Tamara Bystrak

PharmD Candidate
 Introduce Visual Snow (VS) as a newly
recognized neurological disorder
 Highlight visual symptoms in proposed
diagnostic criteria for VS
 Discuss and debate possible
pathophysiology behind VS
 Review treatment used in recent trials and
case reports
 Understand the impact VS has on lifestyle
  A disorder of altered visual perception
 Patients see continuous tiny dots across the
entire visual field
 Analogous to the pixels on an old analogue
television
 Occurs 24/7 with eyes open and closed
 Simulation: http://www.visualsnow.eu/visual-snow-simulators/

Schankin CJ. Visual snow--persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015.
Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995 Apr;45(4):664-8.

 Visual snow was conventionally regarded as a variant of

migraine aura known as Persistent Migraine Aura Without
Infarction (PAWOI)
 Case reports from 1995 describe patients with “unusual
migraine complications” involving “persistent positive visual
phenomena …usually consisted of diffuse small particles
such as TV static, snow, lines of ants, dots, and rain”
 Visual snow is also commonly confused with floaters by
opticians and doctors
 In the last few years, the medical community has begun to
recognize visual snow as a distinct neurological disorder,
separate from migraine
Haas DC. Prolonged migraine aura status. Ann Neurol 1982; 11: 197-199
Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent positive visual phenomena in migraine. Neurology. 1995, 45(4):664-8.
  Spearheading research in visual snow, with help
from Dr. Peter Goadsby and the rest of the
Headache Group at UCSF
 Leading author of several VS studies in the last 5
years
 Spread visual snow awareness by presenting at
the American Academy of Neurology's 64th Annual
Meeting in New Orleans and the 54th Annual
Scientific Meeting of the American Headache
Society (2012)

Eye on Vision Foundation. Update of visual snow study from UCSF. URL: http://www.eyeonvision.org/news/102-update-on-visual-snow-study-from-ucsf.html
 The relation between migraine, typical migraine
aura and visual snow. Headache. 2014
A prospective semi-structured telephone interview
 N=120 patients with visual snow
 N=70 had comorbid migraine

Positron emission tomography (PET) Scans

 N=17 patients with visual snow matched against n=17 control
subjects

Conclusions:
• Comorbid migraine aggravates the clinical phenotype of the "visual
snow" syndrome
• The hypermetabolic lingual gyrus confirms a brain dysfunction in
patients with "visual snow"
 'Visual snow' - a disorder distinct from persistent
migraine aura. Brain. 2014

1. A chart review of n=22 presented for diagnosis and management

2. A retrospective internet survey (n = 275) of self-assessed 'visual snow'
subjects done by Eye On Vision Foundation. Two random samples
from 235 complete data sets
3. A prospective semi-structured telephone interview in 142 patients
(n=78 confirmed VS)

Conclusions:
• Visual snow is a unique visual disturbance clinically distinct from
migraine aura that can be disabling for patients
• Migraine is a common concomitant although standard migraine
treatments are often unhelpful
 Migraine (with • 70/120 (58%) 1
• 46/78 (59%) 2
or without • Often present with more severe
aura) symptoms

• Ringing or buzzing in the ears

Tinnitus • 77/120 (64%) 1
• 48/78 (62%) 2

• 1/17 depression (6%) 1

Anxiety and/or • 12/53 anxiety (23%) 2
• 11/53 depression (21%) 2
Depression • 23/53 (43%) overall 2

1) Schankin CJ. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014
2) Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
  Populations
 Majority young and otherwise healthy
 Often in the second to fourth decade of life

 Trigger
 Most cannot remember a clear trigger
 A small percentage report head trauma, migraine attacks, or use of
illicit or prescription drugs precipitating initial episode of VS
 Aminoglycoside antibiotics, opioids

 Onset
 At 21 + 9 years (76%)
 Since earliest memory (24%)

 Duration
 visual snow rarely disappears once it appears

Schankin CJ. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014
Fulton, James T., Processes in Biological Vision {online} {Corona Del Mar, CA. USA} Vision Concepts, 2000, URL:http://neuronresearch.net/vision/
 Progressive Worsening

Progressive Worsening to
Constant Symptoms

Stepwise Worsening to
Constant Symptoms

Constant Symptoms

Schankin CJ. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014
 ADDITIONAL SYMPTOMS

Entopic
phenomena

Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
 Persistence of a visual image
following removal of the exciting
stimulus

Trailing: A moving object

in the visual field is not
perceived as one object,
but as a smear of objects
’following‘ the object

Afterimages: an image
continuing to appear in
one's vision after the
exposure to the original
image has ceased

Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
 Sensitivity to light
(too bright, painful, or both)

http://dxline.info/img/new_ail/photophobia_1.jpg
 Impaired night vision

https://upload.wikimedia.org/wikipedia/commons/b/b6/P360_Onderdendam_goed_nachtzicht_ns_nachtblind.jpg
 Visual effects whose source is
within the eye itself

Spontaneous photopsia:
Short-lasting sensation of light in one or both eyes in
absence of an external light source
Described to patients as: bright flashes occur briefly,
then fade

Self light of the eye:

Patient notes luminous moving clouds of purple or
orange color with eyes closed
Described to patients as: swirls, clouds or waves with
eyes closed

Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
 Floaters: Perception of spots or
strands in vision, which follow the
eye movement and have the
tendency to drift slowly

Blue field entopic phenomenon:

Moving corpuscles (small cells)
when looking at bright surfaces or
the blue sky. Movement was
usually pulsating (wiggling)

Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
 A diagnosis of exclusion

Tegetmeyer H. [Visual Snow Syndrome: Symptoms and Ophthalmological Findings]. Klin Monbl Augenheilkd. 2016 Aug 10. [Epub ahead of print] German.
 Hallucinogen
Persistent migraine Psychogenic
Persisting Perception
aura Disorder/Malingering
Disorder (HPPD)
• Estimated 40% of • Also experience • Complaints often
patients with VS do starbursts, ignored or thought of
not have migraines afterimages, as an exaggeration
• Symptomatic palinopsia • Brain scans show
differences distinguish • Less than 5% of anomaly in lingual
migraine aura from patients with gyrus
visual snow confirmed VS report • Consistency of
• Episodic vs constant any drug use symptomatic reports
(or progressive) • Even fewer report use
of hallucinogenic
drugs
• Presence in children

Schankin CJ. Visual snow--persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015.
Schankin CJ, 'Visual snow' - a disorder distinct from persistent migraine aura. Brain. 2014
  Chemical imbalance of glutamic acid and gamma amino butyric acid
(GABA) associated with the higher visual centers of the thalamus (upper
brainstem), parietal lobe, or prefrontal lobe 1

 Alterations in synaptic connectivity along the visual pathway2

 Corticocortical interconnectivity
 Corticosubcortical interconnectivity (thalamocortical)

 Hypermetabolism in the supplementary visual cortex, or lingual gyrus3

 Modulates visual processing
 Pathophysiologic overlap with migraine but hypermetabolism not seen
in interictal migrainers without VS

1) Fulton, James T., Processes in Biological Vision {online} {Corona Del Mar, CA. USA} Vision Concepts, 2000. URL:http://neuronresearch.net/vision/
2) Lauschke JL, Plant GT, Fraser CL. Visual snow: A thalamocortical dysrhythmia of the visual pathway? J Clin Neurosci. 2016 Jun;28:123-7.
3) Schankin CJ, Curr Pain Headache Rep. 2015
 Visual
Snow

Healthy
Control

When comparing the brain metabolism of patients with “visual snow” to healthy
controls in [18F]-FDG PET using a paired t-test in SPM8, the right lingual
gyrus and the anterior lobe of the left cerebellum were metabolically more
active in patients with “visual snow.” P ≤ .001.

Schankin CJ. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014
 Using [18F]-FDG positron emission tomography, brain hypermetabolism in the lingual gyrus
was demonstrated in B) visual snow patients. Comparison with H215O positron emission
tomography after light stimulation during migraine attacks C) was used as a model for
photophobia in migraineurs. This revealed the same area in the lingual gyrus being
hyperperfused, suggesting some relevance of this area for visual snow pathophysiology and
for the clinical overlap of migraine/aura with visual snow

Schankin CJ. Visual snow--persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015.
  It has been hypothesized that the lingual gyrus is involved in1…
 Encoding visual memory
 Processing letters
 Perception of color
 Identification of facial expressions of emotions

 Disease states involving the lingual gyrus

 MDD responsiveness to antidepressant treatment 2
 PTSD severity 3
 Social Anxiety 4
 Grapheme-color Synesthesia 5

1. Schankin CJ. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014
2. Jung J, Kang J, Won E, Nam K, Lee MS, Tae WS, Ham BJ. Impact of lingual gyrus volume on antidepressant response and neurocognitive
functions in Major Depressive Disorder: a voxel-based morphometry study. J Affect Disord. 2014 Dec;169:179-87.
3. Wang T, Liu J, Zhang J, Zhan W, Li L, Wu M, Huang H, Zhu H, Kemp GJ, Gong Q. Altered resting-state functional activity in posttraumatic
stress disorder: A quantitative meta-analysis. Sci Rep. 2016 Jun 2;6:27131
4. Waugh CE, Hamilton JP, Chen MC, Joormann J, Gotlib IH. Neural temporal dynamics of stress in comorbid major depressive disorder and
social anxiety disorder. Biol Mood Anxiety Disord. 2012 Jun 22;2:11.
5. Jäncke L, Beeli G, Eulig C, Hänggi J. The neuroanatomy of grapheme-color synesthesia. Eur J Neurosci. 2009 Mar;29
 The lingual gyrus is part of Brodmann area
19 (BA 19). This is in the occipital lobe of the cortex.
Schankin CJ, Goadsby PJ. Visual snow--persistent positive visual phenomenon distinct from migraine aura. Curr Pain Headache Rep. 2015 Jun;19(6):23.
  SSRIs
 Lamotrigine
 Benzodiazepines
 Acetazolamide
 Valproic acid
 Levetiracetam
 Tricyclic antidepressants
 Calcium channel blockers
 Beta blockers
 Analgesics
 Melatonin
 Vitamins: B6, B12, Mg
 Gabapentin
 Furosemide

Bessero AC, Plant GT. Should 'visual snow' and persistence of after-images be recognised as a new visual syndrome? J Neurol Neurosurg Psychiatry. 2014.
Schankin CJ,. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014.
 Table 3 Summary. Current and Past Treatment Trials for “Visual Snow” in 17
VS Patients Who Took Part in the [18F]-FDG PET Study

Patient Drugs Trial Effect

Patient 2 Sertraline 6 months No effect
Fluoxetine
Patient 4 Bupropion Current No effect
Topiramate
Patient 5 Fluoxetine, N/A No effect
Verapamil
Lamotrigine
Patient 5 Sertraline N/A Worsening
Patient 6 Amitriptyline, 2 months No effect
Propranolol
Patient 7 Naproxen N/A Improvement
Patient 8 Sertraline N/A No effect
Clonazepam
**11/17 patients had no current or trialed medications for VS

Schankin CJ,. The relation between migraine, typical migraine aura and "visual snow". Headache. 2014
  A case report of 25 y.o female with migraine with
aura x 10yr and VS x 1yr
 Comorbid anxiety treated with fluoxetine 40mg
daily x 2 months
 No illicit drug use
 Normal EEG
 Sx: continuous bright and colorful lights,
palinopsia, floaters, nyclatopsia, photopsia
 Chose lamotrigine based on 2 previous case
reports of success with lamotrigine for persistent
migranous visual phenomena
Unal-Cevik I, Yildiz FG. Visual Snow in Migraine With Aura: Further Characterization by Brain Imaging, Electrophysiology, and Treatment--Case Report.
Headache. 2015 Nov-Dec;55(10):1436-41.
  Lamotrigine titrated up to 50mg BID
 After tx repetitive pattern reversal visual evoked
potentials (rVEP) show improved cortical
excitability
 After tx patient reports
 Palinopsia improved 80%
 Brightness/density of floaters, dots, photopsia
improved 50%
 Improvements in blue field entopic phenomena and
nyctalopia < 30%
 Now able to sleep
 Migraine frequency from 2-3 attacks/wk to 2
attacks/month

Unal-Cevik I, Yildiz FG. Visual Snow in Migraine With Aura: Further Characterization by Brain Imaging, Electrophysiology, and Treatment--Case Report.
Headache. 2015 Nov-Dec;55(10):1436-41.
The Newest Data
TREATMENT EFFECT IN VISUAL SNOW
Puledda F, Schankin C, Goadsby PJ
 • Topiramate, sodium valproate,
propranolol, amitriptyline, lamotrigine,
NOT gabapentin, nortriptyline, tizanidine,
HELPFUL verapamil, imipramine

• Riboflavin 400mg, magnesium citrate

600mg + coenzyme Q-10 350 mg,
HELPFUL acetazolamide, levetiracetam 1000 mg
 Fatigue  Drinking alcohol
 Excessive stress  Smoking cigarettes
 Poor Diet  Marijuana
 Obsessing over sx  Adderall
 Treatment is often aimed at comorbid
depression, anxiety, tinnitus, migraine
 Most drugs trialed don’t work at all
 Some drugs have partial effect
 Some drugs work but only temporarily
 Highest evidence for efficacy is with
benzodiazepines, antiepileptic drugs, and
vitamins
 Psychotherapy:

Yellow-blue Diet: low salt,

color filters sugar, alcohol

Distraction Exercise

Relaxation:
(meditation yoga)

Lauschke JL, Plant GT, Fraser CL. Visual snow: A thalamocortical dysrhythmia of the visual pathway? J Clin Neurosci. 2016 Jun;28:123-7.
  Thinks that this phenomenon is actually extremely common, just
neglected in the scientific literature
 Those with the disturbance "tend to be people who are very
observant ... people who are really hyper-observant of their world.
There's a real controversy as to how much of this is a heightened
awareness of normal phenomena"
 She suggests that visual snow is probably nothing more than an
exaggerated type of normal visual function.
 "When we have entopic firing from our retina ... if you look at the
white wall and really focus on it with the right lighting, you can see
it. It's the same thing with afterimages. It's normal to see
afterimages…most of us pay no attention to it…but if you're a really
observant person, you start noticing it, and then you notice it all the
time."

MEDPAGE TODAY. Meeting coverage: Visual snow called ‘real’ not drug related. URL: http://www.medpagetoday.com/MeetingCoverage/AAN/32416
Difficulty reading
Insomnia
Chronic distraction/discomfort
Dietary limitations
Social limitations: movie theater, beach, etc
Constant fear of losing eyesight
Treatment side effects: anticonvulsants especially
Dependent on: sunglasses, earplugs, sound
machines, nightlights
 SUMMARY
Visual snow is a complex neurological disorder with limited data

Patients often report a sudden onset of constant symptoms,

including palinopsia, entopic phenomena, photophobia, and
nyctalopia

Visual snow may occur because of alterations in neurotransmitter

release or synaptic connectivity along the visual pathway

PET brain scans show increased activity in the lingual gyrus

Treatment has been mostly unsuccessful, with contradictory

outcomes in the literature

Increased awareness is needed to uncover more about this

unique disorder
 Schankin CJ, Goadsby PJ. Visual snow--persistent positive visual phenomenon
distinct from migraine aura. Curr Pain Headache Rep. 2015 Jun;19(6):23.
 Schankin CJ, Maniyar FH, Digre KB, Goadsby PJ. 'Visual snow' - a disorder distinct
from persistent migraine aura. Brain. 2014 May;137(Pt 5):1419-28.
 Haas DC. Prolonged migraine aura status. Ann Neurol 1982; 11: 197-199
 Liu GT, Schatz NJ, Galetta SL, Volpe NJ, Skobieranda F, Kosmorsky GS. Persistent
positive visual phenomena in migraine. Neurology. 1995, 45(4):664-8
 Schankin CJ, Maniyar FH, Sprenger T, Chou DE, Eller M, Goadsby PJ. The relation
between migraine, typical migraine aura and "visual snow". Headache. 2014
Jun;54(6):957-66.
 Fulton, James T., Processes in Biological Vision {online} {Corona Del Mar, CA. USA}
Vision Concepts, 2000, URL:http://neuronresearch.net/vision/
 Tegetmeyer H. [Visual Snow Syndrome: Symptoms and Ophthalmological Findings].
Klin Monbl Augenheilkd. 2016 Aug 10. [Epub ahead of print] German.
 Lauschke JL, Plant GT, Fraser CL. Visual snow: A thalamocortical dysrhythmia of the
visual pathway? J Clin Neurosci. 2016 Jun;28:123-7.
 Bessero AC, Plant GT. Should 'visual snow' and persistence of after-images be
recognised as a new visual syndrome? J Neurol Neurosurg Psychiatry. 2014.
Sep;85(9):1057-8.
 Unal-Cevik I, Yildiz FG. Visual Snow in Migraine With Aura: Further Characterization
by Brain Imaging, Electrophysiology, and Treatment--Case Report. Headache. 2015
Nov-Dec;55(10):1436-41.
 MEDPAGE TODAY. Meeting coverage: Visual snow called ‘real’ not drug related.
URL: http://www.medpagetoday.com/MeetingCoverage/AAN/32416