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.

Assessment and treatment of visuospatial neglect using active learning with Gaussian processes regression.

Visuospatial neglect is a disorder characterised by impaired awareness for visual stimuli located in regions of space and frames of reference. It is often associated with stroke. Patients can struggle with all aspects of daily living and community participation. Assessment methods are limited and show several shortcomings, considering they are mainly performed on paper and do not implement the complexity of daily life. Similarly, treatment options are sparse and often show only small improvements. We present an artificial intelligence solution designed to accurately assess a patient's visuospatial neglect in a three-dimensional setting. We implement an active learning method based on Gaussian process regression to reduce the effort it takes a patient to undergo an assessment. Furthermore, we describe how this model can be utilised in patient oriented treatment and how this opens the way to gamification, tele-rehabilitation and personalised healthcare, providing a promising avenue for improving patient engagement and rehabilitation outcomes. To validate our assessment module, we conducted clinical trials involving patients in a real-world setting. We compared the results obtained using our AI-based assessment with the widely used conventional visuospatial neglect tests currently employed in clinical practice. The validation process serves to establish the accuracy and reliability of our model, confirming its potential as a valuable tool for diagnosing and monitoring visuospatial neglect. Our VR application proves to be more sensitive, while intra-rater reliability remains high.

The Association of Personal Neglect with Motor, Activities of Daily Living, and Participation Outcomes after Stroke: A Systematic Review.

Despite its potential clinical impact, the association of personal neglect (PN) with motor, activities of daily living (ADL), and participation outcomes after stroke is not well-understood. This first-ever systematic review on the topic therefore evaluates this association, taking into account suggested subtypes of PN, including body representation neglect, somatosensory neglect, motor neglect, and premotor neglect. A systematic literature search was conducted on February 17, 2023 in PubMed, Web of Science, Scopus, PubPsych, and PsycArticles databases. The study adheres to the guidelines of Preferred Reporting Items for Systematic Reviews and Meta-Analyses, and its protocol was registered on PROSPERO (CRD42020187460). Eleven observational studies were included, gathering 1,400 individuals after stroke (429 showed PN). Results show that individuals with body representation neglect after stroke have significantly decreased movement control and motor strength, lower functional mobility, and ADL independency compared with those without body representation neglect after stroke. Individuals with motor neglect after stroke showed worse motor function and spasticity than to those without motor neglect after stroke. Nonspecified PN (i.e., PN evaluated with an outcome measure that does not allow subcategorization) was related to worse lateropulsion with pushing, longer length of stay and greater odds of being discharged to somewhere other than home. No study evaluated somatosensory and premotor neglect. This review highlights the limited research in this area and emphasizes the need for a more comprehensive PN assessment. However, currently available assessment tools show limited ability to accurately diagnose PN subtypes and future research should prioritize the development of comprehensive diagnostic test batteries.

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.

A parcellation-based connectomic model of hemispatial neglect.

BACKGROUND AND PURPOSE: Hemispatial neglect is characterized by a reduced awareness to stimuli on the contralateral side. Current literature suggesting that damage to the right parietal lobe and attention networks may cause hemispatial neglect is conflicting and can be improved by investigating a connectomic model of the "neglect system" and the anatomical specificity of regions involved in it. METHODS: A meta-analysis of voxel-based morphometry magnetic resonance imaging (MRI) studies of hemispatial neglect was used to identify regions associated with neglect. We applied parcellation schemes to these regions and performed diffusion spectrum imaging (DSI) tractography to determine their connectivity. By overlaying neglect areas and maps of the attention networks, we studied the relationship between them. RESULTS: The meta-analysis generated a list of 13 right hemisphere parcellations. These 13 neglect-related parcellations were predominantly linked by the superior longitudinal fasciculus (SLF) throughout a fronto-parietal-temporal network. We found that the dorsal and ventral attention networks showed partial overlap with the neglect system and included various other higher-order networks. CONCLUSIONS: We provide an anatomically specific connectomic model of the neurobehavioral substrates underlying hemispatial neglect. Our model suggests a fronto-parietal-temporal network linked via the SLF supports the functions impaired in neglect and implicates various higher-order networks which are not limited to the attention networks.

The Neurophysiological Features Associated with Unilateral Spatial Neglect Recovery: A Scoping Review.

The purpose of this scoping review is to provide updated information on the neural basis and neurophysiological features associated with unilateral spatial neglect (USN) recovery. We applied the Preferred Reporting Systems for Systematic Reviews and Meta-Analyses Extension for Scoping Reviews (PRISMA-ScR) framework and identified 16 relevant papers from the databases. Critical appraisal was performed by two independent reviewers using a standardized appraisal instrument developed by the PRISMA-ScR. We identified and categorized investigation methods for the neural basis and neurophysiological features of USN recovery after stroke using magnetic resonance imaging (MRI), functional MRI, and electroencephalography (EEG). This review found two brain-level mechanisms underlying USN recovery at the behavioral level. These include the absence of stroke-related damage to the right ventral attention network during the acute phase and compensatory recruitment of analogous areas of the undamaged opposite hemisphere and prefrontal cortex during visual search tasks in the subacute or later phases. However, the relationship between the neural and neurophysiological findings and improvements in USN-related activities of daily living remains unknown. This review adds to the growing body of evidence regarding the neural mechanisms underlying USN recovery.

Brain Topological Reorganization Associated with Visual Neglect After Stroke.

Background/Purpose: To identify brain hubs that are behaviorally relevant for neglect after stroke as well as to characterize their functional architecture of communication. Methods: Twenty acute right hemisphere damaged patients underwent neuropsychological and resting-state functional magnetic resonance imaging sessions. Spatial neglect was assessed by means of the Center of Cancellation on the Bells Cancellation Test. For each patient, resting-state functional connectivity matrices were derived by adopting a brain parcellation scheme consisting of 153 nodes. For every node, we extracted its betweenness centrality (BC) defined as the portion of all shortest paths in the connectome involving such node. Then, neglect hubs were identified as those regions showing a high correlation between their BC and neglect scores. Results: A first set of neglect hubs was identified in multiple systems including dorsal attention and ventral attention, default mode, and frontoparietal executive-control networks within the damaged hemisphere as well as in the posterior and anterior cingulate cortex. Such cortical regions exhibited a loss of BC and increased (i.e., less efficient) weighted shortest path length (WSPL) related to severe neglect. Conversely, a second group of neglect hubs found in visual and motor networks, in the undamaged hemisphere, exhibited a pathological increase of BC and reduction of WSPL associated with severe neglect. Conclusion: The topological reorganization of the brain in neglect patients might reflect a maladaptive shift in processing spatial information from higher level associative-control systems to lower level visual and sensory-motor processing areas after a right hemisphere lesion.

Spatial neglect in the digital age: Influence of presentation format on patients' test behavior.

OBJECTIVE: Computerized neglect tests could significantly deepen our disorder-specific knowledge by effortlessly providing additional behavioral markers that are hardly or not extractable from existing paper-and-pencil versions. This study investigated how testing format (paper versus digital), and screen size (small, medium, large) affect the Center of cancelation (CoC) in right-hemispheric stroke patients in the Letters and the Bells cancelation task. Our second objective was to determine whether a machine learning approach could reliably classify patients with and without neglect based on their search speed, search distance, and search strategy. METHOD: We compared the CoC measure of right hemisphere stroke patients with neglect in two cancelation tasks across different formats and display sizes. In addition, we evaluated whether three additional parameters of search behavior that became available through digitization are neglect-specific behavioral markers. RESULTS: Patients' CoC was not affected by test format or screen size. Additional search parameters demonstrated lower search speed, increased search distance, and a more strategic search for neglect patients than for control patients without neglect. CONCLUSION: The CoC seems robust to both test digitization and display size adaptations. Machine learning classification based on the additional variables derived from computerized tests succeeded in distinguishing stroke patients with spatial neglect from those without. The investigated additional variables have the potential to aid in neglect diagnosis, in particular when the CoC cannot be validly assessed (e.g., when the test is not performed to completion).

Spatial neglect encompasses impaired verticality representation after right hemisphere stroke.

Spatial neglect after right hemisphere stroke (RHS) was recently found to encompass lateropulsion, a deficit in body orientation with respect to gravity caused by altered brain processing of graviception. By analogy, we hypothesized that spatial neglect after RHS might encompass an altered representation of verticality. We also assumed a strong relation between body neglect and impaired postural vertical, both referring to the body. To tackle these issues, we performed contingency and correlation analyses between two domains of spatial neglect (body, extra-body) and two modalities of verticality perception (postural, visual) in 77 individuals (median age = 67) with a first-ever subacute RHS (1-3 months). All individuals with a transmodal (postural and visual) tilt in verticality perception (n = 26) had spatial neglect, but the reverse was not found. Correlation and multivariate analyses revealed that spatial neglect (and notably body neglect) was associated more with postural than visual vertical tilts. These findings indicate that after RHS, an impaired verticality representation results from a kind of graviceptive neglect, bearing first on somaesthetic graviception and second on vestibular graviception. They also suggest that the human brain uses not only a mosaic of 2D representations but also 3D maps involving a transmodal representation of verticality.

Transcranial direct current stimulation in neglect rehabilitation after stroke: a systematic review.

Hemispatial neglect is one of the most frequent attention disorders after stroke. The presence of neglect is associated with longer hospital stays, extended rehabilitation periods, and poorer functional recovery. Transcranial direct current stimulation (tDCS) is a new technique with promising results in neglect rehabilitation; therefore, the objective of this systematic review, performed following the PRISMA guidelines, is to evaluate the effectiveness of tDCS on neglect recovery after stroke. The search was done in MEDLINE (PubMed), Web of Science, Scopus, Cochrane Library, and BioMed Central databases. A total of 311 articles were found; only 11 met the inclusion criteria, including 152 post-stroke patients in total. Methodological quality and risk of bias were assessed for all the studies, and methodological characteristics of the studies, sample sizes, methods, main results, and other relevant data were extracted. tDCS intervention ranged from one to twenty sessions distributed in 1 day to 4 weeks, with intensity ranged from 1 to 2 mA. We found moderate evidence for the efficacy of tDCS in the rehabilitation of hemispatial neglect after a stroke, being more effective in combination with other interventions. Nonetheless, the limited number of studies and some studies' design characteristics makes it risky to draw categorical conclusions. Since scientific evidence is still scarce, further research is needed to determine the advantage of this treatment in acute, sub-acute and chronic stroke patients. Future studies should include larger samples, longer follow-ups, and broader neurophysiological assessments, with the final aim of establishing the appropriate use of tDCS as an adjuvant intervention in neurorehabilitation settings.

A CASE OF MIGRAINE WITH SYMPTOMS OF PERCEPTION DISORDERS INCLUDING A FAMILY HISTORY.

Alice in Wonderland Syndrome is characterized by distortions in perception, especially with signs of micropsia or macropsia, peropia, teleopsia.The etiology of this syndrome includes migraine attacks, epilepsy, infections or substance abuse.In this case study, a 15-year-old adolescent girl was admitted to our child and adolescent psychiatry clinic with complaints of seeing objects and people as larger or smaller than they are and/or perceiving objects closer or farther than they are, before her migraine attacks.It was also stated that his father had similar complaints during his adolescence..In this case report, we present a case of migraine with aura.

Disruptions of the Human Connectome Associated With Hemispatial Neglect.

BACKGROUND AND OBJECTIVES: Hemispatial neglect is a heterogeneous and complex disorder that can be classified by frame of reference for "left" vs "right," including viewer-centered neglect (VCN, affecting the contralesional side of the view), stimulus-centered neglect (SCN, affecting the contralesional side of the stimulus, irrespective of its location with respect to the viewer), or both. We investigated the effect of acute stroke lesions on the connectivity of neural networks that underlie VCN or SCN. METHODS: A total of 174 patients within 48 hours of acute right hemispheric infarct underwent a detailed hemispatial neglect assessment that included oral reading, scene copy, line cancellation, gap detection, horizontal line bisection tests, and MRI. Each patient's connectivity map was generated. We performed a linear association analysis between network connectivity strength and continuous measures of neglect to identify lesion-induced disconnections associated with the presence or severity of VCN and SCN. Results were corrected for multiple comparisons. RESULTS: About 42% of the participants with right hemisphere stroke had at least one type of neglect. The presence of any type of neglect was associated with lesions to tracts connecting the right inferior parietal cortex, orbitofrontal cortex, and right thalamus to other right-hemispheric structures. VCN only was strongly associated with tracts connecting the right putamen to other brain regions and tracts connecting right frontal regions with other brain regions. The presence of both types of neglect was most strongly associated with tracts connecting the right inferior and superior parietal cortex to other brain regions and those connecting left or right mesial temporal cortex to other brain regions. DISCUSSION: Our study provides new evidence for the specific white matter tracts where disruption can cause hemispatial neglect in a relatively large number of participants and homogeneous time after onset. We obtained MRI and behavioral testing acutely, before the opportunity for rehabilitation or substantial recovery. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that damage to specific white matter tracts identified on MRI are associated with the presence of neglect following right hemispheric stroke.

A novel computerized assessment of manual spatial exploration in unilateral spatial neglect.

Unilateral spatial neglect is a neuropsychological syndrome commonly observed after stroke and defined by the inability to attend or respond to contralesional stimuli. Typically, symptoms are assessed using clinical tests that rely upon visual/perceptual abilities. However, neglect may affect high-level representations controlling attention in other modalities as well. Here we developed a novel manual exploration test using a touch screen computer to quantify spatial search behaviour without visual input. Twelve chronic stroke patients with left neglect and 27 patients without neglect (based on clinical tests) completed our task. Four of the 12 "neglect" patients exhibited clear signs of neglect on our task as compared to "non-neglect" patients and healthy controls, and six other patients (from both groups) also demonstrated signs of neglect compared to healthy controls only. While some patients made asymmetrical responses on only one task, generally, patients with the strongest neglect performed poorly on multiple tasks. This suggests that representations associated with different modalities may be affected separately, but that severe forms of neglect are more likely related to damage in a common underlying representation. Our manual exploration task is easy to administer and can be added to standard neglect screenings to better measure symptom severity.

Aligning formal and functional assessments of Visuospatial Neglect: A mixed-methods study.

ABSTRACTThe occurrence of visuospatial neglect acts as a key predictor of recovery outcome following stroke. However, the specific behavioural profiles associated with various neglect subtypes are not well understood. This study aims to identify real-world functional impairments associated with neglect, to determine whether functional impairment profiles differ across patients with egocentric and allocentric neglect, and to investigate how neglect severity predicts functional impairments.Notes from 290 stroke patients' occupational therapy functional assessments were qualitatively and quantitatively analysed in the context of neglect type and severity as reported by the OCS Cancellation Task. Overall, neglect patients had more references to having difficulty initiating tasks, finding items, exhibiting spatial inattention, and having difficulty using both arms than patients without neglect. The proportion of theme references did not differ significantly across patients with egocentric and allocentric neglect. The quantitative severity of egocentric neglect was acted as a significant predictor of reference occurrence over and above stroke severity within difficulty finding items, spatial inattention, body inattention, and upper limb use.This study expands on previous findings by identifying real-world functional impairments differentiating patients with and without neglect. This data provides novel insight into the impact of neglect on functional abilities.

Migraine Features in Patients with Persistent Postural-Perceptual Dizziness.

OBJECTIVES: To evaluate the presence of migraine features in patients with persistent postural-perceptual dizziness (PPPD). METHODS: In a retrospective survey study, consecutive patients presenting to a tertiary care neurotology clinic during an 18-month period were given questionnaires about headache and dizziness symptoms. The survey responses plus history and examination of the patient were used to diagnose patients with PPPD. The prevalence of migraine headache, vestibular migraine (VM), and migraine characteristics was evaluated. RESULTS: In total, 36 subjects with PPPD were included in the study. The mean age of the subjects was 56 ± 16 years with a female (72%) predominance. A total of 19 (53%) patients met the International Classification of Headache Disorders criteria for migraine headache, and 6 of those (17%) met the criteria for definite VM. Of the patients who did not meet full migraine headache criteria, 6 (17%) patients met 4 of 5 criteria, and 5 (14%) patients met 3 of 5 criteria. There was no significant difference between PPPD patients who fulfilled full migraine headache criteria and those who did not in sensitivity to light, sound, smells, weather changes, feelings of mental fog/confusion, and sinus pain/facial pressure. CONCLUSIONS: This study demonstrates that a majority of patients with PPPD fulfill the criteria for migraine headache. A large proportion of PPPD patients who do not meet the full criteria for migraine headache still meet a majority of the migraine headache criteria. This suggests an association between the 2 conditions. PPPD may be a part of the spectrum of otologic migraine, where migraine manifests as otologic symptoms.

Application of digital practice to improve head movement, visual perception and activities of daily living for subacute stroke patients with unilateral spatial neglect: Preliminary results of a single-blinded, randomized controlled trial.

BACKGROUND: Virtual reality (VR) based digital practice is an attractive way to provide a patient engagement, motivation and adaptable environment for stroke rehabilitation. However, clinical evidence of efficacy with VR-based digital practice is very limited. In this study, we investigated the effects of VR-based digital practice program on unilateral spatial neglect (USN) rehabilitation in patients with subacute stroke. METHODS: Twenty-four subacute stroke patients with USN were enrolled and randomly assigned to digital practice group (n = 12) and control group (n = 12). Patients in digital practice group received training programs with VR-based applications with leap motion environment. Control group received conventional USN specific training programs. All patients were underwent 4 week practice program (3 sessions/week, a half-hour/session). We analyzed training effects before and after training by assessing the line bisection test, Catherine Bergego Scale, modified Barthel index, Motor-Free Visual Perception Test Vertical Version (MVPT-V), and horizontal head movements (rotation degree and velocity during the VR-based applications), and compared the results between the two groups. RESULTS: Compared to control group, digital practice group showed significantly greater improvements in the line bisection test (P = .020), and visual perceptual tasks (MVPT-V, responded more on left visual task, P = .024; correctly respond more on both left and right visual tasks, P = .024 and P = .014, respectively; and faster response time, P = .014). Additionally, horizontal head movement of rotation degree and velocity during the VR based practice in the digital practice group were significantly increased more than control group (P = .007 and P = .001, respectively). CONCLUSIONS: VR-based digital practice program might be an affordable approach for visual perception and head movement recovery for subacute stroke patients with USN.

Fatal consequences of decreased sensitivity to pain and temperature in a frontotemporal dementia patient.

Frontotemporal dementia (FTD) is a heterogeneous syndrome characterized by the progressive damage of frontal and temporal brain regions. These networks largely overlap with those involved in pain and temperature processing. Although the impaired perception of pain and temperature has been previously described to be relatively common in patients with FTD, these symptoms are often not consistently assessed by Neurologists. We present the case of a patient with a probable behavioral variant FTD who died due to scalding with hot water in the shower. Impairments in the perception of pain and temperature might have played a fundamental role in this accident.

Occipital cortex and cerebellum gray matter changes in visual snow syndrome.

OBJECTIVE: To determine whether regional gray and white matter differences characterize the brain of patients with visual snow syndrome, a newly defined neurologic condition, we used a voxel-based morphometry approach. METHODS: In order to investigate whole brain morphology directly, we performed an MRI study on patients with visual snow syndrome (n = 24) and on age- and sex-matched healthy volunteers (n = 24). Voxel-based morphometry was used to determine volumetric differences in patients with visual snow. We further analyzed cerebellar anatomy directly using the high-resolution spatially unbiased atlas template of the cerebellum. RESULTS: Compared to healthy controls, patients with visual snow syndrome had increased gray matter volume in the left primary and secondary visual cortices, the left visual motion area V5, and the left cerebellar crus I/lobule VI area. These anatomical alterations could not be explained by clinical features of the condition. CONCLUSION: Patients with visual snow syndrome have subtle, significant neuroanatomical differences in key visual and lateral cerebellar areas, which may in part explain the pathophysiologic basis of the disorder.

Ocular motor measures of visual processing changes in visual snow syndrome.

OBJECTIVE: To determine whether changes to cortical processing of visual information can be evaluated objectively using 3 simple ocular motor tasks to measure performance in patients with visual snow syndrome (VSS). METHODS: Sixty-four patients with VSS (32 with migraine and 32 with no migraine) and 23 controls participated. Three ocular motor tasks were included: prosaccade (PS), antisaccade (AS), and interleaved AS-PS tasks. All these tasks have been used extensively in both neurologically healthy and diseased states. RESULTS: We demonstrated that, compared to controls, the VSS group generated significantly shortened PS latencies (p = 0.029) and an increased rate of AS errors (p = 0.001), irrespective of the demands placed on visual processing (i.e., task context). Switch costs, a feature of the AS-PS task, were comparable across groups, and a significant correlation was found between shortened PS latencies and increased AS error rates for patients with VSS (r = 0.404). CONCLUSION: We identified objective and quantifiable measures of visual processing changes in patients with VSS. The absence of any additional switch cost on the AS-PS task in VSS suggests that the PS latency and AS error differences are attributable to a speeded PS response rather than to impaired executive processes more commonly implicated in poorer AS performance. We propose that this combination of latency and error deficits, in conjunction with intact switching performance, will provide a VS behavioral signature that contributes to our understanding of VSS and may assist in determining the efficacy of therapeutic interventions.

Clinical and functional differences between right and left stroke with and without contralateral spatial neglect.

OBJECTIVE: To examine the clinical and functional characteristics associated with contralateral spatial neglect in right compared with left subacute stroke, and to investigate the correlations between neglect severity and stroke severity, functional ability and outcome. METHODS: Cross-sectional study comparing neurological impairment and disability. The same data-set was used in part 2 of this study. RESULTS: Contralateral neglect was present in 79.7% of right stroke and 68.3% of left stroke rehabilitants, and was, on average, equally mild. Left stroke rehabilitants with neglect had higher stroke severity, cognitive and total disability and dependence level and more impaired sphincter control than right stroke rehabilitants with neglect, while the occurrence of depression, motor and sensory impairment was similar. Rehabilitants with neglect, irrespective of stroke side, had higher stroke severity, cognitive, motor and total disability and dependence level than rehabilitants without neglect. CONCLUSION: In left and right stroke rehabilitants with equally mild neglect, those with left stroke had higher stroke severity, cognitive and total disability and dependence level. Neglect severity correlated with right or left stroke severity and functional ability, moderate to severe neglect correlated significantly more with functioning. Neglect severity was independently associated with functional outcome in right stroke.