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Cerebral/cortical visual impairment (CVI) is a leading cause of pediatric visual impairment in nations with developed economies and is increasing in those with developing economies. Because vision is the predominant sense used for learning, delay in diagnosis of CVI can negatively affect education, making early detection and management important. The American Academy of Pediatrics has published the policy statement "Visual System Assessment in Infants, Children, and Young Adults by Pediatricians" and an accompanying clinical report that are based on identifying potential causes of ocular visual impairment in children. Yet, routine vision screening may not accurately identify the brain-based visual impairment in children with CVI. Moreover, children with CVI often have medical complexity with other neurocognitive impairments and serious medical conditions that can make the diagnosis of CVI more difficult. Strategies are necessary for early identification of CVI to promote early diagnosis and referral for vision services that may allow a child with CVI to engage more fully in school, activities of daily living, vocational pursuits, and recreational activities. Knowledge of the characteristics of CVI as well as risk factors for CVI will assist the pediatrician in identifying children with CVI. This clinical report is complementary to previous vision screening policies, allowing both ocular and brain-based visual impairments in children to be identified and addressed. Pediatricians, other primary care physicians, pediatric ophthalmologists, neurologists, and other specialized pediatric eye care clinicians can identify children with CVI and coordinate effective evaluation, diagnosis, and referrals for vision services for these children., (Copyright © 2024 by the American Academy of Pediatrics.)

Background: Cerebral visual impairment (CVI) is the most common form of paediatric visual impairment in developed countries. CVI can arise from a host of genetic or acquired causes, but there has been limited research to date on CVI in the context of genetic disorders., Methods: We carried out a retrospective analysis of genotypic and phenotypic data for participants with CVI within the DECIPHER database and 100 000 Genomes Project (100KGP)., Results: 158 individuals with CVI were identified across both cohorts. Within this group, pathogenic or likely pathogenic sequence variants in 173 genes were identified. 25 of these genes already have known associations with CVI, while the remaining 148 are candidate genes for this phenotype. Gene ontology analysis of the CVI gene sets from both DECIPHER and 100KGP suggests that CVI has a similar degree of genetic heterogeneity to other neurodevelopmental phenotypes, and a strong association with genetic variants converging on ion channels and receptor functions. Individuals with a monogenic disorder and CVI have a higher frequency of epilepsies and severe neurodisability than individuals with a monogenic disorder but not CVI., Conclusion: This study supports the availability of genetic testing for individuals with CVI alongside other neurodevelopmental difficulties. It also supports the availability of ophthalmological screening for individuals with genetic diagnoses linked to CVI. Further studies could elaborate on the links between specific genetic disorders, visual maturation and broader neurodevelopmental characteristics., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ.)

Competing Interests: The authors report no conflicts of interest.

Hepatic encephalopathy describes a spectrum of potentially reversible neuropsychiatric abnormalities seen in a patient with severe liver dysfunction with porto-systemic shunting. Cortical blindness can be a rare presentation of hepatic encephalopathy and can even precede the onset of altered sensorium. We report a case of 57 years female with chronic liver disease who presented with bilateral loss of vision, with no focal neurological deficits. From clinical and laboratory examination, a diagnosis of hepatic encephalopathy with cortical blindness was proposed. Her visual disturbances gradually improved with the treatment of hepatic encephalopathy. Keywords: Cortical blindness; end stage liver disease; hepatic encephalopathy; papilledema.

Purpose: To report a case of typical delayed-onset hypoxic cortical blindness that occurred few days after resuscitation from drowning in a young male., Methods: Neurological and ophthalmological examination were performed including optical coherence tomography (OCT), Goldmann perimetry, pattern electroretinogram (pERG), pattern and flash visual evoked potentials (pVEP and fVEP) and brain magnetic resonance imaging (MRI)., Results: At presentation, at day 12 post-hypoxic incident, best corrected visual acuity (BCVA) was reduced to hand motion OU with an abolished optokinetic nystagmus, a normal fundus and no relative afferent pupillary defect. Macular and peripapillary OCT were normal. Goldmann perimetry revealed bilateral centrocecal scotoma. pERG was normal while pVEPs were undetectable and fVEPs were abnormal with delayed, decreased and disorganized responses, without interhemispheric asymmetry. Brain MRI disclosed a bilateral cortical-subcortical occipital hypersignal with laminar necrosis and thus confirmed the diagnosis of delayed-onset hypoxic cortical blindness. Visual rehabilitation, including visual stimulation in the scotomatous areas, was associated with a dramatic and rapid visual improvement with a BCVA of 20/32 OU, an ability to read after 2 weeks (day 30 post-hypoxic incident), and a reduction in the size of the scotoma., Conclusion: Delayed-onset hypoxic cortical blindness is a rare presentation of cortical blindness that develops few days after a cerebral hypoxic stress. While initial presentation can be catastrophic, visual improvement may be spectacular and enhanced with visual rehabilitation., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Introduction: Cortical visual impairment (CVI) in children is a retro chiasmal visual tract disorder where there is with an impairment in the visual acuity and/or functionality of vision-guided task, including motor planning in the presence of normal ocular findings or minimal ocular morbidity. The study was conducted to assess the knowledge about CVI among ophthalmologists practicing in Nepal., Materials and Methods: This was a cross sectional study. Data collection was done by administering a preformed, validated questionnaire that was sent via email to all the ophthalmologists registered under the Nepal Ophthalmic Society. The email mentioned the aim of the study along with the questionnaire., Results: A total of 146 (37.82%) ophthalmologists responded to the questionnaire. Forty four percent of the participants were general ophthalmologists, 28% were pediatric ophthalmologists and 67% were ophthalmologists from other subspecialty. The median age of participants was 37.6 years. Most of the ophthalmologist had a good knowledge about the cause, common risk factors, clinical risk factors, management and prognosis of CVI. However only 29.5% of participants were aware of the investigation of choice for diagnosing CVI and 31.7% were aware of the leading causes of visual impairment in the developed countries. The study also established that the knowledge score was higher in pediatric ophthalmologists than the general ophthalmologist and ophthalmologists from other specialties., Conclusion: Most of the ophthalmologists had a good knowledge about the cause, common risk factors, clinical features, management and prognosis of CVI. However only a limited number of participants were aware of the investigation of choice for diagnosing CVI and the leading causes of visual impairment in the developed countries. Majority of the participants rarely examined patients with CVI which does not correlate with the high prevalence of perinatal hypoxia, the commonest cause of CVI, in our country., (© NEPjOPH.)

Competing Interests: Competing interests: None declared.

Background: There are no published cases of tonic-clonic seizures and posterior bilateral blindness during pregnancy and Severe Acute Respiratory Syndrome (SARS) Coronavirus (COV) 2 (SARS-COV-2) infection. We do not just face new and unknown manifestations, but also how different patient groups are affected by SARS-COV-2 infection, such as pregnant women. Coronavirus Disease 2019 (COVID-19), preeclampsia, eclampsia and posterior reversible leukoencephalopathy share endothelium damage and similar pathophysiology., Case Presentation: A 35-year-old pregnant woman was admitted for tonic-clonic seizures and SARS-COV-2 infection. She had a normal pregnancy control and no other symptoms before tonic-clonic seizures development. After a Caesarean section (C-section) she developed high blood pressure, and we initiated antihypertensive treatment with labetalol, amlodipine and captopril. Few hours later she developed symptoms of cortical blindness that resolved in 72 h with normal brain computed tomography (CT) angiography., Conclusion: The authors conclude that SARS COV-2 infection could promote brain endothelial damage and facilitate neurological complications during pregnancy.

Purpose: To compare the final diagnosis of the causes of low vision in children attending a tertiary rehabilitation centre for visually impaired children versus referral diagnosis., Methods: Retrospective review of clinical charts of all children referred to the Robert Hollman Foundation, a tertiary centre for visually impaired children, between January 2010 and June 2011. The following clinical data were analysed: entry diagnosis made by the referral ophthalmologist and final diagnosis made at Robert Hollman Foundation based on a complete ophthalmic evaluation., Results: Ninety-two consecutive children (mean age = 2.37 ± 1.98 years, range = 0-9) were included. A referral diagnosis was retrieved in 76 cases (82.6%), including cerebral visual impairment (14.1%), retinopathy of prematurity (14.1%), hereditary retinal diseases (10.9%), nystagmus (8.7%) and other rarer diseases (34.8%). In the remaining 16 children (17.4%), a precise referral diagnosis was unavailable. Final clinical diagnosis made at Robert Hollman Foundation was normal visual function in 8.7%, cerebral visual impairment in 30.4%, retinopathy of prematurity in 10.9%, hereditary retinal disease in 9.8% and other in 40.2%. In 17 cases (18.5%), the diagnosis made at the Robert Hollman Foundation did not confirm the entry diagnosis. Among patients where measurement of visual acuity was possible (84), 66.7% were blind or seriously visual impaired, and the main causes were cerebral visual impairment (32.1%) and retinopathy of prematurity (16.1%)., Conclusion: The most frequent diseases were cerebral visual impairment, retinopathy of prematurity and hereditary retinal diseases. Approximately one-third of referred children had not a correct diagnosis at baseline. The activity of an ophthalmic tertiary centre is essential to offer a precise diagnosis to visually impaired (sometimes with other deficits) children.

We describe an unusual case of a 68-year-old male affected by cerebral amyloid angiopathy and cortical blindness associated with Anton's syndrome. In addition, our patient presented with autotopagnosia, a form of agnosia characterized by loss of body spatial representation. Neuropsychological assessment evidenced cognitive impairment. Magnetic Resonance Imaging showed hemorrhagic foci in the left occipital and right occipito-parietal lobe, paratrigonal white matter, and post-ischemic parenchymal gliosis. The pattern-reversal of visual evoked potentials were indicative bilateral visual pathway of integrity of the. After a neurological damage, patients could show a denial of their own deficit; however, the association between anosognosia and autotopagnosia represents a rare neurological condition. The simultaneous onset of unusual neuropsychological syndromes could be related to involvement of a complex brain network.

Purpose: To document the association of prenatal brain disruption with secondary perinatal distress in children diagnosed as having cortical visual impairment (CVI)., Design: Retrospective case series., Methods: Eight children with severe CVI and clinical history of perinatal events were included. Case histories and neuroimaging studies were reviewed. The main outcome measures were perinatal history, visual and neurologic findings, and magnetic resonance (MR) imaging., Results: In our patient cohort, MR imaging showed signs of cortical dysgenesis leading to congenital brain malformations such as polymicrogyria consistent with a prenatal timing of CNS injury. Although subcortical white matter changes were common, signs of watershed injury to the visual cortex were absent, suggesting that the visual loss was attributable to a prenatal etiology with secondary birth complications., Conclusion: Some children with CVI and a history of perinatal distress have prenatal dysgenesis of the developing brain. Therefore, a clinical history of perinatal hypoxia-ischemia is nonspecific and merits neuroimaging to identify antecedent brain malformations and timing of injury, which can influence patient diagnosis and management., (Copyright © 2019 Elsevier Inc. All rights reserved.)

For several reasons, cerebral visual impairment (CVI) is emerging as a major cause of visual impairment among children in the developing world and we are seeing an increasing number of such children in our clinics. Owing to lack of early training about CVI and it being a habilitation orientated subject, we need to become equipped to optimally help the affected children. In this paper we have explained our pragmatic approach in addressing children who present with low functioning CVI. Initially we explain briefly, how vision is processed in the brain. We then present what should be specifically looked for in these children in regular clinics as a part of their comprehensive ophthalmic examination. We discuss the process of functional vision evaluation that we follow with the help of videos to explain the procedures, examples of how to convey the conclusions to the family, and how to use our findings to develop intervention guidelines for the child. We explain the difference between passive vision stimulation and vision intervention, provide some common interventions that may be applicable to many children and suggest how to infuse interventions in daily routines of children so that they become relevant and meaningful leading to effective learning experiences., Competing Interests: There are no conflicts of interest.

Competing Interests: Competing interests: None declared.

Background: Improvement in vision has been noted in children with cortical visual impairment (CVI), resulting from disparate types of brain injury. The purpose of our study was to determine the risk factors associated with poor recovery of vision in this group of patients., Methods: Case records of children who were born before 2010 with at least 4 follow-up visits for CVI were reviewed for underlying etiologies of CVI, visual acuity (VA), and associated neurological and ophthalmological disorders. VA was assessed in 6 qualitative grades. Changes in VA were recorded as the difference between the grades of VA at presentation and the last follow-up visit. The outcome was calculated as a ratio of actual improvement to potential improvement in grades of qualitative VA. Multiple linear regression determined factors associated with lack of vision improvement in all children and based on etiology., Results: Fifty-three children with CVI were identified. The median age at presentation was 13.6 months (range: 2.9-76.4 months) and the median follow-up was 5.8 years (1.1-16.3 years). CVI resulted from central nervous system (CNS) malformation (9.4%), hypoxic/inflammatory injury (15.1%), seizures (24.5%), and combined causes (51.0%). Vision improvement was noted in 83% of children. Lack of VA improvement was associated with older age at presentation in all children with CVI and within each etiological group except CNS malformation. None of the other investigated variables were associated with poor recovery of VA., Conclusions: Most of the children with CVI showed improvement in vision. Older age at presentation, but not etiology of CVI, was associated with poor improvement in VA.

The cerebral cortex is supplied by vascular microlobules, each comprised of a half dozen penetrating arterioles that surround a central draining venule. The surface arterioles that feed the penetrating arterioles are interconnected via an extensively anastomotic plexus. Embolic occlusion of a small surface arteriole rarely produces a local infarct, because collateral blood flow is available through the vascular reticulum. Collateral flow also protects against infarct after occlusion of a single penetrating arteriole. Cortical infarction requires blockage of a major arterial trunk, with arrest of blood flow to a relatively large vascular territory. For striate cortex, the major vessels compromised by emboli are the inferior calcarine and superior calcarine arteries, as well as the distal branches of the middle cerebral artery. Their vascular territories have a fairly consistent relationship with the retinotopic map. Consequently, occlusion by emboli results in stereotypical visual field defects. The organization of the arterial supply to the occipital lobe provides an anatomical explanation for a phenomenon that has long puzzled neuro-ophthalmologists, namely, that of the myriad potential patterns of cortical visual field loss, only a few are encountered commonly from embolic cortical stroke.

Clinical Case: A 22 year-old woman complained about blurred vision after an episode of recovered cardiorespiratory arrest. She had bilateral low visual acuity («count fingers») and no ophthalmological or visual pathways changes. She also had an apparent lack of awareness of the deficit. The Magnetic Resonance Imaging (MRI) showed ischaemic changes in both occipital lobes. As a result, she was diagnosed with Anton-Babinski syndrome., Discussion: This is a rare disease that should be suspected in strange or poorly congruent visual loss. It is usually due to an ischaemic injury in this region of brain, manifesting itself with low vision not perceived by the patient (visual confabulation). It can simulate a non-organic visual loss or psychiatric disease., (Copyright © 2018 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.)

The childhood condition of visual difficulties caused by brain damage, commonly termed cortical or cerebral visual impairment (CVI), is well established but has no internationally accepted definition. Clarification of its core features is required to advance research and clinical practice. This systematic review aimed to identify the definitions of childhood CVI in the original scientific literature to describe and critically appraise a consensual definition of the condition. MEDLINE, EMBASE, PsychINFO, CINAHL and AMED databases were searched in January 2017. Studies were included if they (1) were published original research, (2) contained a childhood CVI sample, (3) contained a definition of CVI and (4) described their CVI identification/diagnostic method. Thematic analysis identified concepts within definitions and narrative synthesis was conducted. Of 1150 articles, 51 met inclusion criteria. Definitions were subdivided according to detail (descriptive definition, description not reaching definition status and diagnostic/operationalising criteria). Three themes concerning visual deficits, eye health and brain integrity were identified (each containing subthemes) and analysed individually across definitions. The most common themes were ' visual impairment' (n=20), 'retrochiasmatic pathway damage'(n=13) and 'normal/near normal eye health' (n=15). The most consensual definition identified here may not be the best quality for advancing our understanding of CVI. We argue for the alternative definition: CVI is a verifiable visual dysfunction which cannot be attributed to disorders of the anterior visual pathways or any potentially co-occurring ocular impairment. We propose reporting guidelines to permit comparison across studies and increase the evidence base for more reliable clinical assessment and diagnosis., Competing Interests: Competing interests: None declared., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Rationale: Splenic artery embolization (SAE) is a common procedure in trauma patients with blunt splenic injuries. We report a case of acute ischemic stroke following orthopedic surgery in a patient with post-SAE reactive thrombocytosis., Patient Concerns: A 37-year-old woman with idiopathic thrombocytopenic purpura (ITP) suffered from multiple trauma scheduled for open reduction and internal fixation for right tibial and left radius fracture five days after SAE. The patient did not have any thromboembolic complications, although the platelet counts increased from 43 × 10/L to 568 × 10/L within two days after SAE. Surgery was completed under general anesthesia with tracheal intubation without complications. The patient complained of visual loss followed by limb weakness on the fourth and eighth hour postoperatively., Diagnoses: Magnetic resonance imaging (MRI) of head demonstrated ischemic change over bilateral basal ganglia, and occipital areas, suggesting the diagnosis of cortical blindness., Interventions: To suppress platelet count and avoid platelet hyper-aggregation, anti-platelet drug (i.e., oral aspirin 100 mg daily), hydration, and hydroxyurea (i.e., 20 mg/kg daily) were used for the treatment of reactive thrombocytosis., Outcomes: Although right-sided hemiparesis persisted, the patient reported mild visual recovery. She was discharged four months after SAE with active rehabilitation., Lessons: Our report highlights an increased risk of acute arterial thromboembolic events in patients with reactive thrombocytosis, especially those undergoing surgery.

Purpose: A pilot study was done to evaluate knowledge regarding "cortical visual impairment (CVI) in children" among ophthalmologists., Methods: This study was conducted during the annual conference of a zonal ophthalmological society. All ophthalmologists who attended the conference were requested to participate in this study. Those who agreed were given a validated questionnaire to assess knowledge regarding CVI. Cronbach's alpha of the questionnaire was 0.6. Participants were asked to respond to multiple choice questions by choosing the single best option. The responses obtained were then evaluated., Results: The total number of registered delegates in the conference was 448. A total of 103 ophthalmologists showed interest to participate in the study with a response rate of 22.9%. Only 89/103 interested delegates were included in the study as remaining were unaware of CVI. No participant gave correct answers to all questions. Although more than 80% of them knew the most common association (87%) and site of pathology (84%), only 52% were sure about clinical features and even lesser respondents (39%) knew that magnetic resonance imaging is the correct investigation of choice. The majority responded correctly that these children need eye examination (89%) and can be managed by rehabilitation through multidisciplinary approach (82%), but only 58% could recognize differential diagnoses and had a correct idea regarding the prognosis of CVI. There was no correlation between the number of patients diagnosed per month by the respondent with knowledge of the disease., Conclusion: In this pilot study, ophthalmologists were found to have limited knowledge regarding clinical features, investigation, differential diagnosis, and visual prognosis of CVI in children. There is a need to improve awareness regarding CVI among ophthalmologists.

Neurological manifestations of preeclampsia are serious and very variable. We report the case of a woman at 34 weeks gestation with preeclampsia who developed blindness within hours without alteration of consciousness. The imagery was in favor of posterior reversible encephalopathy. The therapeutic management consisted of emergency fetal extraction, the administration of antihypertensive drugs and magnesium sulfate. The outcome was favorable with complete recovery of visual function. This case reflects the heterogeneity of the clinical presentation of preeclampsia, as the only neurological manifestation was cortical blindness, with no associated consciousness disorder.

Cerebral hyperperfusion syndrome (CHS) is a well-documented complication after carotid endarterectomy or stenting. In contrast, CHS following vertebral revascularization is extremely rare. Here we present a case of a 77-year-old man with high-grade vertebral stenosis who subsequently underwent balloon angioplasty, complicated by hemorrhagic CHS manifesting as cortical blindness, although strict postoperative blood pressure control was administered. To our knowledge, cortical blindness as a presentation of hemorrhagic CHS has not previously been reported. This study highlights the fact that identifying high-risk patients, as well as making an individual therapeutic plan, is important prior to revascularization. Further studies are needed to elucidate the exact mechanism of this condition and thereby prevent it., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Rationale: The conception that multiple sclerosis may be challenging to distinguish from demyelinating manifestations of Sjögren's syndrome (SS) was introduced more than 30 years ago. However, it is now recognized that the neuromyelitis optica spectrum disorder (NMOSD) may occur more frequently in SS as opposed to multiple sclerosis. Characteristic NMOSD features can include severe attacks of optic neuritis, myelitis which is frequently longitudinally-extensive (spanning at least three vertebral segments on magnetic resonance imaging [MRI]), and an association with anti-aquaporin-4 antibodies. In addition, whereas NMOSD was initially thought to spare the brain, it is now recognized that brain lesions occur in a majority of NMOSD patients. Therefore, it is important for the multi-disciplinary team of physicians who care for SS patients to understand this widening spectrum of NMOSD as encompassing brain lesions. In this case-report we describe clinical features, radiographic findings, and treatment of a SS NMOSD patient presenting with severely decreased visual acuity, visual hallucinations, and encephalopathy., Patient Concerns: The SS NMOSD patient presented with rapid, bilateral onset of severely decreased visual acuity and was therefore suspected as having bilateral optic neuritis., Diagnosis: However, the patient lacked stigmata of optic neuritis, instead had visual hallucinations and encephalopathy suggestive of cortical blindness, and was noted to have occipital lobe lesions on brain MRI. Other radiographic findings included simultaneous enhancement of brainstem and periventricular lesions., Interventions: The patient was initially treated with methylprednisolone with no change in her neurological deficits. She was then treated with plasma exchange therapy., Outcomes: The patient had resolution of decreased visual acuity, visual hallucinations, encephalopathy, and contrast-enhancing brain lesions in response to plasma exchange therapy., Lesson: We provide the first example of severely decreased visual acuity in a NMOSD patient due to cortical blindness and not bilateral optic neuritis. This finding expands the spectrum of central nervous system syndromes and brain lesions which may occur in NMOSD. The synchronous enhancement of a brainstem lesion (known to occur in NMOSD) with occipital lobe lesions also suggests that our patient's occipital lobe findings were due to NMOSD. All of our patient's findings had an excellent clinical and radiographic response to plasma exchange therapy.

Mutations of the cystatin B gene (CSTB; OMIM 601145) are known to cause Unverricht-Lundborg disease or progressive myoclonic epilepsy-1A (EPM1A, MIM #254800). Most patients are homozygous for an expanded (>30) dodecamer repeat in the promoter region of CSTB, or are compound heterozygotes for the dodecamer repeat and a point mutation. We report two adolescent sisters born to consanguineous parents of Sri Lankan descent who presented with profound global developmental delay, microcephaly, cortical blindness and axial hypotonia with appendicular hypertonia. Neither sibling ever developed head control, independent sitting or ambulation, and never developed speech. The elder sister had a seizure disorder. Both sisters had profound microcephaly and distinct facial features. On serial brain imaging, they had progressive atrophy of the corpus callosum and supratentorial brain, and diffuse hypomyelination with progressive loss of myelin signal. Exome sequencing revealed both siblings to be homozygous for a c.218dupT (p.His75Serfs*2) mutation in exon 3 of CSTB. The neuroimaging features of our patients are consistent with those observed in Cstb-knockout mice, which supports the hypothesis that disease severity is inversely correlated with the amount of residual functional cystatin B protein.

Objective: We investigated neuronal correlates of faces versus non-faces processing in a cortically blind patient (TN) and a group of healthy age-matched controls in order to test electrophysiological correlates of the processing of pertinent stimuli in this patient., Methods: An EEG paradigm was used, in which intact and scrambled faces were displayed on a screen. First, time-frequency transforms were conducted on the patients' data alone. These oscillations were then compared to the frontal activity of six control participants., Results: Post stimulus oscillatory modulations (synchronisation in theta and alpha frequency bands) of both intact and scrambled faces at frontal scalp sites were observed in TN. These modulations were different for correct and incorrect responses. A more important increase in the theta band for incorrect responses was observed. The oscillatory rhythms highlighted in blindsight and in frontal regions differ from the ones observed in control participants., Conclusion: Despite the destruction of the visual cortex, oscillatory rhythms are not cancelled out but are shifted to anterior regions, revealing the activity of an alternate pathway for residual visual function., Significance: The results provide evidence for a top-down cognitive control process in blindsight., (Copyright © 2017 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Development of acute neurological symptoms secondary to cerebral oedema is well described in diabetic ketoacidosis (DKA) and often has a poor prognosis. We present the clinical and radiological data of a 17-yr-old girl who developed cortical blindness, progressive encephalopathy, and seizures caused by posterior reversible encephalopathy syndrome (PRES) that developed after her DKA had resolved. Vasogenic oedema in PRES resolves if the underlying trigger is identified and eliminated. In this case, hypertension was identified as the likely precipitating factor and following treatment her vision and neurological symptoms rapidly improved. We suggest how recent DKA may have contributed to the development of PRES in this patient., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

We present a case of a patient with Anton's syndrome due to decompression illness (DCI) after recreational scuba diving. Visual anosognosia, or denial of loss of vision, which is associated with lack of awareness regarding visual loss in the setting of cortical blindness, is known as Anton's syndrome (also termed Anton-Babinski syndrome). Our patient presented with progressive neurological DCI treated with repeated recompression. The anosogosia resolved after 48 h. Subsequent echocardiography revealed a persistent (patent) foramen ovale.

A middle-aged man with a history of type 2 diabetes mellitus, hypertension, hyperlipidemia, prior cerebral vascular accident, and remote history of generalized seizure disorder presented with worsening right-sided visual deficits and focal seizures. On examination the patient had dense homonymous hemianopsia on the right side. He was initially diagnosed with a stroke and underwent further investigation for potential causes. However, upon further review, magnetic resonance imaging (MRI) findings and repeat examination were more consistent with seizure-related effects on cortical brain matter as the cause of his visual disturbances rather than stroke. An EEG confirmed focal seizures from the left posterior quadrant of the brain, and the diagnosis of status epilepticus amauroticus (SEA) was made. The hemianopsia resolved with antiepileptic treatment.

A 54-year-old caucasian male developed bilateral blindness during an oxygen-ozone injection for disc herniation. The visual loss (VL) was immediately followed by severe frontal headache, vomiting, and nausea. The patient underestimated the VL showing Anton's syndrome, with a complete visual recovery after 2-month follow-up. Magnetic resonance data were consistent with recent ischemic lesions in bilateral vascular territories of posterior cerebral arteries.

Cortical blindness is a rare but frightening complication following coronary angiogram probably due to contrast penetration in occipital lobes in susceptible individuals [1, 2]., (Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.)

Background: The vulnerable brain areas in hypoxic-ischemic encephalopathy (HIE) following systemic hypotension are typically the neocortex, deep cerebral gray nuclei, hippocampus, cerebellum, and the parieto-occipital arterial border zone region. The visual cortex is not commonly recognized as a target in this setting., Methods: Single-institution review from 2007 to 2015 of patients who suffered cortical visual loss as an isolated clinical manifestation following systemic hypotension and whose brain imaging showed abnormalities limited to the occipital lobe., Results: Nine patients met inclusion criteria. Visual loss at outset ranged from hand movements to 20/20, but all patients had homonymous field loss at best. In 1 patient, imaging was initially normal but 4 months later showed encephalomalacia. In 2 patients, imaging was initially subtle enough to be recognized as abnormal only when radiologists were advised that cortical visual loss was present., Conclusions: The occipital lobe may be an isolated target in HIE with cortical visual loss as the only clinical manifestation. Imaging performed in the acute period may appear normal or disclose abnormalities subtle enough to be overlooked. Radiologists informed of the clinical manifestations may be more attune to these abnormalities, which will become more apparent months later when occipital volume loss develops.

Cerebral visual impairment (CVI) is a major cause of low vision in children due to impairment in projection and/or interpretation of the visual input in the brain. Although acquired causes for CVI are well known, genetic causes underlying CVI are largely unidentified. DNAs of 25 patients with CVI and intellectual disability, but without acquired (eg, perinatal) damage, were investigated by whole-exome sequencing. The data were analyzed for de novo, autosomal-recessive, and X-linked variants, and subsequently classified into known, candidate, or unlikely to be associated with CVI. This classification was based on the Online Mendelian Inheritance in Man database, literature reports, variant characteristics, and functional relevance of the gene. After classification, variants in four genes known to be associated with CVI (AHDC1, NGLY1, NR2F1, PGAP1) in 5 patients (20%) were identified, establishing a conclusive genetic diagnosis for CVI. In addition, in 11 patients (44%) with CVI, variants in one or more candidate genes were identified (ACP6, AMOT, ARHGEF10L, ATP6V1A, DCAF6, DLG4, GABRB2, GRIN1, GRIN2B, KCNQ3, KCTD19, RERE, SLC1A1, SLC25A16, SLC35A2, SOX5, UFSP2, UHMK1, ZFP30). Our findings show that diverse genetic causes underlie CVI, some of which will provide insight into the biology underlying this disease process.

Background: Visual loss associated with thoracic surgery has been reported mostly after coronary angiography or bypass surgery. The position of video-assisted thoracic surgery (VATS) is usually lateral, thus not compressive to the globe. Visual loss after VATS has not been reported. Herein we report a patient without any cardiovascular risk factors who experienced transient cortical blindness after an uneventful VATS., Case Presentation: A 40-year-old man noticed a visual loss at the recovery room after VATS. He showed normal pupillary reflex, normal optic disc appearance, and homonymous hemianopia respecting the vertical meridian, thus was typical for cortical visual impairment., Conclusions: Transient cortical visual impairment could be encountered after an uneventful VATS in a patient without any cardiovascular risk factors.

A 50-year old, diabetic, hypertensive patient with post-CABG status developed complete loss of vision about one hour after coronary angiogram (CAG). Thorough ophthalmological and neurological examination as well as magnetic resonance imaging of brain especially of the occipital region revealed no abnormality. The patient had complete recovery of vision about 48 hours later. We could not document any specific cause or mechanism for the visual loss, although the selective vulnerability of occipital lobes to contrast agent toxicity (Cortical blindness) was the most likely underlying mechanism.

Ozone (O₃) gas is a molecule that consists of 3 oxygen atoms, found out in the mid-19th century [1]. Ozone gas preserves humans from detrimental influences of ultraviolet radiation [1]. In spite of harmful effects of O₃ gas, investigators think that it has excessive curative effects [1]. Nowadays, O₃ therapy is used for many fields of medicine in precise therapeutic doses [1] and [2]. It is known that O₃ therapy is helpful in dental procedures, cerebrovascular diseases, tinnitus, acquired immunodeficiency syndrome, hypercholesterolemia, sensorial hypoacusis, senile dementia, multiple sclerosis, irradiation sensitive tumors, herpes simplex and herpes zoster virus infections, muscular hypertonia, and chronic otitis media, etc.[2]. The complications and disadvantages of O₃ therapy could be observed in the future. Herein, we presented a case of ischemic stroke after an oxygen-O₃ therapy, which is called also Anton syndrome.