Search

Background Brain imaging of patients with major depressive disorder (MDD) shows evidence of neurodegeneration and inflammation. The retina is believed to be a prolongation of the central nervous system, and therefore optical coherence tomography readings may give an insight into the pathogenesis of MDD. Patients and methods An observational controlled cross-sectional study was performed at Ain Shams University Hospitals from September 2018 to December 2019. Optical coherence tomography was used to compare a group of 30 patients with MDD with a control group of 28 healthy individuals. Measurements recorded were macular thickness, peripapillary retinal nerve fiber layer (RNFL) thickness, and ganglion cell-inner plexiform layer complex (GC-IP) thickness. Results Patients with MDD demonstrated significant macular thinning in all zones of the ETDRS map except the central zone bilaterally, and the inner nasal and inner lower zones in right eye. The peripapillary RNFL thickness of patients with MDD revealed significant thinning in the superior quadrant of both eyes. The GC-IP complex of patients with MDD showed significant thinning in the whole temporal and inner lower nasal subzones of the right eye and the upper nasal and lower temporal subfields of the left eye. Conclusion Patients with MDD showed reduced thickness of the macula, RNFL, and GC-IP. This may indicate that MDD involves an element of neurodegeneration, such as evidenced in gray matter changes. [ABSTRACT FROM AUTHOR]

Identification of ocular manifestations in patients with sensorineural hearing loss (SNHL) can have a large impact on the outcome and treatment of pediatric patients. Due to the common co-incidence of ocular manifestations and SNHL in children, both ophthalmologic and hearing loss screening and routine examinations must be conducted to minimize adverse outcomes and worsening of pathology. Early evaluation and diagnosis is imperative for intervention and further development of the patient. Coincidence requires a thorough evaluation that includes a comprehensive history, examination, and diagnostic testing. In this article, a literature review was conducted to analyze the presentations of various diseases and syndromes, such as Alport Syndrome, Waardenburg Syndrome, Norrie Disease, Usher Disease, Stickler Syndrome, Marfan Syndrome, Congenital Rubella, and Hereditary Optic Neuropathies. We divided the various ocular pathologies into anterior and posterior segment presentations and associated systemic findings for better understanding. Additionally, this review aims to include an update on the management of patients with both ocular and hearing loss manifestations. [ABSTRACT FROM AUTHOR]

Copyright of Die Ophthalmologie is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Neuroretinitis is an inflammatory condition with rapid unilateral vision loss, optic disc edema, and macular star formation. While neuroretinitis is commonly due to infectious causes such as Bartonella henselae, neuroretinitis due to toxoplasmosis is uncommon. A 29-year-old male presents to our neuro-ophthalmology clinic on December 7, 2021, at the University of Arkansas for Medical Sciences with symptoms of left eye pain and blurred vision. Subsequent workup led to the diagnosis and treatment of toxoplasma neuroretinitis. The fundus exam eventually demonstrated a notable macular star. Treatment was well tolerated, and the patient regained total visual acuity in the affected eye. Toxoplasma neuroretinitis is known for a characteristic appearance of optic disc edema prior to appearance of stellate maculopathy with vitreous inflammation and peripheral chorioretinal scars. Although loss of vision due to toxoplasmosis is rare, it should be included as part of the differential diagnosis with pertinent history. [ABSTRACT FROM AUTHOR]

Spaceflight associated neuro-ocular syndrome (SANS) refers to a unique collection of neuro-ophthalmic clinical and imaging findings observed in astronauts after long-duration spaceflight. Current in-flight and postflight imaging modalities (e.g., optical coherence tomography, orbital ultrasound, and funduscopy) have played an instrumental role in the understanding and monitoring of SANS development; however, the precise etiology for this neuro-ophthalmic phenomenon is still not completely understood. SANS may be a potential barrier to future deep space missions, and therefore it is critical to further elucidate the underlying pathophysiology for effective countermeasures. The complexity and unique limitations of spaceflight require careful consideration and integration of leading technology to advance our knowledge of this extraterrestrial syndrome. We describe the current neuro-ophthalmic imaging modalities and hypotheses that have improved our current understanding of SANS, discuss newer developments in SANS imaging (including noninvasive near-infrared spectroscopy) and summarize emerging research in the development of an aspirational future head-mounted virtual reality display with multimodal visual assessment technology for the detection of neuro-ocular findings in SANS. [ABSTRACT FROM AUTHOR]

Purpose: To investigate some diagnostic indicators in optic nerve head (ONH) analysis of children with 'Pseudopapilledema' and 'Papilledema' by Spectral Domain Optical Coherence Tomography (SD-OCT) METHODS: Medical records of 52 children with optic disc swelling, who were followed up by the departments of pediatric neurology and ophthalmology between May 2018 and May 2019 were reviewed retrospectively. Cases were classified as group 1 (Papilledema secondary to Idiopathic Intracranial Hypertension) including 54 eyes of 27 cases, group 2 (Pseudopapilledema secondary to bilateral optic disc drusen) including 50 eyes of 25 cases and control group. Three-dimensional imaging of ONH, peripapillary retinal nerve fiber layer (RNLF) thickness, Bruch's membrane opening height in the nasal respectively temporal quadrant (BMH-N, BMH-T) levels were measured by SD-OCT and B-mode ocular ultrasonography data of all cases were evaluated., Results: When RNFL levels were compared between groups, nasal RNFL levels were found to be significantly higher in group 1 cases compared to group 2 (p<0.001). In 3D imaging of the ONH among group 1 patients, the mean height of the apex in disc swelling was significantly higher than that of group 2 (p=0,024). The apex in disc swelling was mostly observed to be localized at the middle and had a diffuse swelling pattern in group 1; whereas, in group 2, the it was localized mostly at the nasal quadrant. This variation was statistically significant (p<0.001). When, Bruch's membrane opening height in the nasal respectively temporal quadrant (BMH-N, BMH-T) levels were compared, the measurements obtained were found to be significantly higher in group 1 (p=0,050 and p=0,003 respectively)., Conclusion: Nasal RNFL values of SD-OCT, Bruch's membrane opening height in the nasal respectively temporal quadrant (BMH-N, BMH-T) levels, the location of the apex in disc swelling obtained by 3D analysis of the ONH are found to be potential diagnostic parameters when combined with clinical findings. It is important that the nasal quadrant elevation, where the highest peak in 3D imaging was measured, was higher in the papilledema group. The elevation of the optic disc peak in 3D imaging can be used as a parameter to help clinicians distinguish between optic disc drusen (ODD) and papilledema., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2024. Published by Elsevier B.V.)

Purpose: Amyotrophic lateral sclerosis (ALS) clinical variability, along with the lack of conclusive diagnostic instruments, result in average diagnosis delays of 9 months. This study aimed to assess whether metabolomic profiling of basal tears in ALS patients could act as a biological marker for diagnosing ALS, predicting prognosis, and discriminating between endophenotypes., Methods: A single-center prospective case-control study was conducted in France from September 2021 to March 2023 including patients with ALS according to the revised EI Escorial criteria. Two microliters of basal tears were collected using microcapillary glass tubes and analyzed with ultra-high performance liquid chromatography coupled with mass spectrometry. Both univariate and multivariate analyses were performed., Results: Twenty-five patients with ALS and 30 controls were included. No significant differences in metabolite levels were found between ALS and control groups (p > 0.05). The basal tear metabolome significantly discriminated bulbar and spinal forms of ALS based on 6 metabolites, among which 5 were decreased (aniline, trigonelline, caffeine, theophylline and methyl beta-D-galactoside) in the bulbar form and 1 was decreased in the spinal form (dodecanedioic acid)., Conclusion: This study represents the first prospective analysis of basal tear metabolomics in individuals with ALS. Despite the inability to distinguish between ALS patients and controls based on metabolic signatures, these findings could contribute to understanding the phenotypic diversity of ALS. Notably, distinct metabolic profiles were identified that differentiate between the bulbar and spinal forms of the disease., Competing Interests: Declaration of competing interest None of the authors have any conflicts of interest to declare in relation with this study., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

Introduction: Traumatic brain injury (TBI) is a major cause of disability, with annual global incidence estimated as 69 million people. Survivors can experience long-term visual changes, altered mental state, neurological deficits and long-term effects that may be associated with mental illness. TBI is prevalent in military personnel due to gunshot wounds, and blast injury. This study aims to evaluate the relationship between evolving visual, biochemical and mental health changes in both military veterans and civilians, suffering from TBI, and detect preliminary indicators of prognosis for TBI recovery, and quality-of-life outcomes., Methods and Analysis: UNTANGLE is a 24-month prospective observational pilot study recruiting three patient groups: civilians with acute moderate-severe TBI, military veterans with diagnosis of a previous TBI and a control group of civilians or veterans with no history of a previous TBI. Patients will undergo visual, biochemical and mental health assessments, as well as patient-reported quality of life outcome measures over the course of a 1-year follow-up period., Ethics and Dissemination: Ethical approval has been obtained from the Health Research Authority and Health and Care Research Wales with a REC reference number of 23/NW/0203. The results of the study will be presented at scientific meetings and published in peer-reviewed journals, including both civilian and military-related publications. We will also present our findings at national and international meetings of learnt neuroscience and neuropsychiatry and military societies. We anticipate that our pilot study will inform a larger study on the long-term outcomes of TBI and quality of life, specific to military veterans, such that potential interventions may be accessed as quickly as possible., Trial Registration Number: ISRCTN13276511., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2024. Re-use permitted under CC BY. Published by BMJ.)

Abstract Coronavirus disease 2019 (COVID-19) is a current pandemic caused by SARS-CoV-2 that has vastly affected the whole world. Although respiratory disease is the most common manifestation of COVID-19, the virus can affect multiple organs. Neurotropic aspects of the virus are increasingly unfolding, in so far as some respiratory failures are attributed to brainstem involvement. The neuro-ophthalmic manifestations of COVID-19 and the neuro-ophthalmic side effects of vaccination were reviewed. The major findings are that the SARS-CoV-2 infection commonly causes headaches and ocular pain. It can affect the afferent and efferent visual pathways by ischemic or inflammatory mechanisms. Optic nerve may be the origin of transient or permanent visual loss from papillophlebitis, idiopathic intracranial hypertension, or optic neuritis. Cerebrovascular strokes are not uncommon and may lead to cortical visual impairment or optic nerve infarction. SARS-CoV-2 may affect the pupillomotor pathways, resulting in tonic pupil (Adie's syndrome) or Horner's syndrome. Cranial neuropathies including third, fourth, sixth, and seventh nerve palsies have all been reported. Rhino-orbital mucormycosis superinfections in COVID-19 patients receiving steroids or other immunosuppressive therapies may result in unilateral or bilateral visual loss and ophthalmoplegia. Autoimmune conditions such as Guillain-Barré, Miller-Fisher syndrome, and ocular myasthenia have been reported.

Purpose: To report a case of a non-arteritic anterior ischemic optic neuropathy (NAION) in the setting of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Observations: A 60-year-old healthy female without any risk factors for vasculopathy, presented with an acute painless diminution of vision noticed in the lower half of the visual field in the left eye. She was diagnosed with NAION in the setting of a recent SARS-CoV-2 infection. Conclusions and importance: The purpose of this case report is to supplement our knowledge about the neuro-ophthalmological complications of COVID-19 in the form of NAION which might occur even in the early stages of the infection.

The eye’s intricate anatomical barriers pose significant challenges to the penetration, residence time, and bioavailability of topically applied medications, particularly in managing uveitis and neuro-ophthalmologic conditions. Addressing this issue, polymeric nano-based drug delivery systems (DDS) have surfaced as a promising solution. These systems enhance drug bioavailability in hard-to-reach target tissues, extend residence time within ocular tissues, and utilize biodegradable and nanosized polymers to reduce undesirable side effects. Thus, they have stimulated substantial interest in crafting innovative treatments for uveitis and neuro-ophthalmologic diseases. This review provides a comprehensive exploration of polymeric nano-based DDS used for managing these conditions. We discuss the present therapeutic hurdles posed by these diseases and explore the potential role of various biopolymers in broadening our treatment repertoire. Our study incorporates a detailed literature review of preclinical and clinical studies from 2017 to 2023. Owing to advancements in polymer science, ocular DDS has made rapid strides, showing tremendous potential to revolutionize the treatment of patients with uveitis and neuro-ophthalmologic disorders.

Wolfram syndrome is a neurodegenerative disorder caused by pathogenic variants in the genes WFS1 or CISD2. Clinically, the classic phenotype is composed of optic atrophy, diabetes mellitus type 1, diabetes insipidus, and deafness. Wolfram syndrome, however, is phenotypically heterogenous with variable clinical manifestations and age of onset. We describe four cases of genetically confirmed Wolfram syndrome with variable presentations, including acute-on-chronic vision loss, dyschromatopsia, and tonic pupils. All patients had optic atrophy, only three had diabetes, and none exhibited the classic Wolfram phenotype. MRI revealed a varying degree of the classical features associated with the syndrome, including optic nerve, cerebellar, and brainstem atrophy. The cohort’s genotype and presentation supported the reported phenotype–genotype correlations for Wolfram, where missense variants lead to milder, later-onset presentation of the Wolfram syndrome spectrum. When early onset optic atrophy and/or diabetes mellitus are present in a patient, a diagnosis of Wolfram syndrome should be considered, as early diagnosis is crucial for the appropriate referrals and management of the associated conditions. Nevertheless, the condition should also be considered in otherwise unexplained, later-onset optic atrophy, given the phenotypic spectrum.

PURPOSE: Residual visual loss is an important predictor of optic neuritis relapse and progression. This study aimed to investigate the hidden residual visual loss in patients with optic neuritis using automated contrast-sensitivity (CS) function testing. MATERIALS AND METHODS: This cross-sectional study investigated 29 recovered optic neuritis patients (age: 27.69 ± 13.32 years, range: 13-51). Twenty age-matched controls with normal visual acuity (VA, in LogMAR) were recruited, for comparison with patients' VA and CS function after stable recovery from optic neuritis (6 months of follow-up). CS tests used a novel software that displays a single set of Gabor patches (2 cycles per degree at 10° ×10° of visual angle) with contrasts grating from 100% to 0.1%. RESULTS: There were 13 adolescent patients (63.6%: retrobulbar neuritis [RN]; 36.4%: papillitis), 14 adult patients (50%: RN; 42.9%: papillitis), and only 2 older patients (all with neuroretinitis). There was improvement of VA in the patient group at first diagnosis and follow-up (VA initial vs. final: 1.438 ± 1.134 vs. 0.235 ± 0.272, P < 0.001). This VA improvement was similar to control group (P = 0.052). In CS, there were significant differences in patient versus control groups (69.069% ± 70.235% vs. 27.215% ± 25.27%, P = 0.025). Linear regression showed that initial VA and CS function could not predict final VA (P = 0.183 and P = 0.138, respectively). CONCLUSIONS: Patients with optic neuritis showed decreased CS compared to control group which indicated the residual visual loss. Automated CS testing is useful in detecting residual visual loss in patients who recovered from optic neuritis. [ABSTRACT FROM AUTHOR]

Aim of the study. To investigate in a cross-sectional study the correlations of optical coherence tomography (OCT) with clinical and magnetic resonance imaging (MRI) parameters in multiple sclerosis (MS) patients. Material and methods. OCT parameters include the peripapillary retinal nerve fibre layer (pRNFL) and ganglion cell complex (GCC). Brain magnetic resonance volumetry (T2- and T1-lesions volume, whole brain volume and grey matter volume) was evaluated using the Icobrain program. Clinical data was compared according to the history of optic neuritis (HON). Correlations were determined between OCT parameters and demographic (age, gender), clinical (disease duration, Expanded Disability Status Scale score [EDSS]), and MRI data. Results. Out of 83 recruited people with MS, 27 had HON. The mean age of 75 patients with non-ON eyes was 42.08 ± 10.36 years, and 70.67% of the sample were females. Significant correlations were found between pRNFL and disability, along with several brain MRI-volumetry variables (Fluid-attenuated Inversion Recovery lesions volume [FLAIR]; T1-hypointense lesions volume; T1-lesions volume change; T1-volume lesions enlarging; whole brain volume; whole brain volume normative percentile; and volume of periventricular lesions). Multivariable linear regression analysis showed that age, pRNFL and GCC were significantly associated with T1-hypointense lesions volume change (the model explained 24% of the overall variance of the dependent variable). Conclusions. The pRFNL value correlates with disability and brain MRI-volumetric parameters in MS patients, serving as a useful neurodegeneration and inflammation surrogate marker. [ABSTRACT FROM AUTHOR]

Background: Hallucinogen persisting perception disorder (HPPD) is characterized by the re-emergence of perceptual symptoms experienced during acute hallucinogen intoxication following drug cessation. The underlying pathophysiology is poorly understood. We report the clinical characteristics and investigation findings of a series of HPPD cases with a literature review of previous case reports. We draw parallels between the features of HPPD and Visual Snow Syndrome (VSS). Methods: Retrospective case series of 13 patients referred from neuro-ophthalmologists. Literature review with 24 HPPD case reports were identified through database search using the terms "hallucinogenic persisting perception disorder" OR "hallucinogen persisting perception disorder." Results: Lysergic acid diethylamide (LSD), 3,4-Methyl enedioxy methamphetamine (MDMA) and cannabinoid use was common. Cannabinoids and MDMA were mostly used in association with classical hallucinogens. The most frequent symptoms in our patients were visual snow, floaters, palinopsia, photophobia and nyctalopia. In the literature other symptoms included visual hallucinations altered motion perception, palinopsia, tracers and color enhancement. Ophthalmic and neurologic investigations were mostly normal. The majority of patients had ongoing symptoms. Two of our patients fully recovered—one after treatment with benzodiazepine and one without treatment. Twenty-five percent of cases from the literature fully recovered. Conclusions: HPPD presents with heterogeneous visual phenomena on a background of previous classic and non-classic hallucinogen use. Ophthalmic investigations are typically normal. The symptoms of HPPD in our case series overlap with the typical features of Visual Snow Syndrome (VSS). Patients presenting with VSS should be screened for past recreational drug use. The DSM-5 description of HPPD does not include visual snow, nyctalopia, photophobia or floaters. A revision of the diagnostic criteria to include these symptoms may better reflect the typical clinical phenotype. Increased awareness of HPPD as a secondary cause of VSS can avoid extensive investigations. Controlled trials comparing primary and secondary VSS patients are needed to understand the pathophysiology better and optimize treatment for HPPD. [ABSTRACT FROM AUTHOR]

A 29-year-old girl presented complaining of acute bilateral visual loss associated with mild headache and retrobulbar pain. She was diagnosed with chiasmal optic neuritis caused by multiple sclerosis. Her visual acuity and visual field defect promptly improved after steroid therapy. However optical coherence tomography angiography showed a progressive reduction of superficial capillary plexus density of the retina and optic nerve consistent with the progressive impairment of the retinal ganglionar cell layer. Contrary to chiasmal compression, in chiasmal optic neuritis, the superficial capillary plexus density reduction is diffuse and does not reflect the peculiar anatomy of the chiasm. [ABSTRACT FROM AUTHOR]

Purpose: To examine systematically how prechiasmal, chiasmal, and postchiasmal lesions along the visual pathway affect the respective pupillary responses to specific local monochromatic stimuli. Methods: Chromatic pupil campimetry (CPC) was performed in three patient groups (10 subjects with status after anterior ischemic optic neuropathy, 6 with chiasmal lesions, and 12 with optic tract or occipital lobe lesions (tumor, ischemia)) using red, low-intensity red, and blue local stimuli within the central 30° visual field. Affected areas - as determined by visual field defects revealed using conventional static perimetry - were compared with non-affected areas. Outcome parameters were the relative maximal constriction amplitude (relMCA) and the latency to constriction onset of the pupillary responses. Results: A statistically significant relMCA reduction was observed in the affected areas of postchiasmal lesions with red (p = 0.004) and low-intensity red stimulation (p = 0.001). RelMCA reduction in the affected areas seemed more pronounced for low-intensity red stimulation (46.5% mean reduction compared to non-affected areas; 36% for red stimulation), however statistically not significant. In prechiasmal lesions, a statistically significant latency prolongation could be demonstrated in the affected areas with low-intensity red stimulation (p = 0.015). Conclusion: Our results indicate that the choice of stimulus characteristics is relevant in detecting defects in the pupillary pathway of impairment along the visual pathway, favoring red stimuli of low intensity over blue stimuli. Such knowledge opens the door for further fundamental research in pupillary pathways and is important for future clinical application of pupillography in neuro-ophthalmologic patients. [ABSTRACT FROM AUTHOR]

Monocular visual field defects generally localize at or anterior to the optic chiasm, while homonymous hemianopias localize to the retrochiasmal visual pathway. Highly incongruous visual field defects may be difficult to identify on 24-2 Humphrey visual field testing, and this case demonstrates the value of optical coherence tomography (OCT) ganglion cell-inner plexiform layer (GCIPL) in rapidly localizing the lesion. A 54-year-old woman was found on routine examination to have an isolated superonasal quadrant visual field defect respecting the vertical meridian in the left eye only on Humphrey 24-2 SITA-Fast testing. She had a remote history of significant head trauma. Visual acuity, anterior segment, and fundus examination were normal. OCT revealed a bow-tie atrophy of the retinal nerve fiber layer in the right eye (OD), and binocular homonymous hemi-macular atrophy of OCT GCIPL, confirming the localization was the left retrochiasmal visual pathway. A repeat Humphrey 30-2 SITA-Fast visual field demonstrated that the visual field defect was also present in the OD in a highly incongruous manner. Magnetic resonance imaging of the brain with contrast showed mild atrophy of the left optic tract. This case demonstrates that highly incongruous visual field defects may be difficult to identify on Humphrey 24-2 SITA-Fast visual fields, and OCT GCIPL serves as a rapid way to localize the lesion. More detailed visual field testing including 30-2 programs should be considered in these cases.

Vladislav O Soldatov,1,2 Michail S Kukharsky,3,4 Andrey E Belykh,5 Andrey M Sobolev,4 Alexey V Deykin2,6 1Core Facility Centre, Institute of Gene Biology, Russian Academy of Sciences, Moscow, Russia; 2Department of Pharmacology and Clinical Pharmacology, Belgorod State National Research University, Belgorod, Russia; 3Department of General and Cell Biology, Faculty of Medical Biology, Pirogov Russian National Research Medical University, Moscow, Russia; 4Laboratory of Genetic Modelling of Neurodegenerative Processes, Institute of Physiologically Active Compounds, Russian Academy of Sciences, Chernogolovka, Russia; 5Department of Pathophysiology, Kursk State Medical University, Kursk, Russia; 6Center for Precision Genome Editing and Genetic Technologies for Biomedicine, Institute of Gene Biology, Russian Academy of Sciences, Moscow, RussiaCorrespondence: Vladislav O SoldatovBelgorod State National Research University, 85 Pobedy St., Belgorod, Belgorod Region, 308015, Russian FederationTel +7 91 0325 8496Fax +7 47 2230-1012Email pharmsoldatov@gmail.comAbstract: Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease resulting in a gradual loss of motor neuron function. Although ophthalmic complaints are not presently considered a classic symptom of ALS, retinal changes such as thinning, axonal degeneration and inclusion bodies have been found in many patients. Retinal abnormalities observed in postmortem human tissues and animal models are similar to spinal cord changes in ALS. These findings are not dramatically unexpected because retina shares an ontogenetic relationship with the brain, and many genes are associated both with neurodegeneration and retinal diseases. Experimental studies have demonstrated that ALS affects many “vulnerable points” of the retina. Aggregate deposition, impaired nuclear protein import, endoplasmic reticulum stress, glutamate excitotoxicity, vascular regression, and mitochondrial dysfunction are factors suspected as being the main cause of motor neuron damage in ALS. Herein, we show that all of these pathways can affect retinal cells in the same way as motor neurons. Furthermore, we suppose that understanding the patterns of neuro-ophthalmic interaction in ALS can help in the diagnosis and treatment of this disease.Keywords: ALS, retina, retinal involvement, neuro-ophthalmology, mitochondrial dysfunction, excitotoxicity

PurposeMigraine, particularly with aura, has been associated with ocular and systemic ischemic complications, but there are limited data on the ocular vasculature in migraine. We used optical coherence tomography angiography (OCTA) to assess perfusion of the macula and optic nerve in migraine patients, with (MA) and without (MO) aura, compared to healthy controls (HC).MethodsWe recruited 15 MA (mean age 42 years), 12 MO (mean age 46 years), and 22 HC (mean age 39 years) participants from neurology and neuro-ophthalmology clinics. Participants underwent optical coherence tomography and 3 × 3 mm OCTA of the macula and optic nerve. Foveal avascular zone area was automatically measured using AngioVue software, and vessel density was calculated as blood vessel length divided by scan area (mm-1) after skeletonization of OCTA images.ResultsOn macular OCTA, MA participants had an enlarged foveal avascular zone area when compared with HC (0.300 ± 0.019 vs. 0.220 ± 0.066 mm2, P = 0.006). In addition, superficial foveal vessel density was decreased in MA participants when compared with MO participants (7.8 ± 0.31 vs. 9.3 ± 0.44, P = 0.04) and HC (7.8 ± 0.31 vs. 9.4 ± 0.21 mm-1, P = 0.002). On optic nerve OCTA, the MA participants had reduced superior peripapillary vessel density when compared with the MO participants (12.0 ± 0.45 vs. 14.0 ± 0.38 mm-1, P = 0.031) and HC (12.0 ± 0.45 vs. 14.1 ± 0.53 mm-1, P = 0.035). There were no significant differences between the MO and HC groups.ConclusionsMigraine with, but not without, aura was associated with foveal and peripapillary vascular decrements, which may possibly mediate increased risk of ocular and systemic vascular complications in these patients. OCTA could potentially be useful as a biomarker for migraine with aura.

A hallmark of myasthenia gravis (MG) is the variability and fatigability of striated muscles. The majority of people with MG have eye symptoms of ptosis and diplopia. This paper outlines the eye signs in MG, including practical tips on the examination technique relevant to MG, and pitfalls to avoid.

A 30-year-old female presented with complaints of decreased vision and diplopia since 1 week along with headache and gait disturbances. Examination revealed medial restriction of ocular movements in the right with left abducting nystagmus suggestive of right-sided internuclear ophthalmoplegia and right-sided facial palsy. The patient was admitted under neurology as a suspected case of demyelinating disorder. Magnetic resonance imaging with gadolinium contrast revealed features suggestive of probably multiple sclerosis (MS). The patient was given 5 days of high-dose intravenous steroids following which the patient showed improvement and was discharged on multivitamins. Subsequent follow-up showed significant improvement in symptoms, visual acuity, and ocular movements. Hereby presenting a rare case of MS presenting as unilateral internuclear ophthalmoplegia.

Purpose: Polymorphous low-grade adenocarcinoma is a tumor of the salivary glands that typically localizes within the oral cavity. We present a case of isolated third cranial nerve palsy as the initial presentation of polymorphous low-grade adenocarcinoma involving the left cavernous sinus in a patient status post glaucoma surgery. Observations: A 68-year-old woman status post glaucoma drainage device implantation in her left eye presented with an isolated left third nerve palsy ten weeks postoperatively. Differential diagnoses included microvascular ischemic neuropathy, postoperative ptosis, and compressive mass. MRI revealed a left cavernous sinus mass, and subsequent excisional biopsy revealed a diagnosis of polymorphous low-grade adenocarcinoma. Conclusions: There are few cases reporting polymorphous low-grade adenocarcinoma originating from and extending beyond the nasopharynx. This report emphasizes an unexpected neuro-ophthalmic manifestation of this salivary gland tumor.

Purpose: To report convergence insufficiency in a patient with Parkinson's’ disease stimulated by turning on the deep brain stimulator. Observations: 72-year-old male with Parkinson's disease and hypertension presenting for the evaluation of blurry vision at near and mid distance that started after activation of an implanted Deep brain stimulator.Baseline ophthalmologic evaluation prior to deep brain stimulator implantation surgery and with the deep brain stimulator turned off demonstrated a full motility, centered eyes for distance and near and a best corrected visual acuity of 20/20, normal pupil exam, confrontational visual fields and dilated fundus exam. Following this examination, the Deep brain stimulator was turned on and re-evaluation few minutes later demonstrated the same findings except for a 6-prism diopter exotropia at near consistent with convergence insufficiency.Following our evaluation a set of +3 diopters base-in prisms were added to near glasses with total relief of symptoms. The patient did not require surgical adjustment of the deep brain stimulator leads. Conclusions and importance: Given the therapeutic effects of deep brain stimulation on convergence insufficiency reported in several studies, in addition to the influence of deep brain stimulation as Parkinson's Disease treatment in areas possibly associated with vergence control, convergence insufficiency secondary to deep brain stimulation does not seem very unlikely, although not often reported. Further studies are needed to optimize deep brain stimulation surgery to maximize benefits and limit adverse events, as well as being aware of convergence insufficiency as a possible cause for visual disturbance.

BackgroundHallucinogen persisting perception disorder (HPPD) is characterized by the re-emergence of perceptual symptoms experienced during acute hallucinogen intoxication following drug cessation. The underlying pathophysiology is poorly understood. We report the clinical characteristics and investigation findings of a series of HPPD cases with a literature review of previous case reports. We draw parallels between the features of HPPD and Visual Snow Syndrome (VSS).MethodsRetrospective case series of 13 patients referred from neuro-ophthalmologists. Literature review with 24 HPPD case reports were identified through database search using the terms “hallucinogenic persisting perception disorder” OR “hallucinogen persisting perception disorder.”ResultsLysergic acid diethylamide (LSD), 3,4-Methyl enedioxy methamphetamine (MDMA) and cannabinoid use was common. Cannabinoids and MDMA were mostly used in association with classical hallucinogens. The most frequent symptoms in our patients were visual snow, floaters, palinopsia, photophobia and nyctalopia. In the literature other symptoms included visual hallucinations altered motion perception, palinopsia, tracers and color enhancement. Ophthalmic and neurologic investigations were mostly normal. The majority of patients had ongoing symptoms. Two of our patients fully recovered—one after treatment with benzodiazepine and one without treatment. Twenty-five percent of cases from the literature fully recovered.ConclusionsHPPD presents with heterogeneous visual phenomena on a background of previous classic and non-classic hallucinogen use. Ophthalmic investigations are typically normal. The symptoms of HPPD in our case series overlap with the typical features of Visual Snow Syndrome (VSS). Patients presenting with VSS should be screened for past recreational drug use. The DSM-5 description of HPPD does not include visual snow, nyctalopia, photophobia or floaters. A revision of the diagnostic criteria to include these symptoms may better reflect the typical clinical phenotype. Increased awareness of HPPD as a secondary cause of VSS can avoid extensive investigations. Controlled trials comparing primary and secondary VSS patients are needed to understand the pathophysiology better and optimize treatment for HPPD.

The eight-and-a-half syndrome is a rare neuro-ophthalmological condition caused by a structural lesion in the dorsal portion of the pons, involving critical areas of the brainstem, i.e., medial longitudinal fasciculus (MLF), abducens nucleus, facial genu, and colliculus. It is characterized by internuclear ophthalmoplegia with horizontal gaze palsy and peripheral facial palsy. Although the syndrome is most frequently caused by vascular or demyelinating diseases, several different underlying causes might occur. Herein, we describe a case of the eight-and-a-half syndrome caused by a lung adenocarcinoma metastasis localized in the lower pontine tegmentum. Then, we review the current literature on the underlying causes of the eight-and-a-half syndrome. [ABSTRACT FROM AUTHOR]

Purpose of Review: To highlight potential avenues to reduce preventable diagnostic error of neuro-ophthalmic conditions and avoid patient harm. Recent Findings: Recent prospective studies and studies of patient harm have advanced our understanding. Additionally, recent studies of fundus photography, telemedicine, and artificial intelligence highlight potential avenues for diagnostic improvement. Summary: Diagnostic error of neuro-ophthalmic conditions can often be traced to failure to gather an adequate history, perform a complete physical exam, obtain adequate/appropriate neuroimaging, and generate a complete, appropriate differential diagnosis. Improving triage and identification of neuro-ophthalmic conditions by other providers and increasing access to subspecialty neuro-ophthalmology evaluation are essential avenues to reduce diagnostic error. Further research should evaluate the relationship between misdiagnosis and patient harm, and help identify the most impactful potential targets for improvement. [ABSTRACT FROM AUTHOR]

Objectives: The purpose of this study was to compare the peripapillary and macular vascular flow changes between healthy adults and adult patients with superficial optic disk drusen (ODD), as evaluated by optical coherence tomography angiography (OCT-A). Methods: In this retrospective study, 44 eyes of 22 patients with superficial ODD followed-up in our neuro-ophthalmology unit and 54 eyes of 27 healthy individuals admitted to our clinic for routine ophthalmological examination were included in the study. The superior, inferior, nasal, and temporal radial peripapillary capillary plexus (RPC) vessel density (VD) values; superior, inferior, nasal, and temporal parafoveal and foveal region superficial capillary plexus (SCP) and deep capillary plexus (DCP) VD values; and choriocapillaris flow (CCF) area and foveal avascular zone area were evaluated by OCT-A. Results: There was no statistically significant difference between groups in terms of age and sex. While there was a significant decrease in the temporal RPC VD values in cases with ODD (p=0.02), no significant difference was observed in other quadrants. No significant differences in either parafoveal zone four quadrant, foveal SCP and DCP VD values, or foveal avascular zone measurements were found between groups, while CCF area values were significantly higher in the study group (p=0.012). Conclusion: Compared to healthy controls, our results showed a decrease in the RPC temporal quadrant VD values, and an increase in CCF area in cases with superficial ODD. To evaluate the effects of ODD on optic nerve head and macular perfusion, and to understand its underlying mechanisms and secondary complications, longer follow-up studies with larger case series are needed. [ABSTRACT FROM AUTHOR]

Objectives: To evaluate the management of the pediatric ophthalmology and strabismus clinic when strict quarantine conditions were adopted during the coronavirus disease 2019 (COVID-19) pandemic in Turkey. Materials and Methods: The study presents a review of the patients examined during the quarantine period. All patients were assessed with the highest possible level of personal protection. Results: Ten patients (6 girls, 4 boys) with a mean age of 9 years (range: 2-16) were evaluated. The patients presented 3-20 days after symptom onset. Ocular misalignment and diplopia were the main symptoms. Four of the 10 patients were diagnosed with sixth cranial nerve palsy and three patients were diagnosed with acute-onset comitant esotropia. Six patients had significant cranial magnetic resonance imaging findings. Conclusion: Acute-onset neurological conditions are more common during the COVID-19 pandemic. These reports will contribute to global experience and understanding of COVID-19. [ABSTRACT FROM AUTHOR]

We came together in March 2021 for a joyous celebration of Dr Gordon Plant's incredible career. This is a report of the meeting tidings. [ABSTRACT FROM AUTHOR]

Abstract Background Being a newly defined disease, RVCL-S is underrecognized by clinicians globally. It is an autosomal dominantly inherited small vessel disease caused by the heterozygous C-terminal frameshift mutation in TREX1 gene. RVCL-S is featured by cerebral dysfunction, retinopathy, and vasculopathy in multiple internal organs. Misdiagnosis may cause devastating consequences in patients, such as iatrogenic PML caused by misuse of immunosuppressants. Thus, increasing awareness of this disease is in urgent need. Results We uncovered a large Chinese origin RVCL-S pedigree bearing the TREX1 mutation. A comprehensive characterization combining clinical, genetic, and neuropathological analysis was performed. The Intrafamilial comparison showed highly heterogeneous clinical phenotypes. Mutation carriers in our pedigree presented with retinopathy (8/13), seizures (2/13), increased intracranial pressure (1/13), mild cognitive impairment (3/13), stroke-like episode (3/13), mesenteric ischemia (1/13), nephropathy (9/13), ascites (3/13), hypertension (9/13), hyperlipidemia (3/8), hypoalbuminemia (3/8), normocytic anemia (3/8), subclinical hypothyroidism (1/8), hyperfibrinogenemia (1/8), hyperparathyroidism (2/8), and abnormal inflammatory markers (4/8). The constellation of symptoms is highly varied, making RVCL-S a challenging diagnosis. Comparison with reported RVCL-S pedigrees further revealed that the mesenteric ischemia is a novel clinical finding and the MRS pattern of brain lesions is emulating neoplasm and tumefactive demyelination. Conclusion Our reports characterize a highly heterogeneous RVCL-S pedigree, highlight the probability of misdiagnosis in clinical practice, and broaden the clinical spectrum of RVCL-S.

Purpose: The aim of this study was to compare the performance of Octopus 900(OVF) kinetic module with Goldmann perimeter (GVF) and Humphrey 750i (HVF) perimeters in neuro-ophthalmic disorders. Methods: During this prospective observational cross-sectional study, 17 patients (26 eyes) with neuro-ophthalmic disorders underwent visual field examination on the three perimeters. Field defects on OVF were matched with HVF and GVF for the number of quadrants involved. An unmasked observer, and a masked observer (unaware of the clinical diagnosis) were made to separately diagnose the type of field defects on all three fields for the same patient. The pattern of field defect on OVF was compared with GVF and HVF field defects for both observers. Results: When OVF was compared with HVF and GVF, 88% eyes correctly matched for normal or abnormal visual fields, while quadrant-matching was 80% and 89% respectively. For the unmasked observer, the pattern of field defects on OVF was similar to HVF and GVF in 58% and 65% eyes respectively while for a masked observer, it was 54% and 62%. Central and paracentral scotomas showed unmatched fields when OVF was compared with HVF and GVF. When these patients were excluded, sensitivity of OVF increased to 95%. Conclusion: Clinical correlation aids in better characterisation of a field defect. All 3 perimeters are concurrent in the pattern of field defects for non-central defects. However, the default protocol on OVF may not be enough to demarcate the central and para-central scotomas. Development of a customised protocol for the assessment of central and centrocecal field defects increases the accuracy of OVF.

Purpose: Drawing differential diagnoses to a Neuro-ophthalmology clinical scenario is a difficult task for a neurology trainee. The authors conducted a study to determine if a mobile application specialized in suggesting differential diagnoses from clinical scenarios can complement clinical reasoning of a neurologist in training. Methods: A cross-sectional multicenter study was conducted to compare the accuracy of neurology residents versus a mobile medical app (Neurology Dx) in drawing a comprehensive list of differential diagnoses from Neuro-ophthalmology clinical vignettes. The differentials generated by residents and the App were compared with the Gold standard differential diagnoses adjudicated by experts. The prespecified primary outcome was the proportion of correctly identified high likely gold standard differential diagnosis by residents and App. Results: Neurology residents (n = 100) attempted 1500 Neuro-ophthalmology clinical vignettes. Frequency of correctly identified high likely differential diagnosis by residents was 19.42% versus 53.71% by the App (P < 0.0001). The first listed differential diagnosis by the residents matched with that of the first differential diagnosis adjudicated by experts (gold standard differential diagnosis) with a frequency of 26.5% versus 28.3% by the App, whereas the combined output of residents and App scored a frequency of 41.2% in identifying the first gold standard differential correctly. The residents correctly identified the first three and first five gold standard differential diagnosis with a frequency of 17.83% and 19.2%, respectively, as against 22.26% and 30.39% (P < 0.0001) by the App. Conclusion: A ruled based app in Neuro-ophthalmology has the potential to complement a neurology resident in drawing a comprehensive list of differential diagnoses.

Purpose: This case report represents poor nutritional intake and vomiting secondary to COVID-19 resulting in Wernicke's syndrome and blindness. Observations: We report the case of a 36 year old with a post-COVID episode of acute-subacute onset bilateral blindness ultimately diagnosed as Wernicke's syndrome based on MRI findings and clinical response to high dose IV thiamine supplementation. Conclusions and Importance: Given this patient's dramatic presentation of no light perception vision in both eyes and resolution of symptoms with treatment, it is reasonable to consider thiamine deficiency in any individual who presents with acute-subacute onset vision loss, particularly when the history is suggestive of potential nutritional deficiency.

The evaluation of central vestibular syndromes, especially in the acute setting, can pose a challenge even for the most experienced clinician. Of particular importance is the evaluation of ocular torsion and nystagmus, which can be sensitive for central vestibular pathology, but easily missed by the untrained eye. We present two cases of acute vestibular syndrome of central origin in which the use of magnified fundoscopy at the bedside aided the precise anatomical diagnosis to inform appropriate further management. We also review aspects of the pathophysiology and anatomy of vestibular roll plane disorders. In case 1, the finding of position-dependent ocular torsion facilitated a rapid distinction between central skew deviation and a trochlear nerve palsy. In case 2, the fundoscopic magnification identified a pure rotatory nystagmus indicative of a central vestibular disorder. Ophthalmoscopy remains a useful bedside technique in acute vertigo, but the use of inexpensive magnification with a smartphone can provide objective and recordable evidence of central vestibular pathology, aiding therapeutic decisions. [ABSTRACT FROM AUTHOR]

Central retinal artery occlusion (CRAO) is a rare condition. It is a diagnostic and therapeutic emergency. CRAO is analogous to an acute stroke of the eye. The disease usually affects patients after 60 years of age, and rarely young persons. The risk factors for a CRAO are similar to ischemic stroke. It is due to embolic, thrombotic, or coagulation disorders. Typically, patients with acute CRAO present monocular, painless, and severe loss of vision. We report a rare case of a young 33-year-old woman who presented an atypical ischemic stroke revealed by a unilateral CRAO. This case reports a rare condition affecting a young patient, and highlights the interest of ophthalmologic examination in the diagnostic of a neuro disease and the early management of ischemic stroke. [ABSTRACT FROM AUTHOR]

Visual complaints are commonly encountered by the practicing neurologist. We review assessment of vision loss, diplopia, and positive visual phenomena, all of which require a thoughtful evaluation to localize disease and refine management. While many causative entities are unlikely to cause poor visual outcomes, including dry eyes, migraine, and congenital strabismus, others may threaten vision, life, or both, such as posterior communicating artery aneurysms, pituitary apoplexy, or temporal arteritis. A systematic approach to vision loss and diplopia is reviewed along with focused differential diagnoses. [ABSTRACT FROM AUTHOR]

Moyamoya (MM) disease is a chronic cerebrovascular disease that can lead to progressive stenosis of the terminal portions of the internal carotid arteries and their proximal branches. We sought to investigate and quantify retinal vascular changes in patients with MM vasculopathy (MMV) using optical coherence tomography angiography (OCTA) compared to healthy controls. Our findings reveal retinal microvascular changes in patients with MMV and highlights the potential of OCTA imaging for the detection of subclinical retinal pathology. [ABSTRACT FROM AUTHOR]

The 43rd meeting of the Upper Midwest Neuro-Ophthalmology Group (UMNOG) took place on July 23, 2021. For the second sequential year, the meeting was held virtually due to the COVID-19 pandemic. The meeting was held in the honour of the late Ivy Dreizin MD. Ninety people attended virtually marking the highest UMNOG meeting attendance on record. There were 23 podium presentations interspersed with numerous personal testimonials recognising Dr Dreizin and her immense contributions to the UMNOG community. [ABSTRACT FROM AUTHOR]