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Visual snow syndrome (VSS) is an uncommon neurological condition in which the primary symptom is that affected individuals see persistent flickering white, black, transparent, or coloured dots across the whole
visual field The visual field is the "spatial array of visual sensations available to observation in introspectionist psychological experiments". Or simply, visual field can be defined as the entire area that can be seen when an eye is fixed straight at a point ...
. Other common symptoms are palinopsia, enhanced entoptic phenomena,
photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...
, and
headaches Headache is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches. Headaches can occur as a resul ...
. The condition is typically always present and has no known cure, as viable treatments are still under research.
Migraine Migraine (, ) is a common neurological disorder characterized by recurrent headaches. Typically, the associated headache affects one side of the head, is pulsating in nature, may be moderate to severe in intensity, and could last from a few ho ...
and
tinnitus Tinnitus is the perception of sound when no corresponding external sound is present. Nearly everyone experiences a faint "normal tinnitus" in a completely quiet room; but it is of concern only if it is bothersome, interferes with normal hearin ...
are common comorbidities and are both associated with a more severe presentation of the syndrome. The cause of the syndrome is unclear. The underlying mechanism is believed to involve excessive excitability of neurons in the right lingual gyrus and left anterior lobe of cerebellum. Another hypothesis proposes that visual snow syndrome could be a type of thalamocortical dysrhythmia and may involve the thalamic reticular nucleus (TRN). A failure of inhibitory action from the TRN to the thalamus may be the underlying cause for inability to suppress excitatory sensory information. Research has been limited due to issues of case identification and diagnosis, the latter now largely addressed, and the limited size of any studied cohort. Initial functional brain imaging research suggests visual snow is a brain disorder. There is no established treatment for visual snow syndrome. Medications that may be used to treat the condition include
lamotrigine Lamotrigine, sold under the brand name Lamictal among others, is a medication used to treat epilepsy and stabilize mood in bipolar disorder. For epilepsy, this includes focal seizures, tonic-clonic seizures, and seizures in Lennox-Gastaut syn ...
,
acetazolamide Acetazolamide, sold under the trade name Diamox among others, is a medication used to treat glaucoma, epilepsy, altitude sickness, periodic paralysis, idiopathic intracranial hypertension (raised brain pressure of unclear cause), urine alka ...
, or
verapamil Verapamil, sold under various trade names, is a calcium channel blocker medication used for the treatment of high blood pressure, angina (chest pain from not enough blood flow to the heart), and supraventricular tachycardia. It may also be ...
. However, these do not commonly result in benefits and the evidence for their use is very limited.


Signs and symptoms

In addition to visual snow, many of those affected have other types of visual disturbances such as starbursts, increased
afterimages AfterImage is a Filipino rock band formed in 1986, best known for their songs "Habang May Buhay", "Next in Line", and "Mangarap Ka". They disbanded in 1997 and became active again in 2008 after they reunited and released their fourth studio album ...
,
floater Floaters or eye floaters are sometimes visible deposits (e.g., the shadows of tiny structures of protein or other cell debris projected onto the retina) within the eye's vitreous humour ("the vitreous"), which is normally transparent, or between ...
s, trails, and many others. Visual snow likely represents a clinical continuum, with different degrees of severity. The presence of comorbidities such as migraine and tinnitus is associated with a more severe presentation of the visual symptoms.


Diagnosis

Visual snow syndrome is usually diagnosed with the following proposed criteria: * Visual snow: dynamic, continuous, tiny dots in the entire visual field lasting more than three months. ** The dots are usually black/gray on a white background and gray/white on a black background; however, they can also be transparent, white flashing, or colored. * Presence of at least 2 additional visual symptoms of the 4 following categories: ** i. Palinopsia. At least 1 of the following: afterimages or trailing of moving objects. ** ii. Enhanced entoptic phenomena. At least 1 of the following: excessive floaters in both eyes, excessive blue field entoptic phenomenon, self-light of the eye (
phosphene A phosphene is the phenomenon of seeing light without light entering the eye. The word ''phosphene'' comes from the Greek words ''phos'' (light) and ''phainein'' (to show). Phosphenes that are induced by movement or sound may be associated with ...
s), or spontaneous
photopsia Photopsia is the presence of perceived flashes of light in the field of vision. It is most commonly associated with: * posterior vitreous detachment * migraine aura (ocular migraine / retinal migraine) * migraine aura without headache * sc ...
. ** iii.
Photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...
. ** iv. Nyctalopia; impaired night vision. * Symptoms are not consistent with typical migraine aura. * Symptoms are not better explained by another disorder ( ophthalmological, drug abuse). ** Normal ophthalmology tests (best-corrected
visual acuity Visual acuity (VA) commonly refers to the clarity of vision, but technically rates an examinee's ability to recognize small details with precision. Visual acuity is dependent on optical and neural factors, i.e. (1) the sharpness of the retinal ...
,
dilated fundus examination Dilated fundus examination or dilated-pupil fundus examination (DFE) is a diagnostic procedure that employs the use of mydriatic eye drops (such as tropicamide) to dilate or enlarge the pupil in order to obtain a better view of the fundus of ...
, visual field, and electroretinogram); not caused by previous intake of
psychotropic A psychoactive drug, psychopharmaceutical, psychoactive agent or psychotropic drug is a chemical substance, that changes functions of the nervous system, and results in alterations in perception, mood, consciousness, cognition or behavior. Th ...
drugs. Additional and non visual symptoms like
tinnitus Tinnitus is the perception of sound when no corresponding external sound is present. Nearly everyone experiences a faint "normal tinnitus" in a completely quiet room; but it is of concern only if it is bothersome, interferes with normal hearin ...
, ear pressure or brain fog and more might be present. It can also be diagnosed by
PET scan Positron emission tomography (PET) is a functional imaging technique that uses radioactive substances known as radiotracers to visualize and measure changes in metabolic processes, and in other physiological activities including blood flow, r ...
.


Comorbidities

Migraine Migraine (, ) is a common neurological disorder characterized by recurrent headaches. Typically, the associated headache affects one side of the head, is pulsating in nature, may be moderate to severe in intensity, and could last from a few ho ...
and migraine with aura are common comorbidities. However, comorbid migraine worsens some of the additional visual symptoms and
tinnitus Tinnitus is the perception of sound when no corresponding external sound is present. Nearly everyone experiences a faint "normal tinnitus" in a completely quiet room; but it is of concern only if it is bothersome, interferes with normal hearin ...
seen in "visual snow" syndrome. This might bias research studies by patients with migraine being more likely to offer study participation than those without migraine due to having more severe symptoms. In contrast to migraine, comorbidity of typical migraine aura does not appear to worsen symptoms. Psychological side effects of visual snow can include
depersonalization Depersonalization can consist of a detachment within the self, regarding one's mind or body, or being a detached observer of oneself. Subjects feel they have changed and that the world has become vague, dreamlike, less real, lacking in significa ...
,
derealization Derealization is an alteration in the perception of the external world, causing those with the condition to perceive it as unreal, distant, distorted or falsified. Other symptoms include feeling as if one's environment is lacking in spontaneity, ...
, depression,
photophobia Photophobia is a medical symptom of abnormal intolerance to visual perception of light. As a medical symptom photophobia is not a morbid fear or phobia, but an experience of discomfort or pain to the eyes due to light exposure or by presence of ...
and heliophobia in the individual affected. Patients with visual "snow" have normal equivalent input noise levels and contrast sensitivity. In a 2010 study, Raghaven et al. hypothesize that what the patients see as "snow" is eigengrau. This would also explain why many report more visual snow in low light conditions: "The intrinsic dark noise of primate cones is equivalent to ~4000 absorbed photons per second at mean light levels; below this the cone signals are dominated by intrinsic noise".


Causes

The causes are unclear. The underlying mechanism is believed to involve excessive excitability of neurons within the cortex of the
brain The brain is an organ that serves as the center of the nervous system in all vertebrate and most invertebrate animals. It consists of nervous tissue and is typically located in the head ( cephalization), usually near organs for special ...
, specifically the right lingual gyrus and left
cerebellar The cerebellum (Latin for "little brain") is a major feature of the hindbrain of all vertebrates. Although usually smaller than the cerebrum, in some animals such as the mormyrid fishes it may be as large as or even larger. In humans, the cerebe ...
anterior lobe of the brain.Schankin, CJ, Maniyar, FH, Sprenger, T, Chou, DE, Eller, M, Goadsby, PJ, 2014, The Relation Between Migraine, Typical Migraine Aura and "Visual Snow", Headache, Persisting visual snow can feature as a leading addition to a
migraine Migraine (, ) is a common neurological disorder characterized by recurrent headaches. Typically, the associated headache affects one side of the head, is pulsating in nature, may be moderate to severe in intensity, and could last from a few ho ...
complication called persistent aura without infarction, commonly referred to as persistent migraine aura (PMA). In other clinical sub-forms of migraine headache may be absent and the migraine aura may not take the typical form of the zigzagged fortification spectrum (scintillating scotoma), but manifests with a large variety of focal neurological symptoms. Visual snow does not depend on the effect of
psychotropic A psychoactive drug, psychopharmaceutical, psychoactive agent or psychotropic drug is a chemical substance, that changes functions of the nervous system, and results in alterations in perception, mood, consciousness, cognition or behavior. Th ...
substances on the brain. Hallucinogen persisting perception disorder (HPPD), a condition caused by hallucinogenic drug use, is sometimes linked to visual snow, but both the connection of visual snow to HPPD and the cause and prevalence of HPPD is disputed. Most of the evidence for both is generally anecdotal, and subject to
spotlight fallacy In statistics, sampling bias is a bias (statistics), bias in which a sample is collected in such a way that some members of the intended statistical population, population have a lower or higher sampling probability than others. It results in a bia ...
. Another proposed mechanism is a thalamocortical dysrhythmia of the visual pathway, similar to tinnitus, which is a thalamocortical dysrhythmia of the auditory pathway.


Timeline

* In May 2015, visual snow was described as a persisting positive visual phenomenon distinct from migraine aura in a study by Schankin and Goadsby. * In December 2020, a study found local increases in regional cerebral perfusion in patients with visual snow syndrome. * In May 2021, University of Zurich researchers announced a clinical trial on neurofeedback for visual snow syndrome. * In June 2021, University of Colorado researchers started recruiting for a clinical trial on
transcranial magnetic stimulation Transcranial magnetic stimulation (TMS) is a noninvasive form of brain stimulation in which a changing magnetic field is used to induce an electric current at a specific area of the brain through electromagnetic induction. An electric pulse gener ...
for visual snow syndrome. * In September 2021, two studies found
white matter White matter refers to areas of the central nervous system (CNS) that are mainly made up of myelinated axons, also called tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distributi ...
alterations in parts of the visual cortex and outside the visual cortex in patients with visual snow syndrome.


Treatments

It is difficult to resolve visual snow with treatment, but it is possible to reduce symptoms and improve quality of life through treatment, both of the syndrome and its comorbidities. Medications that may be used include
lamotrigine Lamotrigine, sold under the brand name Lamictal among others, is a medication used to treat epilepsy and stabilize mood in bipolar disorder. For epilepsy, this includes focal seizures, tonic-clonic seizures, and seizures in Lennox-Gastaut syn ...
,
acetazolamide Acetazolamide, sold under the trade name Diamox among others, is a medication used to treat glaucoma, epilepsy, altitude sickness, periodic paralysis, idiopathic intracranial hypertension (raised brain pressure of unclear cause), urine alka ...
, or
verapamil Verapamil, sold under various trade names, is a calcium channel blocker medication used for the treatment of high blood pressure, angina (chest pain from not enough blood flow to the heart), and supraventricular tachycardia. It may also be ...
, but these do not always result in benefits. As of 2021, there were two ongoing clinical trials using
transcranial magnetic stimulation Transcranial magnetic stimulation (TMS) is a noninvasive form of brain stimulation in which a changing magnetic field is used to induce an electric current at a specific area of the brain through electromagnetic induction. An electric pulse gener ...
and neurofeedback for visual snow. A recent study in the '' British Journal of Ophthalmology'' has confirmed that common drug treatments are generally ineffective in visual snow syndrome (VSS).
Vitamins A vitamin is an organic molecule (or a set of molecules closely related chemically, i.e. vitamers) that is an essential micronutrient that an organism needs in small quantities for the proper functioning of its metabolism. Essential nutrie ...
and
benzodiazepines Benzodiazepines (BZD, BDZ, BZs), sometimes called "benzos", are a class of depressant drugs whose core chemical structure is the fusion of a benzene ring and a diazepine ring. They are prescribed to treat conditions such as anxiety disorders, ...
, however, were shown to be beneficial in some patients and can be considered safe for this condition. Dr. Victoria Pelak, Professor of Neurology and Ophthalmology in the Department of Neurology at the University of Colorado Anschutz Medical Campus has recently directed, published, and completed enrollment for a TMS study protocol. The study protocol aims to investigate the use of rTMS intervention to improve symptoms and visual dysfunction associated with visual snow (VS); the study protocol also describes the challenges during the
COVID-19 pandemic The COVID-19 pandemic, also known as the coronavirus pandemic, is an ongoing global pandemic of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The novel virus was first identified ...
. In addition, Dr. Pelak described during her practice that she lets patients know that current treatment options are only limited to alleviating symptoms. She recommends that her patients focus on pharmaceutical and non-pharmaceutical treatments to control migraine, headaches, anxiety, and depression. As for light sensitivity complications, Dr. Pelak advises patients to use FL-41 tinted lenses indoors. She also recommends visual occupational therapists who assist patients with color-tinted lenses to alleviate VSS symptoms. Furthermore, Dr. Pelak states that exercising, meditation, and a healthy balanced diet can improve overall daily functioning.


References


External links


Visual Snow Initiative

Visual snow syndrome
at
NIH The National Institutes of Health, commonly referred to as NIH (with each letter pronounced individually), is the primary agency of the United States government responsible for biomedical and public health research. It was founded in the late 1 ...
* http://visualsnowsyndrome.com/ *
Eye On Vision Foundation
{{Visual phenomena Visual disturbances and blindness Hallucinations Ailments of unknown cause Medical controversies Migraine