Effect of visual stimulation using color looming disc in Anton syndrome: a case report.

Objectives: Anton syndrome is arare stroke syndrome that develops after damage to both occipital lobes, leading to cortical blindness and visual anosognosia. This report describes the rehabilitation course and functional progress of a 42-year-old man diagnosed with Anton syndrome.Methods: The patient started visual stimulation therapy using a color looming disc 5 months after Anton syndrome onset, for 30 minutes a day, 5 times a week for 4 weeks, totaling 20 sessions.Results: After 4 weeks of visual stimulation therapy using color-looming discs, reading tests for consonants, numbers, words, and colors and the latency of the P100 of Visual Evoked Potential showed improvement. Additionally, improvements were noted in the Modified Barthel Index and Mini-Mental State Examination scores related to visual function.Conclusions: This case illustrates the effectiveness of visual stimulation therapy using color-looming discs and its potential to achieve positive outcomes.

Customized Visual Discrimination Digital Therapy According to Visual Field Defects in Chronic Stroke Patients.

BACKGROUND AND PURPOSE: Visual perceptual learning (VPL) may improve visual field defects (VFDs) after chronic stroke, but the optimal training duration and location remain unknown. This prospective study aimed to determine the efficacy of 8 weeks of VFD-customized visual discrimination training in improving poststroke VFDs. METHODS: Prospectively enrolled patients with poststroke VFDs initially received no training for 8 weeks (no-training phase). They subsequently underwent our customized VPL program that included orientation-discrimination tasks in individualized blind fields and central letter-discrimination tasks three times per week for 8 weeks (training phase). We analyzed the luminance detection sensitivity and deviation as measured using Humphrey visual field tests before and after the no-training and training phases. The vision-related quality of life was assessed at baseline and at a 16-week follow-up using the National Eye Institute Visual Function Questionnaire-25 (NEI-VFQ-25). RESULTS: Changes in mean total deviation (MTD) scores were greater during the training phase than during the no-training phase (defective hemifield, p=0.002; whole field, p=0.004). The MTD scores improved during the training phase (defective hemifield, p=0.004; whole field, p=0.016), but not during the no-training phase (defective hemifield, p=0.178; whole field, p=0.178). The difference between the improved and worsened areas (≥6 dB changes in luminance detection sensitivity) was greater during the training phase than during the no-training phase (p=0.009). The vision-specific social functioning subscore of the NEI-VFQ-25 improved after the 16-week study period (p=0.040). CONCLUSIONS: Our 8-week VFD-customized visual discrimination training protocol may effectively improve VFDs and vision-specific social functioning in chronic stroke patients.

A case of bilateral cortical blindness followed by generalised tonic-clonic seizure epilepsy in a patient with posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome (PRES) in end-stage kidney disease (ESKD) is rare, with ocular symptoms as the first manifestation being even rarer. Here, we report a case of PRES in a patient with ESKD, characterized by sudden binocular blurred vision followed by epilepsy, to improve the understanding of this syndrome among nephrologists and ophthalmologists. A 50-year-old female requested an ophthalmic consultation due to bilateral vision loss followed by generalised tonic-clonic seizures. One month before onset of current illness, she developed ESKD secondary to rapid progression of previous ANCA vasculitis associated renal damage. Latter magnetic resonance imaging confirmed the diagnosis of PRES. Two weeks later, the patient's vision fully recovered. Conclusion: PRES is not an etiological diagnosis but a neuroimaging sign. In addition, PRES is a danger signal that is usually reversible if recognized and treated early, and can be life-threatening if treatment is delayed.

Transient cortical blindness following peripheral vascular trauma: A case report.

Cortical blindness is characterized by unilateral or bilateral vision loss despite an intact pupillary reflex, full extraocular movements, and normal fundoscopic examination. Common causes include stroke, cardiac emboli, head trauma or rarely, a hypoxic-ischemic event which results to decreased perfusion to the occipital lobes supplied by the posterior cerebral artery. Imaging with computed tomography is usually diagnostic documenting stroke or embolization as well as ensuring an intact cerebral circulation. Prognosis largely depends on the etiology as most reports document an irreversible condition or at least the patient is left with some residual visual symptoms. We present a case of a 25-year-old male who underwent brachial artery repair with reverse saphenous vein graft interposition after sustaining a right upper arm laceration associated with massive hemorrhage and shock due to delayed consult. He presented with profound bilateral loss of vision 12 h after surgery characterized as right homonymous hemianopsia. Computed tomography of the brain demonstrated ischemic infarcts in the occipital lobes. Close observation was instituted, and his symptom resolved spontaneously within a week. This case highlights the importance of considering atypical causes of perioperative vision loss as early recognition and timely diagnosis are essential to improve patient outcomes. To our knowledge, this is the first report of transient cortical blindness after peripheral vascular trauma.

A de novo novel variant in the MT-TD gene is associated with prominent extra-neurologic manifestations.

Defects in the mitochondrial tRNA genes cause a group of highly clinically and genetically heterogeneous disorders, which poses a challenge for clinical identification and genetic diagnosis. Here, we present a pre-school boy with a novel MT-TD variant m.7560T>C at the heteroplasmy level of 76.53% in blood, 93.34% in urine sediments, and absent in the healthy mother's blood and urine. Besides convulsions, brain magnetic resonance imaging abnormalities and high plasma lactate, the boy presented with the prominent extra-neurologic phenotype including steroid-resistant nephrotic syndrome associated with focal segmental glomerulosclerosis characterized by abnormal mitochondria in podocytes, cortical blindness, and pancreatitis. To our knowledge, this is the unique case with MT-TD m.7560T>C-related multi-organ impairments, which expands the phenotypic and mutational spectrum of primary mitochondrial diseases.

Vision-related quality of life after unilateral occipital stroke.

BACKGROUND/OBJECTIVES: Stroke damage to the primary visual cortex induces large, homonymous visual field defects that impair daily living. Here, we asked if vision-related quality of life (VR-QoL) is impacted by time since stroke. SUBJECTS/METHODS: We conducted a retrospective meta-analysis of 95 occipital stroke patients (female/male = 26/69, 27-78 years old, 0.5-373.5 months poststroke) in whom VR-QoL was estimated using the National Eye Institute Visual Functioning Questionnaire (NEI-VFQ) and its 10-item neuro-ophthalmic supplement (Neuro10). Visual deficit severity was represented by the perimetric mean deviation (PMD) calculated from 24-2 Humphrey visual fields. Data were compared with published cohorts of visually intact controls. The relationship between VR-QoL and time poststroke was assessed across participants, adjusting for deficit severity and age with a multiple linear regression analysis. RESULTS: Occipital stroke patients had significantly lower NEI-VFQ and Neuro10 composite scores than controls. All subscale scores describing specific aspects of visual ability and functioning were impaired except for ocular pain and general health, which did not differ significantly from controls. Surprisingly, visual deficit severity was not correlated with either composite score, both of which increased with time poststroke, even when adjusting for PMD and age. CONCLUSIONS: VR-QoL appears to improve with time postoccipital stroke, irrespective of visual deficit size or patient age at insult. This may reflect the natural development of compensatory strategies and lifestyle adjustments. Thus, future studies examining the impact of rehabilitation on daily living in this patient population should consider the possibility that their VR-QoL may change gradually over time, even without therapeutic intervention.

Carbon Monoxide-Related Vision Loss in an Acute Burn Patient.

Carbon monoxide poisoning can occur as part of smoke exposure in the burn population. Here we report the case of a 32-year-old, previously healthy male, with carbon monoxide-related blindness after smoke exposure in an apartment fire. Cerebral hypoperfusion was diagnosed using magnetic resonance imaging of the brain, and the patient was diagnosed with cortical visual impairment. He was treated with hyperbaric oxygen therapy following which he had partial recovery of his vision. There is a paucity of information regarding this phenomenon and its treatment.

CDKL5 deficiency-related neurodevelopmental disorders: a multi-center cohort study in Italy.

CDKL5 deficiency disorder (CDD) is a complex clinical condition resulting from non-functional or absent CDKL5 protein, a serine-threonine kinase pivotal for neural maturation and synaptogenesis. The disorder manifests primarily as developmental epileptic encephalopathy, with associated neurological phenotypes, such as hypotonia, movement disorders, visual impairment, and gastrointestinal issues. Its prevalence is estimated at 1 in 40,000-60,000 live births, and it is more prevalent in females due to the lethality of germline mutations in males during fetal development. This Italian multi-center observational study focused on 34 patients with CDKL5-related epileptic encephalopathy, aiming to enhance the understanding of the clinical and molecular aspects of CDD. The study, conducted across 14 pediatric neurology tertiary care centers in Italy, covered various aspects, including phenotypic presentations, seizure types, EEG patterns, treatments, neuroimaging findings, severity of psychomotor delay, and variant-phenotype correlations. The results highlighted the heterogeneity of seizure patterns, with hypermotor-tonic-spasms sequence seizures (HTSS) noted in 17.6% of patients. The study revealed a lack of clear genotype-phenotype correlation within the cohort. The presence of HTSS or HTSS-like at onset resulted a negative prognostic factor for the presence of daily seizures at long-term follow-up in CDD patients. Despite extensive polypharmacotherapy, including medications such as valproic acid, clobazam, cannabidiol, and others, sustained seizure freedom proved elusive, affirming the inherent drug-resistant nature of CDD. The findings underscored the need for further research to explore response rates to different treatments and the potential role of non-pharmacological interventions in managing this challenging disorder.

[Perioperative visual loss : Rare, unknown, relevant?] / Perioperativer Visusverlust : Selten, unbekannt, relevant?

Perioperative visual loss (POVL) is a rare but severe complication following non-ophthalmological surgery under general anesthesia. A POVL can be caused by lesions in any part of the optical system. The predominant causes include corneal injuries and particularly ischemic damage. The symptoms of POVL substantially vary ranging from reduced vision to complete blindness. The risks involve factors related to the surgery as well as patient-specific factors. In general, the prognosis in cases of mechanical damage is better than for ischemic lesions. The treatment measures depend on the underlying pathomechanism and due to the limited evidence only a few treatment options are available. Therefore, preventive measures and meticulous documentation play a crucial role.

Anton-Babinski Syndrome: A Visual Anosognosia.

Anton-Babinski syndrome is a rare extension of cortical blindness following injury to the occipital lobe. The patient behaves as sighted but has visual function derangements. The posterior cerebral artery (PCA) stroke represents 5% to 10% of total strokes. The COVID-19 pandemic has shown a rise in stroke cases. We present a case of this rare PCA stroke, first diagnosed by an ophthalmologist. This case had an inconsistent initial presentation, but subsequent computed tomography of the brain and other neurological investigations confirmed the diagnosis. If such cases are diagnosed early, they could have better management. Timely intervention can decrease morbidity as well as mortality.

Are we aware of neural activity in primary visual cortex? A neuropsychological case study.

OBJECTIVE: According to a seminal hypothesis stated by Crick and Koch in 1995, one is not aware of neural activity in primary visual cortex (V1) because this region lacks reciprocal connections with prefrontal cortex (PFC). METHODS: We provide here a neuropsychological illustration of this hypothesis in a patient with a very rare form of cortical blindness: ventral and dorsal cortical pathways were lesioned bilaterally while V1 areas were partially preserved. RESULTS: Visual stimuli escaped conscious perception but still activated V1 regions that were functionally disconnected from PFC. INTERPRETATION: These results are consistent with the hypothesis of a causal role of PFC in visual awareness.

Acute encephalopathy with biphasic seizures and late reduced diffusion with concurrent transverse myelitis.

We describe a patient with acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) with unique features, including concurrent transverse myelitis. A 2-year-old previously healthy girl had clinical findings consistent with AESD, occurring in association with influenza A infection. The posterior brain regions were most severely affected, resulting in cortical blindness. She also developed bilateral limb weakness, and spine MRI revealed transverse myelitis in the cervical region. She was treated acutely with intravenous methylprednisolone. Serum anti-myelin oligodendrocyte glycoprotein and anti-aquaporin-4 antibodies were negative, as was an anti-extractable nuclear antigen panel. Although her clinical presentation was severe, she improved dramatically over the following months, and 6 months following initial presentation, her parents felt she had returned to baseline. This is the first report of AESD occurring in combination with transverse myelitis. The co-occurrence of the two conditions is unlikely to be coincidental, suggesting that there may be a shared or overlapping immunological pathway involved. The patient's recovery was impressive, which could partially relate to the acute treatment with corticosteroids.

Perioperative Blindness in Spine Surgery: A Scoping Literature Review.

Perioperative vision loss (POVL) is a devastating surgical complication that impacts both the recovery from surgery and quality of life, most commonly occurring after spine surgery. With rates of spine surgery dramatically increasing, the prevalence of POVL will increase proportionately. This scoping review aims to aggregate the literature pertinent to POVL in spine surgery and consolidate recommendations and preventative measures to reduce the risk of POVL. There are several causes of POVL, and the main contribution following spine surgery is ischemic optic neuropathy (ION). Vision loss often manifests immediately following surgery and is irreversible and severe. Diffusion weighted imaging has recently surfaced as a diagnostic tool to identify ION. There are no effective treatments; therefore, risk stratification for counseling and prevention are vital. Patients undergoing prone surgery of long duration and/or with significant expected blood loss are at greatest risk. Future research is necessary to develop effective treatments.

Insights into Anton Syndrome: When the brain denies blindness.

INTRODUCTION: Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia, manifests as bilateral vision loss coupled with denial of blindness. This systematic review delves into 64 studies encompassing 72 AS cases to explore demographics, clinical presentations, treatments, and outcomes. MATERIALS AND METHODS: The study rigorously followed PRISMA guidelines, screening PubMed, Google Scholar, and Scopus databases without timeframe limitations. Only English human studies providing full text were included. Data underwent thorough assessment, examining patient demographics, etiological variables, and treatment modalities. RESULTS: Sixty-four studies met the stringent inclusion criteria. Examining 72 AS cases showed a median age of 55 (6 to 96 years) with no gender preference. Hypertension (34.7 %) and visual anosognosia (90.3 %) were prevalent. Stroke (40.3 %) topped causes. Management included supportive (30.6 %) and causal approaches (30.6 %). Improvement was seen in 45.8 %, unchanged in 22.2 %, and deterioration in 11.1 %. Anticoagulation correlated with higher mortality (p < 0.05). DISCUSSION: AS, an unusual manifestation of blindness, stems mainly from occipital lobe damage, often due to cerebrovascular incidents. The syndrome shares features with Dide-Botcazo syndrome and dates back to Roman times. Its causes range from strokes to rare conditions like multiple sclerosis exacerbation. Accurate diagnosis involves considering clinical presentations and imaging studies, distinguishing AS from similar conditions. CONCLUSION: This comprehensive review sheds light on AS's complex landscape, emphasizing diverse etiologies, clinical features, and treatment options. Tailored treatments aligned with individual causes are crucial. The study's findings caution against blanket anticoagulation therapy, suggesting a nuanced approach. Further research is pivotal to refine diagnostics and optimize care for AS individuals.

Posterior Reversible Encephalopathy Syndrome in the Immediate Postoperative Period of Gastric Cancer.

A 55-year-old female was referred to the Department of Ophthalmology with complaints of bilateral loss of vision. She had undergone subtotal gastrectomy with gastrojejunostomy and lymphadenectomy for poorly differentiated gastric adenocarcinoma in the antropyloric region the day before. On the first postoperative day, she complained of generalised weakness, drowsiness, altered sensorium, and acute, painless, bilateral loss of vision. Ocular examination revealed visual acuity as no perception of light, bilaterally, and normal pupillary light reflexes. Anterior and posterior segment examination was within normal limits. This clinical presentation of altered sensorium and cortical blindness along with characteristic radiological findings (hyperintensity on T2/fluid-attenuated inversion recovery sequence involving the bilateral parieto-occipital lobe extending in asymmetric fashion to the bilateral cerebellum, brainstem, and thalami predominantly involving the white matter with few areas of diffusion restriction on diffusion-weighted imaging sequence predominantly on the left side with gyriform pattern) confirmed the diagnosis of posterior reversible encephalopathy syndrome (PRES). In cancer patients, PRES has been reported in patients on chemotherapy regimen or two weeks after surgery for gastric cancer. Here, we want to draw attention to the fact that PRES may develop in the immediate postoperative period of gastric cancer surgery, as seen in our case.

Anton-Babinski Syndrome in a Recurrent Ischemic Stroke Patient: A Case Report.

Anton-Babinski syndrome (ABS) is a rare neuropsychiatric condition characterized by visual anosognosia (denial of vision loss) and confabulation in the presence of intact anterior visual tracts. The most common cause of ABS is a cerebrovascular accident involving bilateral occipital lobe injuries with varying degrees of cortical blindness. In this report, we present the case of a woman with suspected ABS following a recurrent ischemic stroke in Malaysia. Establishing a proper diagnosis of stroke is crucial for modifying rehabilitation goals to ensure improved functional outcomes.

Cortical Blindness and Thrombotic Microangiopathy Following a Hemotoxic Snake Envenomation: An Unusual Presentation.

Snake envenomation leads to significant morbidity and mortality with local, hematological, renal, and neurological complications. Hemotoxic envenomation activates a hemorrhagic cascade, leading to many manifestations ranging from skin bleeds to major organ bleeds and fatal intracranial hemorrhage. Although rare, ischemic manifestations are possible following a hemotoxic envenomation, and they may present as cortical blindness, an unusual ocular symptom. Snake envenomation is also known to cause multifactorial acute kidney injury (AKI), precipitated by hemodynamic disturbances secondary to rhabdomyolysis, hemoglobinuria, direct tubular toxicity, and thrombotic microangiopathy. Thrombotic microangiopathy (TMA) is often overlooked in snake bites, as the hematological manifestations are often conveniently attributed to venom-induced consumptive coagulopathy (VICC). The distinct clinical entity of thrombotic microangiopathy should factor into one's differential diagnosis in patients presenting with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury following a snake bite. We report a patient who developed cortical blindness and thrombotic microangiopathy following snake envenomation, which are uncommon sequelae.

Visual stimulation rehabilitation for cortical blindness after vertebral artery interventional surgery: A case report and literature review.

INTRODUCTION AND IMPORTANCE: Cortical blindness (CB) after vertebral artery interventional surgery is not a frequently reported complication. In this study, the efficacy of visual stimulation rehabilitation consisting of visual recovery training and repetitive transcranial magnetic stimulation (rTMS) for cortical blindness was investigated by clinical evaluation, ophthalmologic examination, and electroencephalography (EEG). CASE PRESENTATION: This study reports on a 55-year-old male who showed partial bilateral posterior cerebral artery cortical branch occlusion after timely embolectomy due to thrombus dislodgement during right vertebral artery opening, stenting resulting in basilar artery tip occlusion. The lesions were mainly located in the right cerebellar hemisphere and bilateral occipital lobes, and the patient suffered from bilateral loss of vision, with only light perception preserved. The patient began to receive visual recovery training and 15 sessions of right occipital high-frequency transcranial magnetic stimulation 5 days after the onset. CLINICAL DISCUSSION: After treatment, the patient's capacity to identify things improved, allowing him to watch television, as did the precision and fluency of random hand movements, walking, and self-care. CONCLUSION: Visual stimulation rehabilitation composed of visual recovery training and rTMS is a promising therapy option for cortical blindness, and our case report provides clinical experience with vision recovery for patients with cortical blindness.

Erratum: Case report: SMART ANTON: Anton-Babinski syndrome in stroke-like migraine attacks (SMART) after radiation therapy: Two rare syndromes, one case.

[This corrects the article DOI: 10.3389/fneur.2022.887287.].