 5, Issue 4 • December 2019 CORRESPONDING AUTHOR Jersey Institute of Technology, both in the department of BIOGRAPHY: biomedical engineering. He also helped establish a school Kenneth J. Ciuffreda, OD, PhD of optometry in Harbin, China. He has conducted research New York, New York in many areas: amblyopia, strabismus, reading, myopia, eye Kenneth J. Ciuffreda received his B.S movements, accommodation, bioengineering applications in biology from Seton Hall University in to optometry, and more recently with an emphasis in the area 1969, his O.D. from the Massachusetts of acquired brain injury, both the diagnostic and therapeutic College of Optometry in 1973, and his aspects. His goal has been the use of objective recording Ph.D. degree in physiological optics from techniques in the diagnosis and treatment of neurological the University of California/School Optometry at Berkeley in and ocular conditions. He holds two patents, and has 1977. He has been a faculty member at the SUNY/State received many awards and honors from the AAO, AOA, College of Optometry in New York City since 1979, where NORA, COVD, and various state optometric associations he is presently a Distinguished Teaching Professor. He has and colleges. He has authored over 400 research papers/ also had adjunct appointments for many years at Rutgers/ chapters, and 10 books. His hobbies are playing jazz guitar The State University of New Jersey, as well as at the New and enjoying the visual aspects of art.

254 Vision Development & Rehabilitation Volume 5, Issue 4 • December 2019