Visual Snow Syndrome Prevalence Among Young Adults in the Russian Federation: a Research Perspective.

BACKGROUND: Visual snow syndrome (VSS) is a poorly understood neurological condition of unclear etiology, characterized by visual and non-visual symptoms that reduce quality of life. The objective of our study was to estimate the prevalence of young adults with visual snow in Russia. MATERIALS AND METHODS: We conducted an online survey among 1,177 respondents over the age of 18 residing in Russia. The questionnaire was based on MIDAS, HIT-6, ICHD-3, GAD-7, CES-D. RESULTS: A total of 1085 individuals, divided into three groups: 48 participants with Visual Snow Syndrome (VSS), 36 participants with visual snow symptoms (VS), and 1001 participants without visual snow (control group). Tinnitus (p<0.001) and paresthesia (p<0.001) were more common in participants with VSS compared to the control group. VSS group also reported mood disorders more frequently than those in the other groups (29.2% VSS, 13.9% VS, 7.0% control, p<0.001). Additionally, VSS group exhibited more elevated anxiety levels on the GAD-7 scale compared to the other groups (p=0.005), suggesting a weak association between anxiety and VSS. CONCLUSIONS: Diagnosed migraine, tinnitus, concentration problems, paresthesia, and verified psychiatric conditions were significantly more prevalent in the VSS group in our study. Tinnitus was significantly more frequent in the VS group. Diagnosed conditions across all ICD-10 classes were more frequently identified in the VSS group, with the strongest associations (moderate) found with ICD-10 codes: F80-F89 and F60-F69. Additionally ICD-10 codes F30-F39 were more frequently found in the VS group compared to the control group. Our study revealed that nearly all individuals with VSS in our sample (89.6%) had experienced symptoms for as long as they can remember. The prevalence of VS symptoms in Russia is 7.7% (6.2-9.3%) and VSS is 4.4% (3.2-5.7%).

Prevalence and characteristics of visual snow syndrome in a cohort of young Italian adults.

BACKGROUND: Visual snow (VS) and visual snow syndrome (VSS) are becoming increasingly recognized. However, their prevalence worldwide is unknown. This study aimed to investigate lifetime prevalence and describe the clinical characteristics of VS and VSS in a representative population sample from Italy. METHODS: This cross-sectional study was conducted among students attending different faculties in three universities in the central and southern regions of Italy. Eligible participants completed a self-administered questionnaire. In patients fulfilling possible criteria for VS/VSS, the diagnosis was validated by an on-site visit conducted by experienced neurologists and neuro-ophthalmologists that included optical coherence tomography angiography (OCTA). RESULTS: A total of 750 participants completed the study. Seven (0.9%) reported symptoms compatible with VS (mean age 24.8 ± 3.85 years). Among the seven patients, five (0.7%) also met the phenomenological and temporal criteria for VSS. Neuroimaging and ophthalmological examinations showed normal results upon review or during the on-site visit including OCTA. For the five patients with full VSS, the other visual symptoms reported were enhanced entoptic phenomenon (n = 5), photophobia (n = 5), palinopsia (n = 1), and nyctalopia (n = 4). In four of the seven patients (57%) reporting VS symptoms, there was a concomitant diagnosis of migraine with aura, and in one (14%) migraine without aura. All patients (n = 7) reported tinnitus. Six of the seven (85.7%) patients with VS/VSS had never used specific treatments for the condition. None of the seven patients had received a previous diagnosis of VS/VSS. CONCLUSIONS: The prevalence in Italy of VSS is around 1%. However, there is a limited tendency for affected individuals to seek medical attention, leading to a low rate of diagnosis and treatment.

The Pressure of Headache at the United Kingdom CSF Disorders Day 2023.

Headache in intracranial disorders drives significant disability in our patients. Management principles for secondary headaches require a multidisciplinary approach with expertise from the neurological, neuro-ophthalmological, neuroradiological, and neurosurgical specialities. The Pressure of Headache was the theme of the annual United Kingdom CSF Disorders Day and saw speakers and panellists from Australia, Europe, and the United States. Among the topics presented were the newest concepts underpinning idiopathic intracranial hypertension management and spontaneous intracranial hypotension. Advances in our knowledge of cerebrospinal fluid secretion and the visual snow syndrome debate were particular highlights. This review provides a summary of the research on headache disorders that overlap the neuro-ophthalmological axis of care for patients.

Visual Snow Syndrome in Patient with Migraine: Case Report and Literature Review.

Visual snow syndrome (VSS) is a rarely diagnosed neurological phenomenon. It is a visual disorder characterised by the presence of numerous white, black, or translucent dots in the visual field, resembling the 'snow' of an analogue TV set experiencing reception interference. According to The International Classification of Headache Disorders, 3rd edition, visual snow is defined as a pattern of continuous small dots across the visual field lasting >3 months and accompanied by at least two of the following four additional symptoms: palinopsia, increased entoptic phenomena, photophobia, and nyctalopia. These complaints are not consistent with a typical migraine with visual aura and cannot be better explained by another disorder. The authors present the case of a 39-year-old woman who was diagnosed with VSS. The symptoms appeared after a migraine attack and had not alleviated. The patient reported a sensation of constant 'TV screen snow'. A neurological examination found no signs of focal damage to the nervous system. The results of the ophthalmological examination, MRI of the brain with contrast, MRI of the eye sockets, and EEG were normal. VSS is a phenomenon that is still not fully understood, different from migraine aura and associated with a number of additional symptoms. VSS is very difficult to treat. In this case, a lot of drugs were used without improvement. Further research must be conducted to determine the best treatment options for these patients.

Alterations of the alpha rhythm in visual snow syndrome: a case-control study.

BACKGROUND: Visual snow syndrome is a disorder characterized by the combination of typical perceptual disturbances. The clinical picture suggests an impairment of visual filtering mechanisms and might involve primary and secondary visual brain areas, as well as higher-order attentional networks. On the level of cortical oscillations, the alpha rhythm is a prominent EEG pattern that is involved in the prioritisation of visual information. It can be regarded as a correlate of inhibitory modulation within the visual network. METHODS: Twenty-one patients with visual snow syndrome were compared to 21 controls matched for age, sex, and migraine. We analysed the resting-state alpha rhythm by identifying the individual alpha peak frequency using a Fast Fourier Transform and then calculating the power spectral density around the individual alpha peak (+/- 1 Hz). We anticipated a reduced power spectral density in the alpha band over the primary visual cortex in participants with visual snow syndrome. RESULTS: There were no significant differences in the power spectral density in the alpha band over the occipital electrodes (O1 and O2), leading to the rejection of our primary hypothesis. However, the power spectral density in the alpha band was significantly reduced over temporal and parietal electrodes. There was also a trend towards increased individual alpha peak frequency in the subgroup of participants without comorbid migraine. CONCLUSIONS: Our main finding was a decreased power spectral density in the alpha band over parietal and temporal brain regions corresponding to areas of the secondary visual cortex. These findings complement previous functional and structural imaging data at a electrophysiological level. They underscore the involvement of higher-order visual brain areas, and potentially reflect a disturbance in inhibitory top-down modulation. The alpha rhythm alterations might represent a novel target for specific neuromodulation. TRIAL REGISTRATION: we preregistered the study before preprocessing and data analysis on the platform osf.org (DOI: https://doi.org/10.17605/OSF.IO/XPQHF , date of registration: November 19th 2022).

Muscae volitantes: A 19th-century precursor of visual snow syndrome?

PURPOSE: Visual snow syndrome comprises a whole-field static-like visual disturbance, with increased awareness of entopic phenomena, an inability to suppress the 'just seen' and photophobia. Visual snow syndrome is often associated with other problems such as headache, tinnitus, and anxiety. The earliest reported case of a patient experiencing symptoms consistent with visual snow syndrome dates only to 1995. This paper seeks to find patterns of experience in the medical literature of the past that are reminiscent of visual snow syndrome, to challenge the view that it is in any sense a novel disorder. Descriptions of subjective visual sensations such as experienced by patients suffering from visual snow syndrome were sought in treatises, textbooks and other literature generated by leading figures in 19th-century ophthalmology, physiology and physics. CONCLUSION: While retrospective diagnosis of modern illness categories in historical medical literature is an enterprise fraught with pitfalls, it is nonetheless possible to see patterns of experience in the 19th-century medical literature that are strongly reminiscent of visual snow syndrome.

Visual snow syndrome in patients with migraine: widening the clinical spectrum of the syndrome with permanent and episodic manifestations.

PURPOSE: Migraine is the comorbidity most frequently associated with visual snow syndrome (VSS), but the prevalence of VSS in patients with migraine (PWM) has not been studied. Our objective was to evaluate the frequency of VSS in PWM and to analyze if symptoms of VSS happened in a permanent or episodic manner (eVSS) in this population. METHODS: We conducted a multicenter observational cross-sectional study. PWM was recruited from headache units, and a survey about the presence of visual snow symptoms was administered. The frequency and characteristics of patients that met current VSS criteria were analyzed. Demographic and clinical features of patients with VSS, eVSS, and PWM with no visual snow were compared. RESULTS: A total of 217 PWM were included. Seventeen patients (7.8%) met the VSS criteria. VSS patients had visual aura more frequently (58.8% vs. 31%; p = 0.019) and a higher MIDAS score (96.6 vs. 47.7; p = 0.014). Fifty-eight PWM (26.7%) showed visual snow and associated features in an episodic way and were classified as eVSS. Patients with VSS showed a tendency towards a higher frequency of visual symptoms than patients with eVSS (p > 0.05). No statistically significant differences in sociodemographic characteristics and comorbid conditions were found between VSS and eVSS. CONCLUSION: The prevalence of VSS in PWM may be higher than that described for the general population. Some PWM may present similar visual symptoms to patients with VSS but in an episodic manner. Our study reinforces the observation that the clinical spectrum of visual snow is likely to be broader than previously described.

Persistent aura, visual snow, and other visual symptoms.

In neurology practice, it is common to encounter a variety of visual complaints. Historically, in the absence of known ocular pathology, epilepsy, or insult to the central nervous system, positive symptoms were assumed to be migrainous in origin. This assumption was sometimes made even in the absence of a history of migraine. In the past decade, there has been considerable effort to better delineate and study nonmigrainous visual phenomena, with the most extensive focus on a newly defined syndrome, visual snow syndrome (VSS). The heightened awareness of visual snow as a symptom and syndrome has greatly enhanced the understanding of this visual phenomenon; however, in the last few years, there has been an almost pendulous swing in clinic, with patients now being given the diagnosis of VSS for any dots or flickering they may have in their vision. To avoid clinical misdiagnosis, it is critical that we expand our understanding not just of VSS but also of underlying pathologies that may present similarly. This chapter will review classical migraine aura, persistent migraine aura, visual snow and a number of positive and negative visual complaints that are on the differential when seeing patients with suspected aura or visual snow. This is followed by an in-depth discussion on the current understanding of the presenting symptoms, pathophysiology, evaluation and management of VSS. We also outline secondary causes of visual snow.

Visual snow syndrome and its relationship with migraine.

INTRODUCTION: Visual snow syndrome (VSS) is a central nervous system disorder that consists of the constant perception of small black and white dots throughout the entire visual field. DEVELOPMENT: VSS can present from infancy to old age, with greater prevalence in the young population, and shows no difference between sexes. The diagnostic criteria include the presence of visual snow and such other visual phenomena as palinopsia, photophobia, nyctalopia, and other persistent visual phenomena. The pathophysiology of VSS is unknown, but hyperexcitability of the visual cortex and a dysfunction in higher-order visual processing are postulated as potential mechanisms. The prevalence of migraine among patients with VSS is high, compared to the general population, and symptoms are more severe in patients presenting both conditions. No effective treatment is available, but the drug with the best results is lamotrigine, which is recommended only in selected cases with severe functional limitation. CONCLUSIONS: VSS is a little-known and underdiagnosed entity, but the increasing number of studies in recent years has made it possible to establish diagnostic criteria and begin studying its pathophysiology. This entity is closely related to migraine, with overlapping symptoms and probably shared pathophysiological mechanisms.

Visual Snow: Updates and Narrative Review.

PURPOSE OF REVIEW: Visual snow (VS) involves visualization of innumerable dots throughout the visual field, sometimes resembling "TV static." Patients who experience this symptom may also have additional visual symptoms (e.g., photophobia, palinopsia, floaters, and nyctalopia) with a pattern now defined as visual snow syndrome (VSS). This manuscript describes both VS and VSS in detail and provides an updated review on the clinical features, pathophysiology, and optimal management strategies for these symptoms. RECENT FINDINGS: VS/VSS may be primary or secondary to a variety of etiologies, including ophthalmologic or brain disorders, systemic disease, and medication/hallucinogen exposure. Evaluation involves ruling out secondary causes and mimics of VS. Increasing evidence suggests that VSS is a widespread process extending beyond the visual system. Pathophysiology may involve cortical hyperexcitability or dysfunctional connectivity of thalamocortical or attention/salience networks. VSS is typically a benign, non-progressive syndrome and can be managed with non-medicine strategies. Though no medication provides complete relief, some may provide partial improvement in severity of VS.

Clinical reading-related oculomotor assessment in visual snow syndrome.

PURPOSE: Visual snow syndrome (VSS) is a complex neurological condition presenting with an array of sensory, motor, and perceptual dysfunctions and related visual and non-visual symptoms. Recent laboratory studies have found subtle, basic, saccadic-based abnormalities in this population. The objective of the present investigation was to determine if saccadic-related problems could be confirmed and extended using three common clinical reading-related eye movement tests having well-developed protocols and normative databases. METHODS: This was a retrospective analysis of 32 patients (ages 16-56 years) diagnosed with VSS in the first author's optometric practice. There was a battery of three reading-related tests: the Visagraph Reading Eye Movement Test, the Developmental Eye Movement (DEM) Test, and the RightEye Dynamic Vision Assessment Test, all performed using their standard documented protocols and large normative databases. RESULTS: A high frequency of oculomotor deficits was found with all three tests. The greatest percentage was revealed with the Visagraph (56%) and the least with the RightEye (23%). A total of 77% of patients failed at least one of the three tests. CONCLUSION: The present findings confirm and extend earlier investigations revealing a high frequency of saccadic-based oculomotor problems in the VSS population, now including reading-related tasks. This is consistent with the more general oculomotor/motor problems found in these individuals.

Visual Snow: A Review on Pathophysiology and Treatment.

OBJECTIVE: Visual snow (VS) is a rare clinical entity in neuro-ophthalmology. It is described as the presence of flickering dots affecting the whole visual field, often compared by patients to snow or pixelated television static. Importantly, it can be an alarming symptom for many patients, lowering their quality of life. Our purpose is to increase awareness of this disease, because many healthcare professionals have difficulty identifying symptoms as the nature of the condition is subjective. In this review, we aimed to describe the updates in the etiology and treatment of visual snow. We searched for articles in English, presenting original data and published after December 2019. Different studies show inconsistent data. Neuroimaging studies found, among other things, hypermetabolism of the lingual gyrus, increased gray matter in different brain areas, and altered connectivity in visual pathways. However, these findings were not present in all patients. According to the literature, among the most effective drugs is lamotrigine. Unfortunately, it also carries a risk of worsening the symptoms. It is crucial to remember that VS can be worsened or induced by alcohol, recreational drugs, and certain medication. In terms of treatment, nonpharmacological approaches such as color filters and repetitive Transcranial Magnetic Stimulation were also used. INTERPRETATION: Further studies are needed to understand the nature of VS fully. Even though the pathophysiology and effective treatment of the condition remains unknown, expanding the knowledge about visual snow can impact the comfort of patients.

Visual Disturbances Spectrum in Pediatric Migraine.

Migraine is a complex neurological disorder with partially unknown pathophysiological mechanisms. The prevalence in childhood ranges from 7.7% to 17.8%, thus representing the most frequent primary headache. In half of the cases, migraine is accompanied or preceded by various neurological disturbances, among which the visual aura is the best known. In literature, other conditions, such as Alice in Wonderland Syndrome and Visual Snow syndrome, are characterized by visual manifestations and are often associated with migraine. The aim of this narrative review is to describe the spectrum of visual disturbances in pediatric migraine and their pathophysiological mechanisms.

[Temporary and persistent visual phenomena in neurology]. / Passagere und anhaltende visuelle Phänomene in der Neurologie.

Temporary and persistent visual phenomena are a frequent reason for a neurological presentation. The diagnosis can usually be made with the help of a structured anamnesis with determination of the time of onset, the course and symptoms as well as the monocular vs. binocular manifestation. The visual aura in migraine is certainly the most frequent entity to be differentiated. In particular, persistent visual phenomena such as visual snow syndrome, hallucinogen persisting perception disorder and the Charles Bonnet syndrome (CBS) seem to be underdiagnosed in clinical practice for various reasons and are probably not that rare. Instrumental diagnostics are mostly used for exclusion diagnostics and are a component of a complete patient education, but can be indicative for certain questions (CBS, epilepsy). This article presents the most frequent visual phenomena from the neurological practice and their differential diagnoses, guided by a case history.

Symptomatic visual snow in acute ischemic stroke: A case series.

Visual snow is the main symptom of visual snow syndrome, a disorder of predominantly visual disturbances initially described in patients without abnormalities on ancillary investigations. We present a case series of patients with visual snow in the setting of acute ischemic stroke. The first and second patient reported previous episodic visual snow with migraine attacks. The third patient experienced visual snow for the first time during the ischemic stroke. In the first patient, the ischemic stroke affected the right and left precuneus and the right lingual gyrus. In the second patient, the ischemic stroke was located in the left lingual gyrus, parts of the left fusiform and parahippocampal gyrus, left dorso-lateral thalamus, and left cerebellar hemisphere. In the third patient, occipital pole, trunk of the corpus callosum on the right, right paramedian pons, right cerebellar hemisphere, and vermis were affected. Our case series indicates that the symptom visual snow can be caused by vascular lesions in areas of visual processing. Because patients did not meet criteria for visual snow syndrome, dysfunction in the affected areas might only explain part of the complex pathophysiology of visual snow syndrome.

Differential Diagnosis of Visual Phenomena Associated with Migraine: Spotlight on Aura and Visual Snow Syndrome.

Migraine is a severe and common primary headache disorder, characterized by pain as well as a plethora of non-painful symptoms. Among these, visual phenomena have long been known to be associated with migraine, to the point where they can constitute a hallmark of the disease itself. In this review we focus on two key visual disorders that are directly or indirectly connected to migraine: visual aura and visual snow syndrome (VSS). Visual aura is characterized by the transient presence of positive and negative visual symptoms, before, during or outside of a migraine attack. VSS is a novel stand-alone phenomenon which has been shown to be comorbid with migraine. We discuss key clinical features of the two disorders, including pathophysiological mechanisms, their differential diagnoses and best treatment practices. Our aim is to provide an aid for clinicians and researchers in recognizing these common visual phenomena, which can even appear simultaneously in patients with an underlying migraine biology.

Perceived severity of Visual Snow Syndrome is associated with visual allodynia.

OBJECTIVE: To investigate whether sensory sensitivity is associated with the perceived severity of Visual Snow Syndrome (VSS) symptoms. BACKGROUND: Visual Snow (VS) is a perceptual anomaly which manifests as flashing pinpricks of light throughout the visual field. VSS has an estimated population prevalence of 2.2% and is thought to be at least moderately debilitating for all who experience it. However, some people who meet the criteria for VSS have no awareness of it. This may be because they have lower sensory sensitivity, allowing them to ignore their visual phenomena. METHOD: Our study used a cross-sectional design. We recruited two distinct samples of people with VSS: a sample of people with confirmed VSS; and a sample of people who met the criteria for the condition but had no prior knowledge of it (latent VSS). The latter group was recruited and screened for symptoms via an online crowd-sourcing platform. In total, 100 participants with VSS (49 with confirmed VSS, 51 with latent VSS) completed the Visual Snow Handicap Index and three measures of sensory hypersensitivity. RESULTS: The 100 participants (52 female, 47 male, 1 non-binary) had a mean age of 35.1 years (SD = 12.2). Leiden Visual Sensitivity Scale scores were associated with both the perceived severity of VSS, ß = 0.35, p = 0.003, and the number of VSS symptoms endorsed, ß = 0.45, p < 0.001. On average, participants with VSS experienced elevated sensory hypersensitivity across all measures. Furthermore, longer duration of VSS was associated with lower perceived severity, F(1, 98) = 11.37, p = 0.001, R2  = 0.103. CONCLUSIONS: Our results suggest that sensory hypersensitivity may be prevalent in people with VSS and indicate that visual allodynia is associated with increased severity of VSS. Additionally, our findings indicate that inclusion of cases of latent VSS in future research may be important for researchers to develop a more complete understanding of the perceptual experiences of people with VSS.