What's new in NMOSD and MOGAD?

In this mini-review, we focus on novelties in the field of neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD). We first describe the proposed criteria for MOGAD and evaluate their impact and potential limitations, with a highlight on the subgroups of patients tested MOG-antibody positive only in the cerebrospinal fluid. We then propose a brief state of the art on the current knowledge on the so-call "double seronegative" NMOSD group, regarding nosology, clinical, biological and imaging features and the unmet need in this field. The last part is dedicating to the present and future of acute treatment in NMSOD and MOGAD.

Longitudinal Optical Coherence Tomography Imaging Reveals Hyperreflective Foci Characteristics in Relapsing-Remitting Multiple Sclerosis Patients.

Background/Objectives: Retinal hyperreflective foci, 25-50 µm in diameter, that can be imaged by noninvasive optical coherence tomography (OCT) may represent microglial activity related to inflammation. This study aimed to detect hyperreflective foci in the OCT-hyporeflective avascular outer nuclear layer of the retina in relapsing-remitting MS (RRMS) patients without ongoing eye or optic nerve disease. Methods: A cohort of 13 RRMS patients (8 eyes with and 18 eyes without prior optic neuritis) underwent retinal OCT at baseline, after 1 month, after 6 months, and then every 6 months for 3 years. The data were compared with single-examination data from 106 eyes in 53 age-matched healthy subjects. Results: The prevalence of hyperreflective foci at baseline was higher in RRMS patients than in healthy subjects (46.2% vs. 1.8%, p < 0.005). Patients with optic neuritis had much more foci than those without (p < 0.001). Hyperreflective foci recurred in 23.1% of RRMS patients, bilaterally in one with prior optic neuritis and unilaterally in two without. Conclusions: Patients with RRMS, notably those with prior optic neuritis, had elevated rates of retinal infiltration in the absence of retinal disease, suggesting that the phenomenon may represent elevated activity of an immune surveillance or housekeeping mechanism rather than retinal disease.

Abnormal dynamic features of spontaneous brain activity and their concordance in neuromyelitis optica spectrum disorder related optic neuritis: A resting-state fMRI study.

OBJECTIVE: Characterizing the neuropathological features of neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) is crucial for understanding its mechanisms. Given the important role of dynamic features in the brain's functional architecture, we aim to investigate the dynamic features of spontaneous brain activity and their concordance using resting-state functional magnetic resonance imaging (rs-fMRI) in NMOSD-ON. METHODS: Fourteen NMOSD-ON patients and 21 healthy controls (HCs) underwent rs-fMRI and ophthalmological examinations. Five dynamic indices depicting different aspects of functional characteristics were calculated using a sliding window method based on rs-fMRI data. Kendall's coefficient was utilized to measure concordance among these indices at each time point. The differences of dynamic features between two groups were evaluated using two-sample t-tests, with correlations explored between altered dynamics and clinical parameters. RESULTS: Compared to HCs, NMOSD-ON patients exhibited significant decreases in dynamic regional homogeneity (dReHo) and dynamic degree centrality (dDC) in visual regions, including bilateral cuneus, lingual gyrus, calcarine sulcus, and occipital gyrus. Conversely, increases were observed in left insula, left thalamus, and bilateral caudate. The concordance of NMOSD-ON patients was significantly lower than HCs. The dReHo of right cuneus negatively correlated with mean deviation of visual field (r = -0.591, p = 0.026) and the dReHo of left cuneus negatively correlated with disease duration (r = -0.588, p = 0.030). CONCLUSION: The evidence suggests that regional dynamic functional alterations involving vision, emotional processing, and cognitive control may provide a new understanding of brain changes in the progression of NMOSD-ON.

Changes in the incidence of optic neuritis before and after COVID-19: A nationwide study 2017-2022.

PURPOSE: To determine the risk of optic neuritis (ON) during non-pharmaceutical interventions (NPI), vaccination and infection phase of coronavirus disease-19 (COVID-19) in comparison to pre-outbreak levels in pediatric and adult populations in South Korea. DESIGN: A nationwide, population-based, retrospective study. PARTICIPANTS: South Korean individuals with a primary diagnosis of ON between January 2017 and December 2022. METHODS: The Korean Health Insurance Review and Assessment database was queried for new diagnoses of ON between January 2017 and December 2022. Data were divided into 4 periods: pre-COVID-19 (2017-2019), NPI (2020), nationwide vaccination (2021) and nationwide infection (2022). The risk of ON development for each period was calculated and compared to pre-COVID-19 levels with 95% confidence intervals (CI) reported. MAIN OUTCOME MEASURES: Incidence rate ratio (IRR) of ON for each period. RESULTS: A total of 7,216 patients (52.7 % females) were included in the study, with 3,770 patients diagnosed with ON pre-COVID-19 (2017-2019), 1,193 patients during NPI, 1,135 patients during the vaccination and 1,118 patients during the infection phases. The annual incidence of ON during NPI (IRR 0.92 (95% CI 0.85-1.00), P=0.043), vaccination (IRR 0.88 (95% CI 0.81-0.95), P=0.001) and infection (IRR 0.86 (95% CI 0.80-0.93), P<0.001) phases significantly decreased compared to pre-COVID-19 levels when adjusted for age and sex. The proportions of diagnosis with multiple sclerosis (MS), neuromyelitis optica (NMO), and acute disseminated encephalomyelitis (ADEM) among patients who developed ON significantly increased in 2021 in comparison to the pre-outbreak levels (9.87% vs. 5.81%; P=0.0002). CONCLUSIONS: The risks of ON development during NPI, vaccination and infection phases of COVID-19 did not increase in comparison to the pre-outbreak levels in general population. However, COVID-19 vaccination may be associated with increased risks of ON associated with diseases such as ADEM, MS and NMOSD.

A rare case of systemic lupus erythematosus-associated neuromyelitis optica spectrum disorder with cystic lesions and dual seropositivity for anti-AQP4 and anti-MOG antibodies.

Key Clinical Message: In patients with SLE, concurrent NMOSD can manifest with optic neuritis and transverse myelitis. AQP-4 antibody positivity confirms the diagnosis. Prompt treatment is critical to manage the acute symptoms and prevent relapses, as highlighted by a young patient's case with optic neuritis and extensive spinal cord lesions. Abstract: Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder of the central nervous system that affects the optic nerve and spinal cord. It is associated with autoantibodies against aquaporin-4 (AQP-4) and/or myelin oligodendrocytes glycoproteins. It is diagnosed based on clinical, radiological, and serological criteria, and treated with immunosuppressants in the acute phase. Long-term immunosuppression is essential to prevent potential relapses. In this case report, we present the case of a 19-year-old female patient with systemic lupus erythematosus (SLE), who presented with blurriness and loss of vision in her left eye. Optical coherence tomography was normal, but a gadolinium-enhanced cervico-dorsal MRI showed multiple lesions extending from the brainstem to the C7-T1 junction suggestive of longitudinally extensive transverse myelitis (LETM), the largest of which was a cystic lesion at the cervico-spinal junction. A contrast injection also revealed left optic neuritis. Cerebrospinal fluid analysis showed elevated IgG and red blood cell count, but no oligoclonal bands. The patient tested positive for AQP-4 autoantibodies, confirming the diagnosis of NMOSD. Treatment with intravenous methylprednisolone led to partial improvement, but the patient experienced a relapse with severe neurological symptoms, including tetraplegia and bladder and bowel dysfunction. This case illustrates the importance of considering NMOSD in the differential diagnosis of patients with SLE who present with optic neuritis and/or myelitis, especially when MRI findings are suggestive of LETM. Early diagnosis and adherence to treatment are crucial to prevent further relapses and deleterious sequelae.

Radiological features in pediatric myelin oligodendrocyte glycoprotein antibody-associated disease-diagnostic criteria and lesion dynamics.

The spectrum of acquired pediatric demyelinating syndromes has been expanding over the past few years, to include myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD), as a distinct neuroimmune entity, in addition to pediatric-onset multiple sclerosis (POMS) and aquaporin 4-IgG-seropositive neuromyelitis optica spectrum disorder (AQP4+NMOSD). The 2023 MOGAD diagnostic criteria require supporting clinical or magnetic resonance imaging (MRI) features in patients with low positive myelin oligodendrocyte glycoprotein IgG titers or when the titers are not available, highlighting the diagnostic role of imaging in MOGAD. In this review, we summarize the key diagnostic features in MOGAD, in comparison to POMS and AQP4+NMOSD. We describe the lesion dynamics both during attack and over time. Finally, we propose a guideline on timing of imaging in clinical practice.

Rapid differentiation of MOGAD and MS after a single optic neuritis.

BACKGROUND: Optic neuritis (ON) is a common manifestation of multiple sclerosis (MS) and myelin-oligodendrocyte-glycoprotein IgG-associated disease (MOGAD). This study evaluated the applicability of optical coherence tomography (OCT) for differentiating between both diseases in two independent cohorts. METHODS: One hundred sixty two patients from seven sites underwent standard OCT and high-contrast visual acuity (HCVA) testing at least 6 months after first ON. Of these, 100 patients (32 MOGAD, 68 MS) comprised the primary investigational cohort, while 62 patients (31 MOGAD, 31 MS) formed a validation cohort. A composite score distinguishing between MOGAD and MS was developed using multivariate logistic regression. RESULTS: Bilateral simultaneous ON occurred more frequently in MOGAD compared to MS (46.9 vs. 11.8%, p < 0.001). OCT revealed more peripapillary retinal nerve fiber layer (pRNFL) atrophy in all segments in MOGAD compared to predominantly temporal pRNFL atrophy in MS (p < 0.001). HCVA was better preserved in MS (p = 0.007). pRNFL thickness in all except for temporal segments was suitable for differentiating MOGAD and MS. Simultaneous bilateral ON and critical atrophy in nasal (< 58.5 µm) and temporal superior (< 105.5 µm) segments were included into the composite score as three independent predictors for MOGAD. The composite score distinguished MOGAD from MS with 75% sensitivity and 90% specificity in the investigational cohort, and 68% sensitivity and 87% specificity in the validation cohort. CONCLUSION: Following a single ON-episode, MOGAD exhibits more pronounced global pRNFL atrophy and lower visual acuity after ON compared to MS. The introduced OCT-based composite score enabled differentiation between the two entities across both cohorts.

Comparison between contrast-enhanced fat-suppressed 3D FLAIR brain MR images and T2-weighted orbital MR images at 3 Tesla for the diagnosis of acute optic neuritis.

PURPOSE: The purpose of this study was to compare the capabilities of contrast-enhanced fat-suppressed (CE FS) three-dimensional fluid-attenuated inversion recovery (3D FLAIR) brain magnetic resonance imaging (MRI) with those of coronal T2-weighted orbital MRI obtained at 3 Tesla for the diagnosis of optic neuritis (ON). MATERIALS AND METHODS: Patients who presented to our center with acute visual loss and underwent MRI examination of the orbits and the brain between November 2014 and February 2020 were retrospectively included. Three radiologists independently and blindly analyzed CE FS 3D FLAIR and coronal T2-weighted images. Disagreements in image interpretation were resolved by consensus with an independent neuroradiologist who was not involved in the initial reading sessions. The primary adjudication criterion for the diagnosis of ON was the presence of an optic nerve hypersignal. Sensitivity, specificity, and accuracy of CE 3D FLAIR brain images were compared with those of coronal T2-weighted orbital images using McNemar test. Artifacts were classified into three categories and compared between the two image sets. RESULTS: A total of 1023 patients were included. There were 638 women and 385 men with a mean age of 42 ± 18.3 (standard deviation) years (age range: 6-92 years). Optic nerve hyperintensities were identified in 375/400 (94%) patients with ON using both 3D FLAIR and coronal T2-weighted images. Sensitivity, specificity, and accuracy of both sequences were 94% (95% CI: 91.3-96.1), 79% (95% CI: 75.5-82.2), and 89% (95% CI: 86.8-90.7), respectively. Optic disc hypersignal was detected in 120/400 patients (30%) using 3D FLAIR compared to 3/400 (0.75%) using coronal T2-weighted images (P < 0.001). Optic radiation hypersignal was observed in 2/400 (0.5%) patients using 3D FLAIR images. Significantly more artifacts (moderate or severe) were observed on coronal T2-weighted images (801/1023; 78%) by comparison with 3D FLAIR images (448/1023; 44%) (P < 0.001). CONCLUSION: The performance of 3D FLAIR brain MRI for the diagnosis of ON is not different from that of coronal T2-weighted orbital MRI and its use for optic nerve analysis may be beneficial.

Detecting optic nerve lesions in multiple sclerosis patients with a 1,5 T MRI: Evaluation of a 3D DIR sequence compared to a 2D STIR sequence.

OBJECTIVES: Optic neuritis is a common clinical presentation in patients suffering from multiple sclerosis (MS). Even though optic neuritis is not part of the MS diagnostic criteria, the diagnosis and consideration of differential diagnoses are important in clinical routine. For the evaluation of the optic nerves with MRI, T2-weighted images with fat suppression, known as short tau inversion recovery sequences (STIR), are often used. Besides that, double inversion recovery (DIR) sequences are being used increasingly in MS patients, especially to determine cortical lesions. The Aim of this study was to evaluate the 3D-DIR for the detection of lesions in the optic nerves in MS patients. METHODS: MR examinations of 45 MS-patients containing both STIR and DIR images were independently assessed by two neuroradiologic experienced radiologists, blinded to clinical data. A third neuroradiologic, an experienced radiologist, evaluated the images together, also considering clinical data. These results were considered ground truth and statistically compared to the results of the single readings. To objectify our findings, ROI measurements of affected and unaffected optic nerve segments were made, and a contrast ratio (CR) was calculated. RESULT: DIR images are statistically equivalent to STIR images concerning the detection of lesions in the optic nerve (p < 0.001). The sensitivity of DIR images (84.7 %) and STIR images (77 %), as well as the specificity (92.2 % and 91.2 %), are comparable. The interrater reliability was substantial for both sequences (κ = 0,73) as well as separated for the STIR images (κ = 0.744) and the DIR images (κ = 0.707). The objective analysis revealed significantly higher CRs in DIR images (p < 0.001). CONCLUSION: 3D DIR images showed similar sensitivity and specificity for detecting optic nerve lesions in comparison to dedicated 2D images of the optic nerve. When 3D DIR images are part of the routine scan protocol for evaluating MS patients, additional 2D imaging of the optic nerve is no longer necessary.

Optic neuritis following COVID-19 vaccination: Case series and review of the literature.

OBJECTIVE: To review cases of optic neuritis after COVID-19 vaccination and add similar cases to the literature. METHODS: Thorough PubMed and Scopus searches were conducted, and data from studies describing optic neuritis after COVID-19 vaccination were extracted, tabulated, pooled, and reviewed. RESULTS: We present 6 cases of optic neuritis following COVID-19 vaccination. Our literature search yielded 48 similar cases. All 54 cases were divided into 3 groups with respect to their serostatus: (1) double-seronegative or unknown serostatus optic neuritis cases, (2) myelin oligodendrocyte glycoprotein (MOG)-associated optic neuritis cases, and (3) aquaporin-4-associated optic neuritis cases. Data from each group were separately pooled and reviewed. While the most frequent vaccine among the anti-AQP4+ subgroup was BNT162b2 (Pfizer-BioNTech) (2/3), recombinant vaccines, e.g., AZD122 and Ad26.Cov2.s were mostly injected in the other subgroups (23/51). No significant gender inclination was seen among different subgroups. The mean interval from vaccination to symptom onset was less than one month in all subgroups; symptom manifestations mainly occurred after the first dose (28/54). Almost all cases showed improvement after steroid therapy±plasma exchange (52/54). CONCLUSION: Despite having rare side effects such as optic neuritis, vaccination remains our most helpful protection against SARS-CoV-2. Nevertheless, larger studies are needed to ascertain the pathophysiology of such adverse effects. Likewise, the association between COVID-19 vaccination and optic neuritis warrants further investigation.

Successful Maintenance Therapy with Intravenous Immunoglobulin to Reduce Relapse Attacks and Steroid Dose in a Patient with Refractory Myelin Oligodendrocyte Glycoprotein Antibody-positive Optic Neuritis.

A 25-year-old Japanese man developed visual disturbance with eye pain and was diagnosed with optic neuritis associated with anti-myelin oligodendrocyte glycoprotein antibodies. His symptoms improved temporarily after steroid therapy but chronically relapsed many times after tapering the steroid dose. He became highly steroid-dependent and was referred to our department for reconsideration of the treatment strategy. Maintenance intravenous immunoglobulin (IVIg) therapy successfully decreased the annual recurrence rate from 1.15 to 0.27 times/year and the maintenance dose of oral prednisolone from 35 to 5 mg/day. Maintenance IVIg therapy is a promising option for preventing disease relapse in such cases.

Incidence and Clinical Characteristics of COVID-19 Ophthalmopathy Following the Termination of Dynamic Zero COVID-19 Strategy in China.

Ocular involvement is not uncommon in patients with COVID-19. However, the incidence of COVID-19 ophthalmopathy in COVID-19 patients is still not clear. In this prospective case series study, we recruited 2445 consecutive cases presenting at Neuro-ophthalmology clinic of our Eye Center during the last resurgence of SARS-CoV-2 infection from 8 December 2022 to 15 March 2023 in China, 149 cases were diagnosed as COVID-19 ophthalmopathy, 87 cases were female, with a mean age of 43.2 years, and the mean follow-up time was 15.4 weeks. One hundred and twenty of 149 cases suffered from systemic symptoms mostly manifesting as fever, cough and muscle pain prior to or soon after ocular involvement. The most common COVID-19 ophthalmopathy was optic neuritis (51/149), followed by acute zonal occult outer retinopathy complex disease (31/149), uveitis (17/149), ocular mobility disorder-related (third, fourth, or sixth) cranial nerve neuritis (15/149), anterior ischaemic optic neuropathy (9/149), retinal artery occlusion (8/149), retinal microangiopathy including retinal haemorrhage and cotton wool spot (8/149), viral conjunctivitis (7/149), retinal vein occlusion (3/149), viral keratitis (2/149), ptosis (2/149), and other rare ocular diseases. Except 5 cases with central retinal artery occlusion, other 144 COVID-19 ophthalmopathy cases showed good response to steroid therapy. Our study revealed an incidence of 6.09% for COVID-19 ophthalmopathy in outpatients at our Neuro-ophthalmology clinic during last resurgence of COVID-19 in China, and demonstrated that SARS-CoV-2 infection could induce an initial onset or a relapse of ophthalmic diseases, and that ocular involvement might manifest as the initial or even the only presentation of COVID-19.

Ethambutol-Induced Optic Neuritis and Vision Loss: A Case Report.

Ethambutol is a first-line chemotherapeutic agent, which is commonly used in combination with other drugs for the treatment of tuberculosis. Ethambutol-induced optic neuritis is a serious and rare side effect that is either dose or duration-related and causes progressive painless vision loss, and cecocentral scotomas in the visual field. A rare case of ethambutol-induced optic neuritis was reported in a 52-year-old female who was taking anti-tubercular treatment for pulmonary tuberculosis for five months. She presented with painless diminished vision in both eyes. The patient was diagnosed with a rare case of optic neuritis through various examination methods. Ethambutol was stopped and therapy was continued with oral prednisone, zinc, and vitamin B complex being started along with anti-TB treatment. She showed no marked improvement in visual parameters until the last follow-up. The patient died due to cardiopulmonary arrest as a consequence of pulmonary tuberculosis.

Trastuzumab-induced optic neuritis: "blindness" side effect.

INTRODUCTION: Trastuzumab improved the prognosis of patients with human epidermal growth factor receptor 2 (HER2)+ breast cancer (BC). Here, we present a patient who developed acute vision loss due to optic atrophy in both eyes after trastuzumab. CASE REPORT: A 60-year-old female patient was diagnosed with locally advanced HER2+ BC in January 2021. After four cycles of neoadjuvant anthracycline-based chemotherapy followed by four cycles of docetaxel, trastuzumab, and pertuzumab combined treatment, the patient underwent a right modified radical mastectomy. Three days after the end of the second cycle of adjuvant trastuzumab, she presented with acute vision loss. The patient's visual acuity was 90% in the right eye and 60% in the left eye. The left eye had optic nerve edema and spindle hemorrhages. First, on suspicion of optic neuritis, the patient was given a 1 gram/day pulse steroid for three days. However, optic neuritis was not considered during the follow-up. Metastasis was considered at the exit of the left optic nerve. Trastuzumab was started by making a mutual decision with the patient. Six days after the sixth dose of adjuvant trastuzumab, she presented with almost complete vision loss. MANAGEMENT AND OUTCOME: The patient was diagnosed with optic neuritis, and a pulse steroid was administered. Trastuzumab was permanently discontinued. However, the patient's visual acuity in both eyes remained at 5-10%. DISCUSSION: Vision loss due to optic neuritis is a devastating side effect. Understanding that trastuzumab-induced optic neuritis may develop will help clinicians detect side effects early and manage them more effectively.

Radiological predictors of visual outcome in myelin oligodendrocyte glycoprotein-related optic neuritis.

OBJECTIVE: This study aimed to determine whether magnetic resonance imaging (MRI) biomarkers are associated with visual prognosis in myelin oligodendrocyte protein (MOG)-associated optic neuritis (MOG-ON). DESIGN: Cross-sectional analysis. SUBJECTS: Patients meeting 2023 international diagnostic criteria for MOG antibody-associated disease who were seen for first episodes of MOG-ON at three tertiary neuro-ophthalmology practices between January 2017 and July 2023 were enrolled. Patients who received less than 3 months of neuro-ophthalmic follow-up and did not demonstrate visual recovery (visual acuity [VA] ≥20/20 and visual field mean deviation [VFMD] >-5.0 dB) during this time were excluded. METHODS: Patients received contrast-enhanced, fat-suppressed MRI of the brain and orbits within one month of symptom onset. MAIN OUTCOME MEASURES: The associations between radiological biomarkers and poor VA outcome (<20/40), incomplete VA recovery (<20/20), and poor VFMD outcome (VFMD <-5.0 dB) were assessed using multivariable logistic regression adjusting for time from symptom onset to treatment and nadir VA or VFMD. Radiological biomarkers included length of optic nerve enhancement (below vs. above 25%, 50%, and 75%); degree of orbital, canalicular, and intracranial or chiasmal optic nerve enhancement (mild vs. moderate-severe compared to the lacrimal gland); and absence vs. presence of optic nerve sheath enhancement on baseline T1-weighted MRI. RESULTS: A total of 129 eyes of 92 patients (median [IQR] age 37.0 [20.8-51.3], 65.2% female) were included. Poor VA outcome was seen in 6.2% of cases, incomplete VA recovery in 19.4%, and poor VFMD outcome in 16.9%. Compared to eyes with moderate-severe enhancement, eyes with mild orbital optic nerve enhancement were more likely to have poor VA outcome (OR 8.57; 95% CI [1.85, 51.14], P=0.009), incomplete VA recovery (OR 7.31, 95% CI [2.42, 25.47], P=0.001), and poor VFMD outcome (adjusting for time to treatment: OR 6.81, 95% CI [1.85, 28.98], P=0.005; adjusting for nadir VFMD: OR 11.65, 95% CI [1.60, 240.09], P=0.04). Lack of optic nerve sheath enhancement was additionally associated with incomplete VA recovery (OR 3.86, 95% CI [1.19, 12.85], P=0.02) compared to the presence of enhancement. These associations remained consistent in subgroup logistic regression analysis of MRIs performed before initiation of treatment but were not seen in pairwise analysis of MRIs performed after treatment. CONCLUSIONS: In eyes with first MOG-ON episodes, milder enhancement in the orbital optic nerve is associated with poorer VA and VF recovery. Prospective and mechanistic studies are needed to confirm the prognostic utility of MRI in MOG-ON.

Neuromyelitis optica spectrum disorder: Exploring the diverse clinical manifestations and the need for further exploration.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disorder characterized by inflammatory assaults on the central nervous system (CNS), particularly on the optic nerves and spinal cord. In recent years, a wider range of clinical manifestations of this complex disease have been observed, emphasizing the importance of gaining a more profound understanding beyond optic neuritis (ON) and transverse myelitis (TM). CURRENT KNOWLEDGE: This study explores the many clinical symptoms of NMOSD, including common and uncommon presentations. Distinctive aspects of ON, TM, and diencephalic/brainstem syndromes are examined, highlighting their unique characteristics in contrast to conditions such as multiple sclerosis. We also discuss extra-CNS involvement, such as unusual signs, including muscle involvement, retinal injury, auditory impairment, and rhinological symptoms. AIMS AND OBJECTIVES: Our study intends to highlight the wide range and complexity of NMOSD presentations, emphasizing the significance of identifying unusual symptoms for precise diagnosis and prompt management. The specific processes that contribute to the varied clinical presentation of NMOSD are not well understood despite existing information. This emphasizes the necessity for more study to clarify the mechanisms that cause different symptoms and discover new treatment targets for this complex autoimmune disorder. CONCLUSION: It is essential to acknowledge the complex and varied clinical manifestations of NMOSD to enhance diagnosis, treatment, and patient results. By enhancing our comprehension of the fundamental processes and investigating innovative therapeutic approaches, we may aim to enhance the quality of life for persons impacted by this illness.

Differentiating optic neuropathies using laser speckle flowgraphy: Evaluating blood flow patterns in the optic nerve head and peripapillary choroid.

PURPOSE: To compare blood flow (BF) impairment patterns in different optic neuropathies using laser speckle flowgraphy (LSFG). METHODS: This retrospective study enrolled 24 eyes of 24 patients with non-arteritic anterior ischemic optic neuropathy (NAAION), 59 eyes of 59 patients with optic neuritis (ON), 677 eyes of 677 patients with open-angle glaucoma (OAG), and 110 eyes of 110 controls. The patient backgrounds of all groups were compared. Ophthalmologic findings were evaluated, adjusting for age, sex, blood pressure, pulse rate, and underlying systemic diseases with 1:1 optimal propensity score matching. We used LSFG to obtain optic nerve head (ONH) vessel-area mean blur rate (MBR; ONH-MV), ONH tissue-area MBR (ONH-MT), and choroidal MBR. The NAAION and ON groups were compared with the control and OAG groups. RESULTS: Best-corrected visual acuity was worse in the NAAION, ON, and OAG groups than in controls (p < 0.001). Circumpapillary retinal nerve fibre layer thickness was higher in the NAAION and ON groups and lower in the OAG group than in controls (p < 0.001). Compared to controls, the NAAION and OAG groups had significantly lower ONH-MV, ONH-MT, and choroidal MBR (p < 0.05). Additionally, the NAAION group had lower ONH-MV and choroidal MBR than the OAG group (p = 0.003 and p < 0.001, respectively) but no difference in ONH-MT (p = 0.857). The ON group had significantly lower ONH-MV and choroidal MBR compared to the controls (p < 0.001 and p = 0.022, respectively) but no difference in ONH-MT (p = 0.773). CONCLUSION: Optic neuropathies showed different patterns of ocular BF impairment. Therefore, LSFG can be a useful tool for differentiating optic neuropathies.

Neuromyelitis Optica: A Deceiving Disorder of Demyelination.

Neuromyelitis optica (NMO), also known as Devic syndrome, is an autoimmune inflammatory and demyelinating disorder that affects the optic nerves and spinal cord. It is believed to be attributed to aquaporin-4 antibodies, a water channel expressed on astrocytes. It commonly presents with isolated or recurrent attacks of myelitis and optic neuritis. Intractable vomiting and hiccups may also be seen as symptoms. Acute treatment is often achieved with high-dose steroids and is imperative to prevent permanent central nervous system damage. Relapse prevention is achieved using long-term immunosuppression. This paper examines the case of an African-American female who presented with ascending lower extremity weakness.

Evaluation of demographic and neuro-ophthalmologic findings of patients with multiple sclerosis.

PURPOSE: The study aims to investigate the demographic and neuroophthalmologic features of patients with multiple sclerosis (MS). METHODS: This retrospective study investigated 270 eyes of 135 patients with MS. All subjects underwent a full ophthalmological examination. RESULTS: The study investigated 270 eyes of 135 patients with MS. The patients included 102 (74.8%) females and 34 (25.2%) males. The mean age at the time of diagnosis of MS was 29.9 ± 9.6 years. The mean follow-up period was 6.7 ± 10.9 months. Initial symptoms of MS at presentation included optic neuritis (ON) in 42 patients (15.6%), numbness of hands and feet in 20 patients (7.4%) and diplopia in 11 patients (4.1%). Additional diseases were observed in 29 patients (21.5%) and autoimmune diseases were observed in 11 patients (8.1%). Thirteen patients (9.62%) had relatives with MS; the relatives of five patients were first-degree relatives and the relatives of the remaining eight patients were second-degree relatives. Table 2 summarizes the additional systemic and ocular diseases and family history data. During MS, 72 patients (53.4%) were diagnosed with ON. ON was bilateral in 49 patients (68%) and unilateral in 23 patients (32%). Retrobulbar ON was observed in 77 eyes (81.6%) and papillitis was observed in 18 eyes (18.4%). Disorders of efferent visual pathway function were found in 43 patients (30.4%). CONCLUSION: Visual impairments are significant in patients with MS. Although ON is the most prevalent symptom of MS, it is important to keep in mind that damage to the efferent visual system can be observed.

Classification of optic neuritis in neuromyelitis optica spectrum disorders (NMOSD) on MRI using CNN with transfer learning and manipulation of pre-processing on augmentation.

Neuromyelitis optica spectrum disorder (NMOSD), also known as Devic disease, is an autoimmune central nervous system disorder in humans that commonly causes inflammatory demyelination in the optic nerves and spinal cord. Inflammation in the optic nerves is termed optic neuritis (ON). ON is a common clinical presentation; however, it is not necessarily present in all NMOSD patients. ON in NMOSD can be relapsing and result in severe vision loss. To the best of our knowledge, no study utilises deep learning to classify ON changes on MRI among patients with NMOSD. Therefore, this study aims to deploy eight state-of-the-art CNN models (Inception-v3, Inception-ResNet-v2, ResNet-101, Xception, ShuffleNet, DenseNet-201, MobileNet-v2, and EfficientNet-B0) with transfer learning to classify NMOSD patients with and without chronic ON using optic nerve magnetic resonance imaging. This study also investigated the effects of data augmentation before and after dataset splitting on cropped and whole images. Both quantitative and qualitative assessments (with Grad-Cam) were used to evaluate the performances of the CNN models. The Inception-v3 was identified as the best CNN model for classifying ON among NMOSD patients, with accuracy of 99.5%, sensitivity of 98.9%, specificity of 93.0%, precision of 100%, NPV of 99.0%, and F1-score of 99.4%. This study also demonstrated that the application of augmentation after dataset splitting could avoid information leaking into the testing datasets, hence producing more realistic and reliable results.