Neuro-ophthalmologic Evaluation Research Articles

Applying the 2022 optic neuritis criteria to noninflammatory optic neuropathies with optic nerve T2-hyperintensity: an observational study.

Recent diagnostic criteria for optic neuritis include T2-hyperintensity of the optic nerve (ON), even without associated contrast enhancement. However, isolated ON-T2-hyperintensity is a nonspecific finding found in any optic neuropathy or severe retinopathy. We applied the 2022 optic neuritis diagnostic criteria to a cohort of patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one eye, to assess the rate of optic neuritis misdiagnosis using these criteria. Retrospective study of consecutive patients who underwent brain/orbit MRI with/without contrast between 07/01/2019 and 06/30/2022. Patients with ON-T2-hyperintensity in at least one eye were included. The 2022 optic neuritis diagnostic criteria were applied to patients with noninflammatory optic neuropathies who had an ophthalmologic examination available for review. Of 150 patients included, 85/150 had compressive optic neuropathy; 32/150 had glaucoma; 12/150 had papilledema; 8/150 had hereditary (3), radiation-induced (3), nutritional (1), traumatic (1) optic neuropathies (none fulfilled the criteria); 13/150 had ischemic optic neuropathy and 4 fulfilled the criteria as definite optic neuritis due to contrast enhancement of the ON head. Seven additional patients would have satisfied the diagnostic criteria if red flags for alternative diagnoses had been overlooked. The application of the 2022 optic neuritis diagnostic criteria in patients with noninflammatory optic neuropathy and ON-T2-hyperintensity in at least one ON resulted in misdiagnosis of optic neuritis in only 4 patients because of ON head enhancement, all with nonarteritic anterior ischemic optic neuropathy. Neuro-ophthalmologic evaluation and exclusion of the ON head as a location in the MRI criteria would have prevented optic neuritis misdiagnosis in our study.

Visual outcomes in tuberculum sellae meningiomas comparing transcranial and endoscopic endonasal approaches

Tuberculum sellae meningiomas (TSM) account for 3-10% of intracranial meningiomas. Visual loss is the presenting symptom in up to 80% of cases. Surgical management poses a great challenge due to tumor proximity to neurovascular structures such as the optic nerve and the internal carotid artery (ICA); hence, there is controversy regarding the optimal approach. The aim of this study is to determine differences in visual outcomes between transcranial (TCA) and endoscopic endonasal (EEA) approaches. A retrospective study including 29 patients with TSM surgically treated by TCA or EEA between 2011 and 2023 in a single referral center was conducted. Pre-and post-operative neuro-ophthalmologic evaluations, focusing on visual acuity and campimetry, were evaluated. Sixteen (55.16%) patients were intervened through a TCA and the remaining 13 (44.84%) via an EEA. The lesions in each group were similar in terms of pre- operative volume (15.12 vs 12.9cm3, p=0.497) and neurovascular invasion (optic canal invasion 48.26 vs 41.37%, p=0.664; ICA 44.81 vs 31.03%, p=0.797). There were no significant differences in visual outcomes between both approaches; TCA presented an improvement of 5.18 points in visual fields (p=0.140), whereas EEA had an improvement of 17.39 points in visual acuity (p=0.114). EEA seems to offer greater improvement in visual acuity than TCA. However, the ideal approach should be individualized; taking into account the tumor's volume and invasiveness, as well as the patient's visual complaints.

Ocular motor and vestibular dysfunction in central nervous system lymphoma.

Diagnosis of lymphoma involving the central nervous system (CNS) is challenging. This study aimed to explore the abnormal vestibular and ocular motor findings in CNS lymphoma. A retrospective search of the medical records identified 30 patients with CNS lymphoma presenting ocular motor and vestibular abnormalities from four neurology clinics of university hospitals in South Korea (22 men, age range 14-81 years, mean 60.6 ± 15.2). The demographic and clinical features and the results of laboratory, radiological and pathological evaluation were analyzed. Patients presented with diplopia (13/30, 43%), vestibular symptoms (15/30, 50%) or both (2/30, 7%). In 15 patients with diplopia, abnormal ocular motor findings included ocular motor nerve palsy (n = 10, 67%), internuclear ophthalmoplegia (n = 2, 13%), external ophthalmoplegia (n = 2, 13%) and exophoria (n = 1, 7%). The vestibular abnormalities were isolated in 14 (82%) of 17 patients with vestibular symptoms and included combined unilateral peripheral and central vestibulopathy in three from lesions involving the vestibular nuclei. CNS lymphoma involved the brainstem (53%), cerebellum (33%), leptomeninges (30%), deep gray nuclei (23%) or cranial nerves (17%). Two patients showed the "double-panda" sign by involving the midbrain. This study expands the clinical and radiological spectra of CNS lymphoma. Neuro-ophthalmological and neuro-otological evaluation may guide the early diagnosis of CNS lymphoma.

Correlation Between Visual Acuity and Foveal Threshold by Automated Perimetry With Size V Stimulus.

Visual acuity has been shown to correlate with foveal threshold as determined by automated perimetry. Although automated perimetry with size V stimulus is commonly used in neuro-ophthalmology practice, the relationship between the visual acuity and the foveal threshold with this larger stimulus is not well known. Retrospective study of patients who had undergone neuro-ophthalmology evaluation and visual field testing with automated perimetry using size V stimulus. Healthy controls were also recruited. Using visual acuity and foveal threshold, Pearson correlation coefficients were calculated, and basic foveal threshold statistics were stratified by visual acuity. Prediction intervals for visual acuities by various foveal threshold were also calculated. A total of 106 unique eyes were included. The final Pearson correlation coefficient between visual acuities was -0.795 for the right eye and -0.578 for the left eye, with a pooled correlation coefficient of -0.751 (P < 0.001). A foveal threshold of at least 34 dB was present in 94.4% of eyes with 20/20 visual acuity, and a foveal threshold of greater than 35 dB was not observed in eyes with visual acuity of 20/40 or worse. Foveal threshold as determined by automated perimetry using size V stimulus has moderate-to-strong correlation with visual acuity in patients undergoing neuro-ophthalmology evaluation.

Planet of the apex

A 63-year-old man with diabetes presented with unilateral ptosis and an exotropia. A diagnosis of isolated diabetic III nerve palsy was made. Subsequent neuro-ophthalmologic evaluation showed multiple cranial nerves involvement consistent with a diagnosis of orbital apex syndrome. Review of past medical history was significant for a previous nasopharyngeal carcinoma, and biopsy of the involved site was consistent with tumor recurrence. This case highlights the importance of correct medical history taking and anatomo-clinical correlation in neuro-ophthalmology.

Diplopía binocular: estudio retrospectivo de 204 casos

ObjectivesTo describe the prevalence and form of onset of different causes of binocular diplopia in our setting. MethodsWe conducted a single-centre, cross-sectional, retrospective study reviewing the medical records of all patients visiting a tertiary-level centre between May 2019 and June 2021 with binocular diplopia as the main symptom. All patients underwent a complete neuro-ophthalmological evaluation and complementary tests for the aetiological diagnosis of diplopia. Data were collected on demographic variables, ocular deviation pattern, complementary test results, and diagnosis. ResultsA total of 204 patients with binocular diplopia were identified during the study period. The most frequent causes of diplopia overall were fourth nerve palsy (19.12%), sixth nerve palsy (14.71%), decompensated strabismus (14.22%), sagging eye syndrome (12.25%), third nerve palsy (10.78%), myasthenia (7.35%), supranuclear disorders (6.37%), and myopic esotropia (5.88%). Presentation was acute (≤2 weeks progression) in 51% of cases. The most frequent causes of acute-onset diplopia were sixth nerve palsy (27.88%), third nerve palsy (21.15%), fourth nerve palsy (19.23%), supranuclear disorders (12.5%), and decompensated strabismus (6.73%). The most frequent causes of subacute/chronic presentation (more than 2 weeks) were decompensated strabismus (22%), sagging eye syndrome (22%), fourth nerve palsy (19%), myopic esotropia (12%), and myasthenia (11%). ConclusionsThe most frequent aetiology of diplopia in our environment was fourth nerve palsy, followed by sixth nerve palsy, decompensated strabismus, and sagging eye syndrome. Knowing the frequency of each cause of diplopia can help prioritise neuroimaging studies in each case.

Opportunities and challenges in the diagnosis and treatment of optic chiasm lesions: a clinical and research perspective

The optic chiasm is a critical component of the visual pathway, and lesions in the pituitary and sellar regions can cause irreversible damage to a patient's visual function, resulting in a significant decrease in their quality of life. As a result, neuro-ophthalmology evaluation is a crucial part of the multidisciplinary treatment of pituitary diseases. However, due to the significant variation in the anatomical structure of the optic chiasm and the sellar region, as well as the complexity of the injury mechanism, chiasm injury can result in diverse manifestations and severity levels, which can make it difficult to correlate with anatomical parameters. In recent years, research has increasingly focused on the early recognition of optic chiasm compression, the prediction of visual function after intervention, and the long-term neurodegenerative effects, while optical coherence tomography (OCT), electrophysiological examinations, and functional magnetic resonance imaging are currently the most commonly used methods for evaluating sellar region lesions. However, the role of these methods, represented by OCT, in clinical diagnosis and treatment, still lacks high-level clinical evidence support, and the evaluation and prediction of optic chiasm function remain key areas for further study. In addition to compression lesions, lesions such as inflammation, infiltration, and demyelination in the sellar region, caused by systemic multi-system diseases, can also lead to visual function damage and require recognition in clinical practice.

Optic nerve T2-hyperintensity: a nonspecific marker of optic nerve damage (P2-9.004)

<h3>Objective:</h3> To determine the significance of optic nerve (ON) T2-hyperintensity and optic atrophy on MRI. <h3>Background:</h3> The best imaging modality to investigate optic neuropathy etiologies is an orbital MRI. In acute optic neuritis, ON T2-hyperintensity and contrast enhancement (CE) are seen in 77% and 90% of patients, respectively. However, in chronic optic neuropathies, ON T2-hyperintensity and atrophy are typically seen. Interestingly, isolated ON T2-hyperintensity is often presumed to be due to optic neuritis, prompting unnecessary investigations and frequent referrals to neuro-ophthalmology. <h3>Design/Methods:</h3> Retrospective study of consecutive patients who underwent brain/orbit MRI with/without contrast at our institution between 07/01/2019–06/30/2022. Patients with ON T2-hyperintensity or atrophy were included. Medical records were reviewed to determine the etiology, if any, of the T2-hyperintensity and/or atrophy. <h3>Results:</h3> 491 patients were included (mean age 52±17 years; 57% men). 381/724 ONs had <b>T2-hyperintensity </b><b><i>without</i></b><b> atrophy</b>; diagnoses: inflammatory (100), compressive (94), glaucoma (19), multifactorial (47), other (69), unspecified (15), normal (37). 219/724 ONs had <b>T2-hyperintensity </b><b><i>and</i></b><b> atrophy</b>; diagnoses: inflammatory (36), compressive (50), glaucoma (30), multifactorial (39), other (36), unspecified (12), normal (16). 124/724 ONs had <b>atrophy </b><b><i>without</i></b><b> T2-hyperintensity</b>; diagnoses: inflammatory (13), compressive (12), glaucoma (32), multifactorial (21), other (24), unspecified (7), normal (15). 68/724 eyes with T2-hyperintensity or atrophy had a normal ophthalmologic examination. <h3>Conclusions:</h3> ON T2-hyperintensity on MRI is a non-specific finding; it can be seen in various acute and chronic optic neuropathies. ON T2-hyperintensity <b>with</b> ON atrophy <b>without</b> associated CE can occur secondary to chronic optic neuropathies, such as compressive and glaucomatous optic neuropathies, and is not highly associated with inflammatory optic neuropathies. Therefore, these radiological findings should not automatically prompt an urgent neuro-ophthalmology evaluation or further work-up for optic neuritis, such as a lumbar puncture. However, in the absence of a known cause of the optic neuropathy, an optic neuropathy work-up should be performed. <b>Disclosure:</b> Dr. Labella Alvarez has received research support from Fundación Alfonso Martín Escudero. Dr. Mosleh has nothing to disclose. Dr. Bouthour has nothing to disclose. Amit Saindane has nothing to disclose. Dr. Dattilo has nothing to disclose. Dr. Newman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for GenSight. Dr. Newman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Chiesi. Dr. Newman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Neurophoenix. Dr. Newman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Stoke. Dr. Newman has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Avidity. Dr. Newman has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Neurodiem. The institution of Dr. Newman has received research support from GenSight. The institution of Dr. Newman has received research support from Chiesi/Santhera. The institution of Dr. Newman has received research support from NINDS/NIH. Dr. Newman has received publishing royalties from a publication relating to health care. Dr. Newman has received publishing royalties from a publication relating to health care. Dr. Newman has received publishing royalties from a publication relating to health care. Dr. Biousse has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gensights Biologic. Dr. Biousse has received personal compensation in the range of $0-$499 for serving as a Consultant for Neurophoenix. The institution of Dr. Biousse has received research support from NIH. Dr. Biousse has received publishing royalties from a publication relating to health care. Dr. Biousse has received publishing royalties from a publication relating to health care.

Surgical Management of Vision Loss in Cerebral Venous Thrombosis: Case Series from a Tertiary Care Stroke Center.

Cerebral venous thrombosis (CVT) presenting as vision loss is uncommon. Raised intracranial tension in CVT is proposed as one of the mechanisms (13.2%). There are still unknown underlying mechanisms to explain vision loss in CVT. The safety and outcome of the surgery (optic nerve sheath fenestration [ONSF] or theco-peritoneal shunt [TPS]) to reduce intracranial hypertension and prevent vision loss has not been studied. A retrospective case record review of CVT patients with impending vision loss who underwent ONSF/TPS from 2007 to 2019 was performed from the stroke registry. All patients had formal neuro-ophthalmological evaluation and documentation of visual acuity, supplemented by visual field assessments by perimetry in a subset of patients. Safety and outcomes were assessed based on vision improvement and adverse effects after the surgery. Among approximately 1400 patients with CVT admitted in the stroke ward over 12 years, surgery for rescuing vision was done in 18. Among these, the males were 6, and the females were 12. The mean age of presentation was 24 (range 18-52 years). All of them had headaches and progressive blurring of vision with papilledema. The number of patients who underwent TPS was 13, ONSF was 1, and both were 4. In the TPS group (26 eyes), vision improved in 15 eyes (57.7%), remained status-quo in 8 eyes (30.7%), and worsened in 3 eyes (11.5%). Four patients underwent both surgeries; three eyes improved, two remained status quo, and three worsened. One patient underwent ONSF, and his vision remained status quo (no perception of light). Three patients (17.6%) of the TPS group had minor complications (low-pressure headache, subdural hygroma), and five (29.4%) had major complications like subdural hemorrhage, abdominal wound infection, and meningitis. In patients with CVT, adequate vision monitoring is mandatory. Shunt surgeries (especially TPS) may help in stabilizing/improving vision in CVT patients with impending vision loss, despite adequate anti-edema measures (53.8% improved). Early diagnosis and precise decisions in referring for surgery are crucial.

Sellar masses: diagnosis and treatment.

Sellar mases can cause a variety of neuro-ophthalmic manifestations, including compressive optic neuropathy, chiasmal syndrome, and ophthalmoplegia due to cranial nerve palsy. Diagnosis involves a thorough history, neuro-ophthalmic examination, and ancillary tests and investigations. Visual field testing is critical in diagnosing and localizing the lesion and determining the extent of visual field loss. Appropriate neuro-imaging is essential in characterizing and localizing the lesion. Neuro-ophthalmologic assessment include meticulous clinical examination and ancillary tests including,visual field testing, which is useful in localizing the lesion, and optical coherence tomography, which is helpful in assessing the degree of axonal and neuronal loss and predicting the visual outcome. Treatment requires a multidisciplinary approach by different specialties, including radiologists, neuro-ophthalmologists, and neurosurgeons. The two primary treatment modalities for these tumors are surgery and radiation therapy. We review the main types of sellar lesions, their neuro-ophthalmologic evaluation, and treatment options.

Bi-nasal sectors of ganglion cells complex and visual evoked potential amplitudes as biomarkers in pituitary macroadenoma management.

The study aimed to evaluate the retinal ganglion cell structure using optical coherence tomography and the visual pathway function employing visual evoked potentials in the diagnosis and monitoring of patients with pituitary macroadenoma. A descriptive, cross-sectional, and longitudinal study (3 and 12 months follow-up) was conducted on forty-two patients. Thirty-five age-matched healthy controls were used in the cross-sectional one. Full neuro-ophthalmological evaluation (structural and functional) was carried out including global and segmented retinal nerve fiber layer/ganglion cell complex analysis and amplitude and latency of P100 component in the electrophysiology. Statistical data analysis was conducted with R version 3.6.3 and Python version 3.8. Associations were evaluated using Spearman's correlations. Amplitude sensitivities were 0.999, and bi-nasal sectors of ganglion cell complex thickness specificities were 0.999. This structural parameter had the highest diagnostic value (area under curve = 0.923). Significant associations were found between bi-nasal sectors with amplitude at 12' (rho > 0.7, p < 0.01) and median deviation of the visual field (rho > 0.5, p < 0.01) at 3 months. Pre-surgical values of bi-nasal sectors and amplitude can predict post-surgically median deviation and amplitude (Oz, 12') at 3 months with r 2 > 0.5. Bi-nasal sectors of ganglion cell complex and visual evoked potentials P100 amplitude are efficient biomarkers of visual pathway damage for pituitary macroadenoma patients' management. Pre-surgical values of the bi-nasal sector and visual evoked potentials' amplitude could help to predict the restoration of parvocellular pathway traffic after decompression.

UNUSUAL CAUSE OF PERIORBITAL ANGIOEDEMA.

<h3>Introduction</h3> We present a case of eyelid edema initially thought to be due to allergic conjunctivitis, angioedema and periorbital cellulitis that was eventually discovered to be due to Immunoglobulin G4-related disease (IgG4-RD). <h3>Case Description</h3> Our patient was a 57 year-old female with asthma and allergic rhinosinusitis, who presented to clinic with right eyelid edema, injected conjunctiva, and pruritus. She had a history of left eyelid swelling attributed to meibomian gland dysfunction. Initially we treated her for allergic conjunctivitis. A year later, she was treated with clindamycin for periorbital cellulitis, and the year after that, treated for angioedema by the emergency department. Soon after, she developed left eyelid swelling and thick eye discharge. Neuro-ophthalmology evaluation was concerning for polyclonal lymphoid proliferation and sclerosing orbital pseudotumor. Serum IgG4 was elevated and a lacrimal gland biopsy revealed reactive lymphoid hyperplasia compatible with IgG4-RD. She was treated with prednisone for several weeks with good response. <h3>Discussion</h3> IgG4-RD is an uncommon medical condition that can present similarly to more common diseases and can be challenging to diagnose.

Interprofessional Electronic Consultations for the Diagnosis and Management of Neuro-Ophthalmic Conditions.

Interprofessional electronic consultation (eConsult) is a telemedicine modality in which consulting providers review outside records and provide recommendations without in-person consultation. The purpose of this study was to describe the utilization of eConsults in the management of neuro-ophthalmic conditions. Retrospective cohort study of all patients who received an eConsult for a neuro-ophthalmic condition at a single quaternary referral center from 2018 to 2020. Main outcome measures included proportion of eConsults in which sufficient data were provided to the neuro-ophthalmologist to generate a definitive management decision, proportion of patients for whom an in-person neuro-ophthalmology evaluation was recommended, and the eConsult's impact on patient care. Eighty eConsults were conducted on 78 patients during the 3-year study period. Forty-eight (60.0%) subjects were female, mean age was 54 years, and 65 (81.3%) were White. The median time from eConsult request to completion was 4 days (range: 0-34 days). The most frequent eConsult questions were vision/visual field disturbances in 28 (35.0%) cases, optic neuropathies in 22 (27.5%), and optic disc edema in 17 (21.3%). At the time of eConsult, sufficient prior information was provided in 35 (43.8%) cases for the neuro-ophthalmologist to provide a definitive management decision. In 45 (56.3%) eConsults, further diagnostic testing was recommended. In-person neuro-ophthalmology consultation was recommended in 24 (30.0%) cases. Sixty-one (76.3%) eConsults provided diagnostic and/or treatment direction, and 12 (15.0%) provided reassurance. eConsults increase access to timely neuro-ophthalmic care and provide diagnostic and treatment direction to non-neuro-ophthalmology providers when sufficient information is provided at the time of eConsult.

Diagnostic Error in Neuro-ophthalmology: Avenues to Improve.

Purpose of ReviewTo highlight potential avenues to reduce preventable diagnostic error of neuro-ophthalmic conditions and avoid patient harm.Recent FindingsRecent 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.SummaryDiagnostic 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.

Neuro-ophthalmological evaluation including optical coherence tomography surrounding venous sinus stenting in idiopathic intracranial hypertension with papilledema: a case series

Venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) has been demonstrated to achieve significant symptom improvement while harboring a low periinterventional morbidity profile. Comprehensive neuro-ophthalmological monitoring represents a cornerstone of disease monitoring. The value of optical coherence tomography (OCT) requires further exploration. Patients with IIH and papilledema who underwent VSS between 04/2018 and 02/2021 were retrospectively reviewed. Clinical and radiological were analyzed. Neuro-ophthalmological data included visual acuity, visual fields, fundoscopy categorized via Frisén scale, and OCT obtained retinal nerve fiber layer (RNFL) thickness were analyzed. Of 39 IIH patients who underwent cerebral angiography with transverse-sigmoid sinus pressure evaluation, 18 patients with IIH and papilledema underwent 21 transverse-sigmoid sinus stenting (TSST) procedures. After TSST, manometry showed a significant reduction of maximum transverse sinus pressures and trans-stenotic gradient pressures (p = 0.005 and p < 0.001, respectively). Chronic headaches, visual disturbance resolved and pulsatile tinnitus improved significantly. Visual fields remained similar, while papilledema Frisén scales and visual acuity significantly improved. The OCT calculated RNFL thickness significantly decreased in all patients. Stratification according to a minimal-low degree (Frisén 1-2) and moderate-marked degree (Frisén 3-4) papilledema demonstrated a significant reduction of RNFL thickness in both groups. Venous sinus stenting provides favorable clinical and neuro-ophthalmological outcomes. This study demonstrates that neuro-ophthalmologic testing augmented with OCT evaluation provides objective data that can be used as a biomarker for treatment success for managing patients with different extents of papilledema and may inform patient management.

Multimodal evaluation of the melanopsin retinal ganglion cells system in relation to circadian rhythms in Alzheimer’s disease and aging

AbstractBackgroundMelanopsin retinal ganglion cells (mRGCs) are deputed to circadian photoentrainment and pupillary light reflex (PLR) regulations. Circadian dysfunction is reported in Alzheimer’s disease (AD) and contributes to dementia. Single‐Nucleotide‐Polymorphisms (SNPs) in clock genes have been associated to AD.MethodWe included 29 mild‐moderate AD and 26 matched controls performing neuroophthalmological evaluation including optical coherence tomography (OCT), actigraphic recordings of rest‐activity rhythms, chromatic pupillometry and brain functional MRI (fMRI) with light stimulation. 84 clock genes were analyzed by NGS and relevant SNPs validated in a larger cohort of AD (n=449) and controls (n=326).ResultIn AD disease duration was 3.9±2.8 years and MMSEc score 20.2±4.2. OCT showed a significant reduction of the infero‐temporal GCL thickness (p=0.036) in AD. Actigraphy did not disclose significant differences for circadian parameters (IS, IV, RA). However, a subgroup of “circadian‐impaired” AD was evident, and most of circadian parameters declined with aging. Pupillometry revealed a significant reduction of PLR peak amplitude in the rod protocol (p=0.006) significantly correlating with aging in AD. Brain fMRI documented the absence of significant responses in AD with sustained blue light stimulation at difference with controls. Genetic analysis in extended AD and control cohorts showed a significant association of the rs30127178 SNP in PER1 gene with AD.ConclusionThese results demonstrate, by innovative multi‐modal approach, that mRGC system in AD is affected by neurodegeneration. This can be envisaged as a possible biomarker also for conversion from MCI to AD with potential implication for light therapy as a counteracting measure for dementia.

Acute optic neuritis: What do complementary tests add to diagnosis?

IntroductionOptic neuritis (ON) is the inflammation of the optic nerve due in many cases, to a pathological immune response. Since its symptoms can be subtle, diagnosis is sometimes challenging. The value of complementary tests for diagnosis and prognosis of ON was demonstrated in retrospective analysis, but their utility in the acute period of ON has been scarcely studied. The aim of this study is to determine the usefulness of clinical assessment, optical coherence tomography (OCT), visual evoked potentials (VEP) and orbit magnetic resonance imaging (MRI) for making diagnosis and prognosis of acute ON (AON). Materials and methodsA cross-sectional study was conducted including patients with ON within 90 days of symptom onset. A complete neuro-ophthalmological evaluation, OCT, VEP and MRI were carried out, determining in each case its sensitivity, specificity and predictive values in the diagnosis of ON and the assessment of its severity. Results75 eyes of 34 patients with ON were included. Regarding diagnosis, low contrast visual acuity (LCVA) displayed the highest sensitivity (100%), being superior than the sensitivity of all complementary tools, always below 80%. Orbit MRI abnormal findings has a Specificity of 100% to confirm diagnosis. Regarding severity assessment and prognosis, Ganglion cell +inner plexiform layer (GCIP) thickness, but not retinal nerve fibre layer (RNFL), correlates significantly with patients’ visual acuity (VA) (p < 0.05). Furthermore, both P100latency and VEP's amplitude showed to be significantly associated with VA (p < 0.05) in the acute period. The combination of two predictors (measurement of RNFL and GCIP) are capable of explaining 60% of the variation of the patient's visual acuity, with statistical significance (p = 0.02) ConclusionsIn depth neuro-ophthalmological assessment during the acute phase of ON, including contrast sensitivity measurement, proved to be superior to complementary tests for diagnosis, surpassing the performance of OCT and VEP. However, these tools can add to prognosis, as GCIP thickness and VEP's amplitude correlate with disease severity and its findings could encourage prompt aggressive treatments in AON.

What are Your Eyes Revealing? The Contemporary Bedside Neuro-Ophthalmological Examination.

The neuro-ophthalmological evaluation is done to assess the integrity of cranial nerves II, III, IV, VI. This work does not intend to substitute the examination in a fully equipped ophthalmology suite. The aim of this manuscript is to describe bedside examination of the eye by using simple apparatus that are easily available. This work incorporates the usage of smartphone application, as smartphone is deemed an accessory in almost every resident’s pocket during review of patients as a consult in the emergency department. However, the essence of traditional physical examination remains the fundamental. Understanding of neuroanatomy and neurophysiology of the eye and the central nervous system can enhance the clinician’s performance at the bedside.

Chromatic Pupillometry in Isolated Rapid Eye Movement Sleep Behavior Disorder.

BackgroundMelanopsin retinal ganglion cell (mRGC)‐mediated pupillary light reflex (PLR) abnormalities have been documented in several neurodegenerative disorders including Parkinson's disease. Overall, isolated rapid eye movement (REM) sleep behavior disorder (iRBD) represents the strongest prodromal risk factor for impending α‐synucleinopathies.ObjectivesTo quantitatively compare PLR and mRGC‐mediated contribution to PLR in 16 iRBD patients and 16 healthy controls.MethodsiRBD and controls underwent extensive neuro‐ophthalmological evaluation and chromatic pupillometry. In iRBD, PLR metrics were correlated with clinical variables and with additional biomarkers including REM atonia index (RAI), DaTscan, and presence of phosphorylated‐α‐synuclein (p‐α‐syn) deposition in skin biopsy.ResultsWe documented higher baseline pupil diameter and decreased rod‐transient PLR amplitude in iRBD patients compared to controls. PLR rod‐contribution correlated with RAI. Moreover, only iRBD patients with evidence of p‐α‐syn deposition at skin biopsy showed reduced PLR amplitude compared to controls.ConclusionThe observed PLR abnormalities in iRBD might be considered as potential biomarkers for the risk stratification of phenoconversion of the disease. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society

Neuro-ophthalmologic outcomes of standard versus hypo-fractionated stereotactic radiotherapy of AVPM

BackgroundMost anterior visual pathway meningiomas (AVPM) are benign and slow-growing, but these tumors may affect visual functions, including visual acuity (VA) and visual field (VF). Due to location, most are treated non-surgically by fractionated stereotactic radiotherapy (FSRT), aiming to prevent tumor progression and visual functions deterioration. Unfortunately, FSRT in itself may affect visual functions. The current preferred treatment regimen (in terms of safety and effectiveness) is undetermined. While most cases are treated with conventional fractionation (cFSRT)—50.4–54 Gy in 28–30 fractions of 1.8–2 Gy, advances in technology have allowed shortening of total treatment length to hypofractionation (hSRT)—25-27 Gy in 3–5 fractions of 5–9 Gy. Our aim was to evaluate the association of radiotherapy regimen for treating AVPM (cFSRT vs. hSRT) with visual function outcomes (VA, VF) at the last neuro-ophthalmologic evaluation.MethodsWe conducted a retrospective cohort study of AVPM cases treated at Sheba Medical Center during 2004–2015. We compared cFSRT and hSRT regimens regarding visual function (VA, VF) outcomes at the last neuro-ophthalmologic evaluation. VA was determined by the logarithm of the minimum angle of resolution (LogMAR). VF was determined by the mean deviation (MD). A clinically relevant change in VA was defined as 0.2 LogMAR.Results48 patients (13 receiving hSRT, 35 receiving cFSRT) were included, with a median follow-up of 55 months. No significant difference was evident regarding LogMAR or MD of involved eyes at the last evaluation. Six (17%) patients in the cFSRT group experienced clinically relevant VA deterioration in the involved eye, compared with six (46%) in hSRT (p = 0.06).ConclusionOur findings, using comprehensive and meticulous investigation of visual outcomes, suggest that hSRT may be associated with higher risk for VA and VF deterioration in AVPM especially in ONSM. We recommend the use of cFSRT for ONSM.