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Journal of Clinical Medicine Sep 2022Acute idiopathic blind spot enlargement syndrome (AIBSES) is characterized by unilateral visual field loss in the blind spot area, acute onset photopsia, and...
Acute idiopathic blind spot enlargement syndrome (AIBSES) is characterized by unilateral visual field loss in the blind spot area, acute onset photopsia, and funduscopically few or no optic disc changes. AIBSES predominantly affects young adults and is often misdiagnosed as optic neuritis because of low awareness. Optical coherence tomography (OCT) has become the gold standard in diagnosing AIBSES as a disease of the outer retina. In our case series, we present three consecutive patients with AIBSES followed prospectively with and without steroid therapy. The patients, aged 25 to 27 years, presented in our neuroophthalmology department between 2020 and 2021. We report their disease course and management and discuss therapeutic options, as no well-established procedures exist. Common pitfalls and diagnostic errors are analysed. Two women and one man showed unilateral acute-onset photopsia and blind spot enlargement on perimetry without visual acuity reduction. Spectral domain OCT (Heidelberg Engineering, Heidelberg, Germany) revealed marked peripapillary changes in the ellipsoid zone and autofluorescence in all patients, corresponding to faint blurring of the optic disc margin. Characteristically, there was no P100 latency delay in the visual evoked potential in any of the patients. Two patients received weight-adapted oral prednisolone, which was gradually tapered over six to eight weeks. Two patients showed full recovery of their symptoms at six and seven months after onset, while mild defect healing was seen in one treated patient after 12 months. Follow-up OCT showed restoration of the outer retinal layers 6-12 months after disease onset. Careful history taking and an unprejudiced ophthalmological workup helps in diagnosing AIBSES in young adults with unilateral acute visual field defects. While its etiology is still unclear, accurate diagnosis of AIBSES can be made with peripapillary OCT. In our cases, the disease course of AIBSES was much better than its reputation. Early corticosteroid treatment may support outer retinal reorganisation, which can be followed with OCT in accordance with visual field restoration. This should be addressed in a prospective study.
PubMed: 36142923
DOI: 10.3390/jcm11185278 -
Frontiers in Bioscience (Elite Edition) Dec 2021The purpose of the study was to analyze the frequency of the spontaneous posterior vitreous detachment (PVD) in patients admitted to an Emergency Eye Department in Italy... (Observational Study)
Observational Study
The purpose of the study was to analyze the frequency of the spontaneous posterior vitreous detachment (PVD) in patients admitted to an Emergency Eye Department in Italy (EED) during the COVID-19 pandemic national lockdown in 2020 compared with the similar time period in 2019. In this retrospective observational study, patient records for ophthalmology EED patients in the month of April 2020 during the COVID-19 Italian national lockdown, were compared with those for an equivalent one-month period in 2019. Diagnoses, gender, and age were assessed. Unpaired Student -tests were used for continuous variables. Poisson regression was used for count analysis to compare categorical variables. Chi-square test was applied to asses proportion differences. In comparison with the 2019 equivalent period, there was a significant decrease in the overall number of EED visits and in the number of patients presenting with a spontaneous PVD during the 2020 lockdown (-41.6% and -49%, respectively). During the 2020 lockdown, all diagnostic categories showed less patient admittance, however, the proportions remained stable when considering the entire cohort. The proportion of urgent visits was 90% in 2020 and 86% in 2019 ( = 0.66). The proportion of EED patients affected by spontaneous PVD was comparable between the two study periods (8.4% in 2020 vs. 9.6% in 2019, = 0.34). Patients presenting with spontaneous PVD in both periods were significantly older when compared to patients with other pathologies (mean age of 63years in 2020 and 64years in 2019, < 0.001). There was a significant bias in female gender (61.2% in 2019 and 60% in 2020, < 0.05). There was a significant decrease of accesses to the EED during COVID-19 2020 lockdown. Patients affected by spontaneous PVD were about 50% less compared with the same period of 2019. Risk factors for the development of spontaneous PVD were older age and female gender. PVD represents a potentially visual function threatening condition because it can cause retinal ruptures and retinal detachment. Patients need to be educated to get urgent ophthalmic assessments in the presence of important acute signs and symptoms, like floaters and flashes, even in the presence of a lockdown.
Topics: COVID-19; Emergency Service, Hospital; Female; Humans; Italy; Male; Middle Aged; Pandemics; Quarantine; Vitreous Detachment
PubMed: 34937313
DOI: 10.52586/E883 -
ESC Heart Failure Apr 2024A therapeutic strategy for chronic heart failure (HF) is to lower resting heart rate (HR). Ivabradine is a well-known HR-lowering agent, but limited prospective data... (Observational Study)
Observational Study
AIMS
A therapeutic strategy for chronic heart failure (HF) is to lower resting heart rate (HR). Ivabradine is a well-known HR-lowering agent, but limited prospective data exist regarding its use in Chinese patients. This study aimed to evaluate the effectiveness and safety of ivabradine in Chinese patients with chronic HF.
METHODS AND RESULTS
This multicentre, single-arm, prospective, observational study enrolled Chinese patients with chronic HF. The primary outcome was change from baseline in HR at 1 and 6 months, measured by pulse counting. Effectiveness was also evaluated using laboratory tests, the Kansas City Cardiomyopathy Questionnaire (KCCQ) clinical summary score (CSS) and overall summary score (OSS), and New York Heart Association (NYHA) class. Treatment-emergent adverse events (TEAEs) were assessed. A post hoc analysis examined the effectiveness and safety of ivabradine combined with an angiotensin receptor-neprilysin inhibitor (ARNI) or beta-blocker. A total of 1003 patients were enrolled [mean age 54.4 ± 15.0 years, 773 male (77.1%), mean baseline HR 88.5 ± 11.3 b.p.m., mean blood pressure 115.7/74.4 ± 17.2/12.3 mmHg, mean left ventricular ejection fraction 30.9 ± 7.6%, NYHA Classes III and IV in 48.8% and 22.0% of patients, respectively]. HR decreased by a mean of 12.9 and 16.1 b.p.m. after 1 and 6 months, respectively (both P < 0.001). At Month 6, improvements in the KCCQ CSS and OSS of ≥5 points were observed in 72.1% and 74.1% of patients, respectively (both P < 0.001). Left ventricular ejection fraction increased by 12.1 ± 11.6 (P < 0.001), and 66.7% of patients showed improvement in NYHA class (P < 0.001). At Month 6, the overall proportion of patients in NYHA Classes III and IV was reduced to 13.5% and 2.1%, respectively. Serum brain natriuretic peptide (BNP) and N-terminal pro-BNP changed by -331.9 ng/L (-1238.6, -134.0) and -1113.8 ng/L (-2202.0, -297.2), respectively (P < 0.001). HR reductions and improvements in NYHA and KCCQ scores with ivabradine were similar with and without use of ARNIs or beta-blockers. Of 498 TEAEs in 296 patients (29.5%), 73 TEAEs in 55 patients (5.5%) were considered related to ivabradine [most frequent sinus bradycardia (n = 7) and photopsia (n = 7)]. TEAEs were reported in a similar number of patients in ARNI and beta-blocker subgroups (21.9-35.6%).
CONCLUSIONS
Ivabradine treatment reduced HR and improved cardiac function and health-related quality of life in Chinese patients with chronic HF. Benefits were seen irrespective of whether or not patients were also taking ARNIs or beta-blockers. Treatment was well tolerated with a similar profile to previous ivabradine studies.
Topics: Adult; Aged; Humans; Male; Middle Aged; Adrenergic beta-Antagonists; Benzazepines; Cardiovascular Agents; China; Heart Failure; Ivabradine; Prospective Studies; Quality of Life; Stroke Volume; Treatment Outcome; Ventricular Function, Left; Vision Disorders; Female
PubMed: 38193606
DOI: 10.1002/ehf2.14581 -
International Journal of Molecular... Jul 2022Pathogenic variants in DNA-damage regulated autophagy modulator 2 gene () cause a rare autosomal recessive retinal dystrophy and its disease course is not well...
Pathogenic variants in DNA-damage regulated autophagy modulator 2 gene () cause a rare autosomal recessive retinal dystrophy and its disease course is not well understood. We present two Slovenian patients harboring a novel variant and a detailed review of all 23 other patients described to date. Whole exome and whole genome sequencing were performed in the two patients, and both underwent ophthalmological examination with a 2-year follow-up. PubMed was searched for papers with clinical descriptions of DRAM2 retinopathy. Patient 1 was homozygous for a novel variant, p.Met1?, and presented with the acute onset of photopsia and retina-wide retinopathy at the age of 35 years. The patient was first thought to have an autoimmune retinopathy and was treated with mycophenolate mofetil, which provided some symptomatic relief. Patient 2 was compound heterozygous for p.Met1? and p.Leu246Pro and presented with late-onset maculopathy at the age of 59 years. On review, patients with DRAM2 retinopathy usually present in the third decade with central visual loss, outer retinal layer loss on optical coherence tomography and a hyperautofluorescent ring on fundus autofluorescence. Either cone-rod or rod-cone dystrophy phenotype is observed on electroretinography, reflecting the importance of DRAM2 in both photoreceptor types. Non-null variants can result in milder disease.
Topics: Autoimmune Diseases; Cone-Rod Dystrophies; Electroretinography; Humans; Membrane Proteins; Mutation; Pedigree; Phenotype; Retinal Dystrophies; Tomography, Optical Coherence
PubMed: 35806404
DOI: 10.3390/ijms23137398 -
European Journal of Ophthalmology May 2024To review all studies reporting the occurrence of white dot syndromes (WDSs) following SARS-COV-2 infection. (Review)
Review
PURPOSE
To review all studies reporting the occurrence of white dot syndromes (WDSs) following SARS-COV-2 infection.
METHODS
On May 12, 2023, we registered our protocol on PROSPERO [registration number: CRD42023426012]. Five different databases including PubMed, Scopus, Web of Science, Google Scholar, and Science Direct were searched up to May 2023. We included all studies that reported the symptoms of WDSs following SARS-COV-2 infection. The data was extracted using a uniform Excel extraction sheet. All statistical tests were conducted with a 95% confidence interval and a 5% error margin. A p-value of less than 0.05 was considered statistically significant. The publication bias of included studies was assessed using JBI Critical Appraisal Checklist for Case Reports and IHE Quality Appraisal Checklist for Case Series studies.
RESULTS
This review included thirty-two studies involving forty-eight patients. Acute macular neuroretinopathy was the most common disease (70.8%) followed by multiple evanescent white dot syndrome (14.6%) with 58.3% of WDS after their first SARS-COV-2 infection, and paracentral acute middle maculopathy (4.1%). They were mostly unilateral (56.2%). The presenting symptoms were blurred vision (70.8%), visual field disturbance (68.7%), and photopsia (20.8%). About 35.4% of the patients improved by their treatment and future complications were persistent scotoma (4.2%) and macular edema (2.1%).
CONCLUSION
White dot syndromes are very rare entities. Our findings suggest a possible association between white dot syndrome onset and SARS-COV-2 infection. We recommend ophthalmologists should be aware of this suggested association to deliver better management and patients' care.
PubMed: 38751139
DOI: 10.1177/11206721241255402 -
Cureus Dec 2022We report on a case of multiple evanescent white dot syndrome (MEWDS) following the simultaneous administration of the human papillomavirus and meningococcal (conjugate)...
We report on a case of multiple evanescent white dot syndrome (MEWDS) following the simultaneous administration of the human papillomavirus and meningococcal (conjugate) vaccines and two recurrences of MEWDS following the administration of the second dose of the human papillomavirus (HPV) vaccine and the COVID-19 vaccine and COVID-19 viral infection. A 17-year-old Hispanic female presented with a one-week history of photopsia and blurred vision in her left eye following the simultaneous administration of the human papillomavirus and meningococcal (conjugate) vaccines. Upon a comprehensive examination, her best-corrected visual acuity was 20/20 in the right eye and 20/100 in the left eye. A left fundus examination revealed multiple white dots in the macula and nasal periphery, consistent with a diagnosis of MEWDS. Ancillary testing, including fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and optical coherence tomography, supported the diagnosis. One month following her initial diagnosis, the patient's symptoms had resolved without any therapy, and a fundus examination revealed multiple relatively ill-defined brown-colored subretinal lesions in the nasal midperiphery, corresponding to the location of the previous MEWDS lesions. Subsequently, she received the second dose of the HPV vaccine and then developed a mild COVID-19 infection. Four months after the initial presentation, she received the first dose of the BNT162b2 COVID-19 vaccine, followed by the second dose a month later. Eight months following her initial presentation, she presented with photopsia in the right eye. Her visual acuity remained 20/20 in the right eye and improved to 20/20 in the left eye, and white dots were identified nasal to the disk and surrounding the peripapillary region; the contralateral MEWDS diagnosis was confirmed by the previously mentioned ancillary tests. At her one-month follow-up, she presented new onset photopsia of the right eye. Her visual acuity remained 20/20 in both eyes, and a fundus examination revealed white lesions suggestive of active MEWDS temporal to the macula and brown-colored spots nasal to the disk, suggestive of recovering MEWDS, nasally. The aforementioned testing confirmed the coexistence of new and resolving lesions; nonetheless, the patient's symptoms resolved without any therapy, and she received the third dose of the BNT162b2 COVID-19 vaccine 11 months after her initial presentation. Our case suggests that vaccines may serve as immunological triggers of MEWDS. Recurrent MEWDS may occur when an individual is exposed to a powerful immune challenge, such as receiving a wide array of vaccinations in a short period of time. We believe this case constitutes a previously undescribed finding of multiple relatively ill-defined brown-colored subretinal lesions present in late MEWDS.
PubMed: 36628035
DOI: 10.7759/cureus.32300 -
Journal of Cancer Research and Clinical... Mar 2022Fluid-conducting extracellular matrix patterns known as vasculogenic mimicry (VM) have been associated with poor prognosis in uveal melanoma and other cancers. We...
PURPOSE
Fluid-conducting extracellular matrix patterns known as vasculogenic mimicry (VM) have been associated with poor prognosis in uveal melanoma and other cancers. We investigate the correlations between VM, presenting symptoms, mortality, and the area density of periodic acid-Schiff positive histological patterns (PAS density).
METHODS
Sixty-nine patients that underwent enucleation for uveal melanoma between 2000 and 2007 were included. Clinicopathological parameters presenting symptoms and outcomes were collected. Histological tumor sections were evaluated for VM and PAS density was quantified with digital image analysis.
RESULTS
Thirty-four patients (49%) presented with blurred vision. 18 (26%) with a shadow in the visual field, 7 (10%) with photopsia and/or floaters, and 2 (3%) with metamorphopsia. Nine patients (13%) had no symptoms at all. Median follow-up was 16.7 years (SD 2.6). A shadow in the visual field, but no other symptom, was positively correlated with the presence of VM (φ 0.70, p < 0.001) and greater PAS density (p < 0.001). In multivariate regression, retinal detachment (RD), presence of VM, and PAS density ≥ median were independent predictors of a shadow, but not tumor distance to the macula, tumor apical thickness, tumor diameter, or ciliary body engagement. The presence of VM was associated with significantly shorter cumulative disease-specific survival (Wilcoxon p = 0.04), but not PAS density ≥ median, presenting symptoms or RD (p > 0.28).
CONCLUSION
Tumors from uveal melanoma patients that report a visual field shadow are likely to display VM and greater PAS density, likely explaining the previously reported association between this symptom and poor prognosis.
Topics: Female; Follow-Up Studies; Humans; Male; Melanoma; Middle Aged; Neovascularization, Pathologic; Prognosis; Retrospective Studies; Survival Rate; Uveal Neoplasms
PubMed: 34775516
DOI: 10.1007/s00432-021-03851-9 -
Case Reports in Neurology 2021Migraine with aura may be confused with a stroke. Magnetic resonance imaging is an important tool for the differential diagnosis. Cerebral hypoperfusion has been...
Migraine with aura may be confused with a stroke. Magnetic resonance imaging is an important tool for the differential diagnosis. Cerebral hypoperfusion has been described in classic migraine, mainly during the aura. A 47-year-old male had an unremarkable past medical history. After sneezing, he developed a left hemi hypoesthesia, bitemporal vision loss, photopsia, and some distortion in the position of letters and words. This lasted <1 h, and it was followed by a severe headache. A magnetic resonance angiography was performed during the headache. It showed a left hemispheric hypoperfusion that did not correlate with the symptoms described by the patient. It is believed that during the aura, cerebral blood flow decreases, leading to hypoxia and decreased cellular energy generation, and these metabolic alterations define the symptoms of the patient. In our case, we documented brain hypoperfusion during the headache in the ipsilateral brain hemisphere to the symptoms, which has no clinical correlation. This condition could be due to spasm in the capillary arteries, and it may persist and influence the clinical manifestations during the headache phase in migraine with aura. A state of generalized cerebral hyperperfusion has been suggested, and there may be a coexistence of both phenomena for some period. This may open a new line of research regarding the pathophysiology and vascular changes of migraine with aura.
PubMed: 34899251
DOI: 10.1159/000519508 -
Survey of Ophthalmology 2022Ocriplasmin is used to treat vitreomacular traction (VMT), with or without full-thickness macular hole (MH). We systematically reviewed the evidence on ocriplasmin's... (Meta-Analysis)
Meta-Analysis Review
Ocriplasmin for treatment of vitreomacular traction and macular hole: A systematic literature review and individual participant data meta-analysis of randomized, controlled, double-masked trials.
Ocriplasmin is used to treat vitreomacular traction (VMT), with or without full-thickness macular hole (MH). We systematically reviewed the evidence on ocriplasmin's effect on vitreomacular adhesion resolution (VMAR), MH closure, vitrectomy, and best-corrected visual acuity (BCVA) and investigated the effect of baseline covariates on outcome. We applied individual participant data meta-analyses to the entire population and to subgroups defined by MH or epiretinal membrane (ERM) presence. Safety data were pooled and tabulated. Five randomized controlled trials (1,067 participants) were included. Six months after treatment, ocriplasmin achieved higher rates of VMAR and MH closure versus control, lowered vitrectomy odds, and increased the likelihood of a ≥10-letter BCVA increase. VMAR rates were lower when ERM, broad VMA (> 1500 µm), diabetic retinopathy, or pseudophakia were present and higher in younger participants, women, and eyes with MHs. Ocriplasmin-treated participants experienced more short-term visual impairment that was not predictive of final BCVA, as well as vitreous floaters, photopsia, photophobia, eye pain, blurred vision, and dyschromatopsia. The most common serious adverse events for ocriplasmin and control, respectively, were MH progression (22.5%, 17.3%), new MH (1.5%, 3.4%) and retinal detachment (0.8%, 1.2%). Ocriplasmin promotes VMAR and MH closure. Transient visual phenomena are not uncommon.
Topics: Female; Fibrinolysin; Humans; Intravitreal Injections; Peptide Fragments; Retinal Diseases; Retinal Perforations; Tomography, Optical Coherence; Traction; Treatment Outcome; Vision Disorders; Visual Acuity; Vitreous Body; Vitreous Detachment
PubMed: 34480895
DOI: 10.1016/j.survophthal.2021.08.003 -
International Ophthalmology Apr 2024To review all studies reporting the onset of white dot syndromes following COVID-19 vaccines. (Review)
Review
PURPOSE
To review all studies reporting the onset of white dot syndromes following COVID-19 vaccines.
METHODS
Our protocol was registered prospectively on PROSPERO [registration number: CRD42023426012]. We searched five different databases including PubMed, Scopus, Web of Science, Google Scholar, and Science Direct up to May 2023. All the studies that reported the occurrence of white dot syndrome following COVID-19 vaccines were included. All statistical tests were conducted with a 95% confidence interval and a 5% error margin. A p value of less than 0.05 was considered statistically significant. The methodological quality of included studies was performed using the IHE Quality Appraisal Checklist for Case Series studies and JBI Critical Appraisal Checklist for Case Reports.
RESULTS
Fifty studies involving seventy-one subjects were included. Multiple evanescent white dot syndrome (MEWDS) was the most common disease (n = 25, 35.2% %), followed by acute macular neuroretinopathy (AMN) (n = 22, 31.0%) and acute posterior multifocal placoid pigment epitheliopathy (APMPPE) (n = 4, 5.6%). They were mostly unilateral (n = 50, 70.4%). The presenting symptoms were blurred vision (n = 26, 36.6%), paracentral scotoma (n = 19, 26.8%), visual field disturbance, and photopsia (n = 7, 9.9%). The mean duration for follow-up was 10.15 ± 14.04 weeks. Nineteen subjects (29.69%) received steroids with improvement reported in 68.4%. Eleven subjects (17.19%) were managed by observation only with reported full recovery and improvement.
CONCLUSION
White dot syndromes are very rare entities. Our findings highlight a possible association between COVID-19 vaccines and the occurrence of white dot syndromes. However, larger studies with good quality should be implemented to confirm these findings.
Topics: Humans; COVID-19; COVID-19 Vaccines; SARS-CoV-2; Tomography, Optical Coherence; White Dot Syndromes
PubMed: 38652153
DOI: 10.1007/s10792-024-03119-4