Peripapillary Retinal Nerve Fiber Layer Thickness as a Predictor of Visual Outcomes in Patients with Acute Nonarteritic Anterior Ischemic Optic Neuropathy.

Objective: To evaluate the utility of peripapillary retinal nerve fiber layer thickness (pRNFLT) for the prediction of visual outcomes, including visual acuity (VA) and visual field (VF), in subjects with acute nonarteritic anterior ischemic optic neuropathy (NAION). Materials and Methods: We performed a retrospective study of data relating to 60 eyes of 60 subjects with acute NAION. Of these, reliable VF values were obtained at both the initial and at 6-month follow-up visits for 30 eyes, which were included in the VF analysis. The pRNFLT was measured globally and separately in all four quadrants (superior, inferior, nasal, and temporal) using optical coherence tomography at the initial visit. Multivariate analysis and the area under the curve (AUC) were used to evaluate the utility of pRNFLT for the prediction of visual outcomes, including favorable VA (VA better than or equal to 20/25) and favorable VF (visual field index (VFI) ≥90%), at the 6-month follow-up visit. Results: The median VA and mean VFI at the initial visit were 0.40 (interquartile range (IQR): 0.40, 0.54; logarithm of the minimum angle of resolution (logMAR)) and 73.07% ± 6.73%, respectively. The median VA and mean VFI at the 6-month follow-up visit were 0.30 (IQR: 0.00, 0.70) logMAR and 69.27% ± 28.94%, respectively. Thinner temporal-quadrant pRNFLT was associated with favorable VA (odds ratio 0.98; p = 0.042) with a cut-off value of 128 µm (AUC 0.839, 95% CI: 0.732-0.947, sensitivity 77.27%, specificity 84.21%). Thinner nasal-quadrant pRNFLT was associated with favorable VF (odds ratio 0.97; p = 0.047) with a cut-off value of 105 µm (AUC 0.780, 95% CI: 0.612-0.948, sensitivity 90.00%, specificity 70.00%). Conclusions: The pRNFLT is clinically useful for the prediction of visual outcomes in patients with acute NAION. A temporal-quadrant pRNFLT ≤128 µm and a nasal-quadrant pRNFLT ≤105 µm predict favorable VA and VF at the 6-month follow-up visit, respectively.

Prevalences of Other Non-Thyroid Autoimmune Diseases and Factor Associated with Their Presence in Ocular Myasthenia Gravis.

Purpose: To report the prevalences of other non-thyroid autoimmune diseases and identify factors associated with their presence in ocular myasthenia gravis (OMG) subjects. Subjects and Methods: A total of 208 subjects with OMG diagnosis were included. Demographic data, clinical characteristics, the ice-pack test, the acetylcholine receptor (AChR) antibody test, electrophysiology tests (single-fiber electromyography and repetitive nerve stimulation), the presence of thymoma, generalized myasthenia gravis conversion, and the presence of other non-thyroid autoimmune diseases (defined as the presence of at least one other non-thyroid autoimmune disease) were retrospectively reviewed. Factors associated with the presence of other non-thyroid autoimmune diseases were analyzed by univariate and multivariate logistic regression. Results: Of the total 208 subjects, 21 (10.10%) exhibited the presence of other non-thyroid autoimmune diseases (19 subjects (9.14%) and 2 subjects (0.96%) had one and two other non-thyroid autoimmune diseases, respectively), and systemic lupus erythematosus (SLE) was diagnosed in 9 subjects, followed by Sjogren's syndrome (7 subjects), rheumatoid arthritis (6 subjects), and ankylosing spondylitis (1 subject). Therefore, the prevalences of SLE, Sjogren's syndrome, rheumatoid arthritis, and ankylosing spondylitis in OMG subjects were estimated to be 4.33% (95% confidence interval (CI): 2.29-8.02%), 3.37% (95% CI: 1.64-6.79%), 2.88% (95% CI: 1.33-6.14%), and 0.48% (95% CI: 0.08-2.67%), respectively. Positivity of the AChR antibody was the only significant factor associated with the presence of other non-thyroid autoimmune diseases (odds ratio 4.10, 95% CI: 1.11-15.21, p = 0.035). Conclusions: The presence of other non-thyroid autoimmune diseases was found in approximately 10% of OMG patients, with SLE displaying the highest prevalence. We recommend screening and monitoring for other non-thyroid autoimmune diseases in OMG patients, particularly those with positivity of the AChR antibody.

Diagnostic value of contrast-enhanced 3D FLAIR sequence in acute optic neuritis.

PURPOSE: Contrast-enhanced fluid-attenuated inversion recovery (FLAIR) sequence of the brain has the potential for detecting optic nerve abnormality. This study aimed to compare the diagnostic value of whole-brain contrast-enhanced three-dimensional FLAIR with fat suppression (CE 3D FLAIR FS) sequence in detecting acute optic neuritis to dedicated orbit MRI and clinical diagnosis. MATERIALS AND METHODS: Twenty-two patients with acute optic neuritis who underwent whole-brain CE-3D-FLAIR FS and dedicated orbit MRI were retrospectively included. The hypersignal FLAIR of the optic nerve on whole-brain CE-3D-FLAIR FS, enhancement, and hypersignal T2W on orbit images were assessed. The optic nerve to frontal white matter signal intensity ratio on CE-FLAIR FS was calculated as maximum signal intensity ratio (SIR) and mean SIR. RESULTS: Twenty-six hypersignals of optic nerves were found on CE-FLAIR FS from 30 pathologic nerves. The sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy of CE FLAIR FS brain and dedicated orbital images for diagnosing acute optic neuritis were 77%, 93%, 96%, 65%, and 82% and 83%, 93%, 96%, 72%, and 86%, respectively. Optic nerve to frontal white matter SIR of the affected optic nerves was higher than that of normal optic nerves. Using a cutoff maximum SIR of 1.24 and cutoff mean SIR of 1.16, the sensitivity, specificity, PPV, NPV, and accuracy were 93%, 86%, 93%, 80%, and 89% and 93%, 86%, 93%, 86%, and 91%, respectively. CONCLUSION: The hypersignal of the optic nerve on whole-brain CE 3D FLAIR FS sequence has qualitative and quantitative diagnostic potential in patients with acute optic neuritis.

Differentiation between aggressive and benign intracranial non-cavernous dural arteriovenous fistulas using cortical venous reflux on susceptibility weighted images.

PURPOSE: This study aimed to evaluate the ability of susceptibility-weighted imaging (SWI) to detect cortical venous reflux (CVR) in patients with intracranial non-cavernous dural arteriovenous fistulas (DAVFs), which can be helpful to differentiate benign and aggressive DAVFs. MATERIAL AND METHODS: Twenty-seven patients (8 women and 19 men) with 33 non-cavernous DAVFs were divided into benign and aggressive groups. Presence of CVR and pseudophlebitic pattern (PPP) and location of fistula on SWI were determined. Digital subtraction angiography was used as the reference standard. Interobserver agreement for the presence of CVR and PPP and location of DAVF on SWI was evaluated using the kappa statistic. Statistical comparisons between the benign and aggressive DAVFs were performed. RESULTS: Sensitivity, specificity, positive predictive value, and negative predictive value of SWI for detecting CVR was 73.7%, 85.7%, 87.5%, and 70.6%, respectively. Corresponding values for detecting PPP were 95.2%, 83.3%, 95.2%, and 83.3%, respectively. SWI correctly identified DAVF location in 78.9%. Prevalence rates of CVR and PPP on SWI were significantly higher in aggressive DAVFs than benign ones. CONCLUSION: SWI exhibited high sensitivity and specificity for detection of CVR, a characteristic used to differentiate benign and aggressive lesions. CVR and PPP on SWI are signs of aggressive DAVFs that guide to perform angiography confirmation and prompt treatment to avoid serious complication.

Significance of Acetylcholine Receptor Antibody Titers in Acetylcholine Receptor Antibody-Positive Ocular Myasthenia Gravis: Generalization and Presence of Thyroid Autoimmune Antibodies and Thymoma.

Objective: To evaluate the association in acetylcholine receptor (AChR) antibody-positive ocular myasthenia gravis (OMG) subjects between AChR antibody titers and conversion to generalized myasthenia gravis (GMG), the presence of thyroid autoimmune antibodies, and the presence of thymoma. Subjects and Methods: A total of 118 subjects with AChR antibody-positive OMG were included. Demographic data, clinical characteristics, serology tests, presence of thymoma, treatment, and conversion to GMG were retrospectively reviewed. The presence of thyroid autoimmune antibodies was defined as the presence of at least one of the following: (1) thyroid peroxidase antibody; (2) thyroglobulin antibody; (3) thyroid-stimulating hormone receptor antibody. Univariate and multivariate logistic regression analyses were used as methods of evaluating association. Results: AChR antibody titers were determined in all subjects with a median of 3.33 (0.46-141.09) nmol/L. The median follow-up period was 14.5 (3-113) months. At the final follow-up time-point, 99 subjects (83.90%) remained with a diagnosis of pure OMG, while 19 subjects (16.10%) had converted to GMG. An AChR antibody titer ≥8.11 nmol/L was associated with the conversion to GMG (odds ratio (OR) 3.66, 95% CI: 1.19-11.26; p = 0.023). Of the 79 subjects with available thyroid autoimmune antibodies data, 26 subjects (32.91%) displayed the presence of thyroid autoimmune antibodies. An AChR antibody titer ≥2.81 nmol/L was associated with the presence of thyroid autoimmune antibodies (OR 6.16, 95% CI: 1.79-21.22; p = 0.004). Finally, of the 106 subjects with available thoracic computed tomography (CT) data, only 9 subjects (8.49%) demonstrated the presence of thymoma. An AChR antibody titer ≥15.12 nmol/L was associated with the presence of thymoma (OR 4.97, 95% CI: 1.10-22.48; p = 0.037). Conclusion: AChR antibody titers should be considered in AChR antibody-positive OMG patients. Those with AChR antibody titers ≥8.11 nmol/L, who are at a greater risk of conversion to GMG, should be closely monitored and encouraged to be aware of early clinical signs of life-threatening GMG. In addition, serum thyroid autoimmune antibodies and thoracic CT screening for thymoma should be performed in AChR antibody-positive OMG patients, particularly in those with AChR antibody titers ≥2.81 nmol/L and ≥15.12 nmol/L, respectively.

Bilateral Posterior Ischemic Optic Neuropathy Due to Bilateral Anterior-Drainage Dural Carotid-Cavernous Fistulas: A Case Report.

Purpose: To report a case of bilateral posterior ischemic optic neuropathy (PION) due to bilateral anterior-drainage dural carotid-cavernous fistulas (CCFs). Case Description: We report on a 62-year-old woman with a history of poorly controlled hypertension who presented with sudden bilateral visual loss and headache for 5 days. She denied a history of head trauma. On examination, her visual acuities were no light perception (NLP) with fixed pupils in both eyes. The ocular motility of both eyes was limited in all directions. Both eyelids were difficult to open. Anterior segment examination revealed bilateral chemosis and episcleral corkscrew vessels. Intraocular pressures were 45 and 48 mmHg in her right and left eyes, respectively. Gonioscopy revealed blood in Schlemm's canal at the nasal angle of the right eye. Fundus examination showed slightly dilated and tortuous retinal veins with normal-appearing optic discs in both eyes. The cup-to-disc ratios were 0.3 bilaterally. Other neurological examinations were unremarkable. Magnetic resonance imaging demonstrated dilation of the bilateral superior ophthalmic veins (SOVs), and marked orbital and periorbital congestion bilaterally. However, there was no compression or stretching of the bilateral optic nerves. Diffusion restriction on diffusion-weighted imaging, with corresponding reduced apparent diffusion coefficient, in the entire bilateral orbital segment of the optic nerves was revealed, consistent with bilateral PION. Magnetic resonance angiography revealed arterialization of the bilateral cavernous sinuses and SOVs. Cerebral angiography confirmed the diagnosis of bilateral anterior-drainage dural CCFs. Treatment with transvenous coil embolization was successful. Three months after embolization, ophthalmic examination demonstrated progressive improvement of aforementioned ophthalmic signs; however, her visual acuities remained NLP in both eyes. Conclusion: To our knowledge, this is the first reported case of bilateral PION due to bilateral anterior-drainage dural CCFs. In spite of its rarity, PION should be considered as a severe, irreversible ophthalmic complication of anterior-drainage dural CCF.

Preoperative Peripapillary Retinal Nerve Fiber Layer Thickness as the Prognostic Factor of Postoperative Visual Functions After Endoscopic Transsphenoidal Surgery for Pituitary Adenoma.

Purpose: To evaluate the prognostic ability of preoperative peripapillary retinal nerve fiber layer thickness (pRNFLT) for predicting postoperative visual functions, including the visual field index (VFI) and visual acuity (VA), of subjects with pituitary adenoma (PA) who were treated with endoscopic transsphenoidal surgery for pituitary adenoma (ETSS-PA) exclusively. Subjects and Methods: This 11-year retrospective study was performed at a single institution in Thailand. Sixty-six eyes of 33 subjects who had a PA compressing the anterior visual pathway and were treated with ETSS-PA alone were included. The pRNFLT was measured globally and in the four quadrants preoperatively, using optical coherence tomography. Multivariable analysis and area under the curve (AUC) were used to demonstrate the prognostic ability of preoperative pRNFLT for postoperative visual functions (> 1 month but < 6 months after ETSS-PA). Results: The mean postoperative VFI and median postoperative VA were 79.45% ± 24.24% and 0.14 [interquartile range: 0.02, 0.40] logarithm of the minimum angle of resolution. Among the 56 eyes with a reliable postoperative VFI, thicker preoperative temporal (odds ratio, 1.18; p = 0.024) and inferior (odds ratio, 1.07; p = 0.013) pRNFLT values were associated with a postoperative VFI > 90%. The strongest association occurred with the preoperative temporal pRNFLT (AUC = 0.821, 95% CI: 0.720-0.923) with a cut-off value of 60 µm. Multivariable analysis for all 66 eyes showed that thicker preoperative inferior-quadrant pRNFLT (odds ratio, 1.05; p = 0.001) was associated with a postoperative VA of at least 20/25. The strongest performance was found with the preoperative inferior pRNFLT (AUC = 0.732, 95% CI: 0.615-0.849) with a cut-off value of 105 µm. Conclusion: Preoperative pRNFLT offers clinical utility for predicting visual functions after ETSS-PA. Temporal pRNFLT ≥ 60 µm and inferior pRNFLT ≥105 µm predicted postoperative VFI > 90% and postoperative VA better than or equal to 20/25, respectively.

Serum Vitamin D Levels and Status in Thai Optic Neuritis Subjects: A Case-Control Study.

Objective: To measure serum total vitamin D or 25-hydroxyvitamin D [25(OH)D] levels and status in immune-based optic neuritis (ON) including neuromyelitis optica spectrum disorder (NMOSD)-ON, myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD)-ON, autoimmune-ON, and idiopathic-ON and compare them with age- and sex-matched healthy controls. The secondary objective was to analyze the association between serum 25(OH)D levels and ON attack severity (nadir best-corrected visual acuity; nadir BCVA). Materials and Methods: This was a single-center, case-control study. We enrolled 59 subjects (19 NMOSD-ON, 6 MOGAD-ON, 11 autoimmune-ON, 23 idiopathic-ON) diagnosed with acute immune-based ON (any ON attacks) over 11 years. Electronic medical records were reviewed and demographic data (age at sampling, sex, aquaporin-4 immunoglobulin (AQP4-IgG); myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG); other biomarkers of autoimmune disorders), ON attack severity (nadir BCVA), and serum 25(OH)D levels in the acute phase of ON were collected. Serum 25(OH)D levels of 236 age- and sex-matched healthy controls were assessed. Results: Mean serum 25(OH)D levels were significantly lower in each group of immune-based ON compared with healthy controls (p < 0.001 for each ON group). However, mean serum 25(OH)D levels were not significantly different between four ON groups (NMOSD-ON, 20.18±5.90 ng/mL; MOGAD-ON, 23.07±4.94 ng/mL; autoimmune-ON, 21.14±5.29 ng/mL; idiopathic-ON, 19.56 ±5.12 ng/mL; p = 0.525). All immune-based ON subjects had vitamin D insufficiency or vitamin D deficiency. The prevalences of vitamin D insufficiency and vitamin D deficiency were significantly higher than in healthy controls in each ON group (both p < 0.05 in each ON group). No associations were observed between serum 25(OH)D levels and ON attack severity (nadir BCVA). Conclusions: Thai immune-based ON subjects had lower serum 25(OH)D levels and higher prevalence of vitamin D insufficiency and vitamin D deficiency compared with age- and sex-matched healthy controls. Serum 25(OH)D levels were not associated with ON attack severity (nadir BCVA). We highly recommend that serum 25(OH)D levels be screened in all subjects with acute immune-based ON.

Long-Term Visual Function After Fractionated Stereotactic Radiotherapy for Primary Optic Nerve Sheath Meningioma: A Retrospective Analysis of 34 Subjects.

Objective: To evaluate long-term visual function after fractionated stereotactic radiotherapy (FSRT) for primary optic nerve sheath meningioma (PONSM). Methods: This 22-year retrospective study included 34 subjects (34 affected eyes) with PONSM who were treated with FSRT exclusively. Subjects with a history of biopsy/resection were excluded. Visual function, including visual acuity (VA) and visual field mean deviation (VF MD), was evaluated at presentation (pre-radiotherapy; pre-RT) and at the final follow-up (post-radiotherapy; post-RT); treatment complications were also evaluated. Treatment success was defined as either stabilization or improvement of visual function. Results: The median pre-RT VA and pre-RT VF MD were 0.70 logarithm of the minimum angle of resolution (logMAR; range: 0.0-2.9 logMAR) and -15.4 decibels (dB) (range: -31.4 to -3.2 dB), respectively. The median total dose of FSRT was 50 Gy (range: 45-54 Gy) and the median number of fractions was 25 (range: 25-30). The median follow-up interval was 89 months (range: 6-251 months). The median post-RT VA and post-RT VF MD were 0.48 logMAR (range: 0.0-2.9 logMAR) (p = 0.010) and -6.8 dB (range: -20.6 to -1.6 dB) (p = 0.005), respectively. Among the 34 included eyes, VA was successfully treated in 29 eyes (85.3%) and worsened in 5 eyes (14.7%). Of the 14 eyes with both VA and reliable VF MD at pre-RT and post-RT time points, VF MD was successfully treated in 13 eyes (92.8%) and worsened in one (7.2%); overall visual function was successfully treated in 13 eyes (92.8%) and worsened in 1 eye (7.2%). Complications occurred in one subject (2.9%; radiation retinopathy). Conclusion: Approximately 90% of PONSM subjects exhibited long-term treatment success in terms of VA, VF MD, and overall visual function after FSRT. Additionally, the incidence of complications was low. Therefore, FSRT is effective and safe treatment for PONSM.

Radiological Characteristics of Extraocular Muscles in Myasthenia Gravis Patients with Ocular Manifestations: A Case-Control Study.

PURPOSE: To analyze radiological characteristics of the extraocular muscles (EOMs) in myasthenia gravis (MG) patients with ocular manifestations. PATIENTS AND METHODS: This retrospective case-control study included all MG cases with ocular manifestations, who attended a neuro-ophthalmology clinic at a university hospital, Bangkok, from April 2009 to June 2018. They experienced double vision and ophthalmoplegia. Control participants had normal eye movements. Orbital scans were thoroughly reviewed. We measured muscle thickness (MT) of the superior rectus, inferior rectus, medial rectus and lateral rectus muscles in both eyes using magnetic resonance imaging or computed tomography scan. The sum of the muscle thickness at all sites was calculated (MTsum). Comparisons of the mean MT of each muscle type and the mean MTsum between the MG and control groups were performed by using Student's t-test. MRI signal intensities of the EOMs were also recorded. RESULTS: Twenty MG cases and 20 controls were included in the study. The mean MTsum was 23.7 (standard deviation 4.8) mm in the MG group and 32.6 (3.5) mm in the controls. There were statistically significant differences between the two groups with respect to the mean MT and mean MTsum (p <0.001). In the MG group, there was a negative correlation between the MTsum and disease duration (p= 0.03). By using coronal T2-weighted orbital MRI with fat suppression (T2W/FS), the most frequent finding was isointensity with central hypointensity of the EOMs in the MG group. CONCLUSION: Atrophic EOMs were frequently found in the MG group, particularly in chronic cases. Isointensity with central hypointensity of EOMs on T2W/FS was also common in the MG group. These findings highlight the importance of muscle involvement in MG and may be helpful for clinical decision-making.

Comparison of Early- and Late-Onset NMOSD-Related Optic Neuritis in Thai Patients: Clinical Characteristics and Long-Term Visual Outcomes.

OBJECTIVE: To compare demographic data, clinical and radiological characteristics, treatments, and long-term visual outcomes between patients with late-onset neuromyelitis optica spectrum disorder-related optic neuritis (LO-NMOSD-ON) (age at onset ≥ 50 years) and patients with early-onset neuromyelitis optica spectrum disorder-related optic neuritis (EO-NMOSD-ON) (age at onset < 50 years). PATIENTS AND METHODS: This retrospective study included 47 patients (69 eyes) who were diagnosed with neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) over a 12-year period. There were 14 patients (21 eyes) and 33 patients (48 eyes) in the LO-NMOSD-ON and EO-NMOSD-ON groups, respectively. RESULTS: LO-NMOSD-ON-affected eyes exhibited significantly worse median nadir visual acuity (VA) at optic neuritis (ON) onset, compared with EO-NMOSD-ON-affected eyes (2.7 logMAR (range 2.6-2.9 logMAR) vs 1.95 logMAR (range 0.4-2.9 logMAR); p = 0.03). Similarly, 100% of LO-NMOSD-ON-affected eyes demonstrated a nadir VA of worse than or equal to 1.0 logMAR, compared with 62.5% of EO-NMOSD-ON-affected eyes (p = 0.03). LO-NMOSD-ON-affected eyes had a worse median final VA, compared with EO-NMOSD-ON-affected eyes (1.3 logMAR (range 0-2.9 logMAR) vs 0.3 logMAR (range 0-2.9 logMAR); adjusted p = 0.037). LO-NMOSD-ON-affected eyes more frequently exhibited a final VA of worse than or equal to 1.0 logMAR, compared with EO-NMOSD-ON-affected eyes (57.1% vs 27.0%; adjusted p = 0.039). A positive correlation was observed between age at ON onset of each eye and the final VA (logMAR) (Spearman r = 0.34, p = 0.0075). The remaining parameters did not significantly differ between the two groups. CONCLUSION: Patients with LO-NMOSD-ON had significantly worse nadir VA at ON onset and significantly worse final VA, relative to patients with EO-NMOSD-ON. Age at ON onset of each eye was positively correlated with final VA (logMAR). Despite the difference in common age at onset, NMOSD-ON should be included in the differential diagnosis of late-onset acute to subacute optic neuropathy, along with ischemic optic neuropathy.

Prognostic Factors for Visual Outcomes Following the First Episode of NMOSD-Related Optic Neuritis in Affected Eyes.

OBJECTIVE: We aim to identify prognostic factors for visual outcomes following a first episode of neuromyelitis optica spectrum disorder-related optic neuritis (NMOSD-ON) in affected eyes. MATERIALS AND METHODS: This was a single-center, retrospective study. The study included 50 individuals who were diagnosed with NMOSD-ON (63 affected eyes) in a tertiary institution over a 12-year period. Data regarding any second, or higher, episodes of NMOSD-ON in each eye were not taken into consideration. Medical records of included individuals were reviewed. Demographic data, clinical and magnetic resonance imaging characteristics, and treatment outcomes were collected. Main outcome measures of the study were prognostic factors for good visual outcome (best-corrected visual acuity (BCVA) ≥ 20/200) following an initial episode of NMOSD-ON in affected eyes. RESULTS: Sixty-three affected eyes of 50 individuals (3 men and 47 women) were included. BCVA at nadir that was better than counting fingers (CF) (odds ratio 10.43, 95% confidence interval 1.04, 104.45, p = 0.046) and time from NMOSD-ON onset to intravenous methylprednisolone (IVMP), less than 21 days (odds ratio 10.73, 95% confidence interval 1.91, 60.01, p = 0.007), were significantly associated with good visual outcomes. CONCLUSION: BCVA at nadir that was better than CF and treatment with IVMP within 21 days of symptom onset were important prognostic factors of good visual outcomes following a first episode of NMOSD-ON in affected eyes.

MOG-IgG- versus AQP4-IgG-Positive Optic Neuritis in Thailand: Clinical Characteristics and Long-Term Visual Outcomes Comparison.

PURPOSE: To compare demographic data, clinical and radiological characteristics, treatment, and long-term visual outcomes between myelin oligodendrocyte glycoprotein autoantibody-positive optic neuritis (MOG-IgG + ON) and aquaporin-4 autoantibody-positive optic neuritis (AQP4-IgG + ON) in Thailand. PATIENTS AND METHODS: We included individuals who were diagnosed with either MOG-IgG + ON or AQP4-IgG + ON over an 11-year period. Demographic data, clinical and radiological characteristics at ON presentation, treatment, and long-term visual outcomes were retrospectively collected. RESULTS: There were 16 patients (28 eyes) and 43 patients (59 eyes) in the MOG-IgG + ON and AQP4-IgG + ON groups, respectively. AQP4-IgG + ON occurred predominantly in female patients whereas MOG-IgG + ON-affected female patients and male patients equally (p < 0.001). Prior or concurrent non-ON demyelinating events were more often observed at AQP4-IgG + ON onset (p < 0.001). At ON presentation, bilaterality and the presence of optic disc edema were predominantly found in the MOG-IgG + ON group (bilaterality: 80% vs 8%, MOG-IgG + ON vs AQP4-IgG + ON patients, respectively (p < 0.001); presence of optic disc edema: 92.3% vs 36.6%, MOG-IgG + ON- vs AQP4-IgG + ON-affected eyes, respectively (p < 0.001)). There was no statistically significant difference in age at ON onset, nadir visual acuity (VA), presence of pain, segmental enhancement, and total enhanced segments of the anterior visual pathways. At the last follow-up, immunosuppressive drugs were used more often in the AQP4-IgG + ON group (43.7% vs 74.4%, MOG-IgG + ON vs AQP4-IgG + ON, respectively; p < 0.027). Remarkably better final VA was achieved in MOG-IgG + ON-affected eyes (median: 0.0 vs 0.4 logMAR, MOG-IgG + ON- vs AQP4-IgG + ON-affected eyes, respectively; p < 0.001). CONCLUSION: Compared with AQP4-IgG + ON, MOG-IgG + ON tended to present with bilaterality and optic disc edema and demonstrated better visual outcomes.

Clinical Characteristics of HIV-Associated Optic Neuritis.

INTRODUCTION: Acute optic neuritis (ON) has been increasingly reported in patients infected with human immunodeficiency virus (HIV). We aimed to describe clinical characteristics of HIV-infected patients with ON. MATERIALS AND METHODS: This observational retrospective study was performed from January 2008 to January 2016 in a university hospital in Bangkok, Thailand. Demographic data and clinical manifestations were retrospectively reviewed. RESULTS: We included 10 HIV-infected patients and divided them into two groups: infectious and non-infectious ON. There were six patients in the infectious ON group (five males, mean age 33.6 years, median CD4 cell counts during ON episodes 36.5 cells/µL, high viral loads, median initial visual acuity [VA] 1.7, median VA difference [initial VA - follow-up VA] 0). Four patients developed cryptococcal meningitis and 2 developed neuro-syphilis. They were treated with amphotericin B plus fluconazole and penicillin G sodium, respectively. There were four patients in the non-infectious ON group (two males, mean age 44.3 years, median CD4 cell count during acute ON 157.5 cells/µL, undetectable viral loads, median initial VA 1.3, and median VA difference 1.2). They were given corticosteroid treatments. There were no statistically significant differences between the two groups with respect to age, sex, and initial VA. There were statistically significant differences in median VA difference and median CD4 cell counts between the two groups (p=0.02 and 0.03, respectively). There was a significant correlation between CD4 counts and duration in three non-infectious ON patients treated with antiretroviral therapy (ART, p<0.001). DISCUSSION: Patients with infectious ON had low initial CD4 counts and high viral loads. Patients with non-infectious ON had increasing CD4 cell counts and undetectable HIV viral load following ART. The findings are highly suggestive of immune reconstitution inflammatory syndrome. The CD4 cell counts and viral loads may help to identify the type of acute ON in HIV-infected patients and establish proper therapies.

Etiologies of Acute Optic Neuritis in Thailand: An Observational Study of 171 Patients.

PURPOSE: To analyze the demographic patterns, clinical characteristics and etiologies of acute optic neuritis (ON). METHODS: This retrospective observational study included patients with acute ON who presented to a university hospital in Bangkok, Thailand, between January 2010 and March 2020. The demographic details, clinical characteristics and etiologies of acute ON were evaluated. RESULTS: A total of 171 patients were included in the study (78.4% [n=134] female; mean age 45 years [standard deviation 15.4 years]; 32.2% [n=55] bilateral involvement). The most common type of acute ON was idiopathic (51.5%), followed by neuromyelitis optica spectrum disorder (NMOSD, 30.9%), other autoimmune disorders (9.9%), myelin oligodendrocyte glycoprotein antibody-associated disorder (MOGAD, 5.3%), multiple sclerosis (MS, 1.8%), and postinfection (0.6%). In the other autoimmune disorders group, 2 patients developed systemic lupus erythematosus (1.2%), 2 Sjogren's syndrome (1.2%), 1 RA (0.6%), 1 anti-NMDAR (0.6%), 3 anti-Jo1 (1.8%), 2 c-ANCA (1.2%), 1 anti-centromere (0.6%), and 5 nonspecific autoimmune disorders (2.9%). In the idiopathic group, 38.6% developed single isolated ON, 1.8% relapsing isolated ON and 11.1% chronic relapsing inflammatory optic neuropathy. CONCLUSION: The most common form of acute ON in this study, similar to other Asian countries, was idiopathic. Idiopathic-ON shared some phenotypes with NMOSD and MOGAD. We also reported patients with anti-NMDAR, anti-Jo1, c-ANCA and anti-centromere disorders. Improvements in antibody detection have widened the range of possible etiologies of acute ON. The study highlighted the important role of antibodies in creating effective treatments in the future.

Evaluation of Retinal Nerve Fiber Layer and Ganglion Cell Layer Thickness in Alzheimer's Disease Using Optical Coherence Tomography.

OBJECTIVE: To evaluate the feasibility of using optical coherence tomography (OCT) for the detection of Alzheimer's disease (AD), by measuring the thickness of the retinal nerve fiber layer (RNFL) and the ganglion cell layer and inner plexiform layer (GCL-IPL). MATERIAL AND METHODS: This was a single-center, cross-sectional study. The study included 29 patients with AD (mean age ± standard deviation: 75.61 ± 6.24 years) and 29 healthy age- and sex-matched controls. All participants underwent cognitive evaluations using the Montreal Cognitive Assessment test. Measurements of the RNFL thickness, as well as GCL-IPL thickness, were obtained for all participants using OCT. Both RNFL and GCL-IPL parameters were adjusted for best-corrected visual acuity, hypertension, diabetes and dyslipidemia. RESULTS: The mean RNFL thickness was significantly thinner in the AD group than in the control group (85.24 and 90.68 µm, respectively, adjusted P=0.014). The superior quadrant was thinner in the AD group (adjusted P=0.033). The thicknesses did not differ significantly between groups for the other quadrants. The mean GCL-IPL thickness in the AD (68.81 µm) was significantly thinner than that in the controls (76.42 µm) (adjusted P=0.014). Overall, there was a negative correlation between age and mean RNFL; and between age and GCL-IPL thickness (r=-0.338, P=0.010 and r=-0.346, P=0.008, respectively). CONCLUSION: The mean RNFL and GCL-IPL thicknesses were thinner in the AD group than in the control group. These findings suggest that RNFL and GCL-IPL thickness may be biological markers for AD.

Initial Treatment Response in Ocular Myasthenia Gravis: A Comparison Between Low and Moderate Doses of Prednisolone.

PURPOSE: To evaluate the initial treatment response to low doses of prednisolone, compared with moderate doses, in ocular myasthenia gravis (OMG). PATIENTS AND METHODS: A retrospective chart review of patients with adult-onset (age ≥15 years old) OMG, who were treated with prednisolone, was conducted. Subjects were divided into two groups according to their prednisolone dosing regimen. The low-dose group was defined as those with an average 12-week cumulative dose of prednisolone <0.435 mg/kg/day and the moderate-dose group averaged 0.435-1.000 mg/kg/day. The primary outcome of interest was the comparison of clinical response to prednisolone at 12 weeks between the low-dose and moderate-dose groups. The secondary outcome was the difference in adverse events between treatment groups. RESULTS: Of 34 subjects, 16 subjects (47.1%) were male. The mean age at onset was 44.0±18.1 years. The most common presenting ocular feature was ptosis with ophthalmoplegia (22 subjects, 64.7%), followed by isolated ptosis (nine subjects, 26.5%) and isolated ophthalmoplegia (three subjects, 8.8%). Half of the subjects were treated with low-dose prednisolone and the other half were treated with moderate-dose prednisolone. There were no substantial differences in baseline characteristics between treatment groups. After 12 weeks of treatment, nine of 17 subjects (52.9%) and 13 of 17 subjects (76.5%) in the low- and moderate-dose groups, respectively, were regarded as responsive to the prednisolone treatment (P=0.28). Adverse events were exclusively observed in the moderate-dose group. CONCLUSION: Treatment of OMG with an average 12-week cumulative dose of prednisolone <0.435 mg/kg/day (low dose) shows a comparable responsive outcome to 0.435-1.000 mg/kg/day of prednisolone (moderate dose). Treating OMG with low-dose prednisolone can minimize prednisolone-related adverse events. However, a prospective randomized controlled trial with a larger study population is warranted in order to gain more insight into the proper dosage of prednisolone for OMG.

Differences in Clinical Features of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis in White and Asian Race.

PURPOSE: To determine whether clinical features and visual outcomes of myelin oligodendrocyte glycoprotein antibody-associated optic neuritis (MOG-ON) differ between White and Asian subjects. DESIGN: Multicenter retrospective cohort. METHODS: This was a multicenter study of 153 subjects who were White or Asian with a history of adult-onset (age 18 years or older) optic neuritis (ON) and positive MOG-IgG serology by cell-based assay. Subjects were enrolled from 2 unpublished cohorts (January 2017-November 2019) and 9 published cohorts with case-level data available (2012-2018). Subjects with alternative etiologies of demyelinating disease and positive or lack of aquaporin-4-IgG serology result were excluded. The main outcome measurements were clinical features and final visual outcomes. RESULTS: Of the 153 subjects who were White (n = 80) or Asian (n = 73) included in the study, 93 (61%) were women, mean age of onset was 40.8 ± 14.9 years, and median follow-up was 35.2 months (range: 1-432 months); all of these characteristics were similar between White and Asian subjects. White subjects were more likely to have recurrent ON (57 [71%] vs 20 [27%]; P = .001) and extra-optic nerve manifestations (35 [44%] vs 8 [11%]; P = .001). Optic disc swelling, neuroimaging findings, presenting visual acuity (VA), treatment, and final VA did not differ according to subjects' race. Despite the high prevalence of severe visual loss (<20/200) during nadir, most subjects had good recovery of VA (>20/40) at final examination (51/77 [66%] White subjects vs 52/70 [74%] Asian subjects). CONCLUSION: White subjects with MOG-ON were more likely to have recurrent disease and extra-optic nerve manifestations. Visual outcomes were similar between White and Asian subjects.

Isolated Horner syndrome as a rare initial presentation of nasopharyngeal carcinoma: a case report.

BACKGROUND: Horner syndrome refers to a set of clinical presentations resulting from disruption of sympathetic innervation to the eye and adnexa. Classically, the clinical triad consists of ipsilateral blepharoptosis, pupillary miosis, and facial anhidrosis. Ocular sympathetic denervation may signify life-threatening causes. Timely investigation and accurate diagnosis are essential in patients with oculosympathetic denervation. CASE PRESENTATION: A 33-year-old Asian man with a heavy smoking habit presented with a 3-week history of left ptosis and no other complaints. His visual acuity was 20/20 bilaterally. An ophthalmic examination was significant for mild ptosis of his left eyelid and anisocoria (smaller left pupil), which was greater in the dark. Both pupils reacted to light briskly without an afferent pupillary defect. Anhidrosis was found on the medial side of the left forehead. A 10% cocaine test was positive. At his first visit, neurologic examination was unremarkable. Comprehensive radiological investigations were scheduled for a left-sided isolated Horner syndrome. Two weeks after his first visit, he experienced a left-sided headache along with ipsilateral Horner syndrome. Neurologic examination revealed hypoesthesia in the left cranial nerve V1-3 territories. Emergent computed tomography angiography was suspected for petrous part of the left internal carotid artery (ICA) dissection. Magnetic resonance imaging demonstrated an enhancing infiltrative lesion with its epicenter at the left sphenoid bone. The lesion encased the left ICA and invaded the left Meckel cave. Rhinoscopy with incisional biopsy revealed squamous cell nasopharyngeal carcinoma. CONCLUSION: This case involved an unusual initial presentation of nasopharyngeal carcinoma: isolated Horner syndrome with clinical progression to adjacent structures. Infiltration involving the Meckel cave and ICA at the foramen lacerum can present as postganglionic Horner syndrome associated with trigeminal pain and hypoesthesia. These clinical findings may mimic carotid artery dissection on computed tomography angiography. Detailed magnetic resonance imaging with careful attention to the skull base should be performed.