Artificial Intelligence to Detect Papilledema from Ocular Fundus Photographs.

BACKGROUND: Nonophthalmologist physicians do not confidently perform direct ophthalmoscopy. The use of artificial intelligence to detect papilledema and other optic-disk abnormalities from fundus photographs has not been well studied. METHODS: We trained, validated, and externally tested a deep-learning system to classify optic disks as being normal or having papilledema or other abnormalities from 15,846 retrospectively collected ocular fundus photographs that had been obtained with pharmacologic pupillary dilation and various digital cameras in persons from multiple ethnic populations. Of these photographs, 14,341 from 19 sites in 11 countries were used for training and validation, and 1505 photographs from 5 other sites were used for external testing. Performance at classifying the optic-disk appearance was evaluated by calculating the area under the receiver-operating-characteristic curve (AUC), sensitivity, and specificity, as compared with a reference standard of clinical diagnoses by neuro-ophthalmologists. RESULTS: The training and validation data sets from 6779 patients included 14,341 photographs: 9156 of normal disks, 2148 of disks with papilledema, and 3037 of disks with other abnormalities. The percentage classified as being normal ranged across sites from 9.8 to 100%; the percentage classified as having papilledema ranged across sites from zero to 59.5%. In the validation set, the system discriminated disks with papilledema from normal disks and disks with nonpapilledema abnormalities with an AUC of 0.99 (95% confidence interval [CI], 0.98 to 0.99) and normal from abnormal disks with an AUC of 0.99 (95% CI, 0.99 to 0.99). In the external-testing data set of 1505 photographs, the system had an AUC for the detection of papilledema of 0.96 (95% CI, 0.95 to 0.97), a sensitivity of 96.4% (95% CI, 93.9 to 98.3), and a specificity of 84.7% (95% CI, 82.3 to 87.1). CONCLUSIONS: A deep-learning system using fundus photographs with pharmacologically dilated pupils differentiated among optic disks with papilledema, normal disks, and disks with nonpapilledema abnormalities. (Funded by the Singapore National Medical Research Council and the SingHealth Duke-NUS Ophthalmology and Visual Sciences Academic Clinical Program.).

Bilateral oculomotor ocular neuromyotonia: a case report.

BACKGROUND: Ocular neuromyotonia (ONM) is characterized by episodic diplopia, which is usually triggered by prolonged eccentric gaze of the affected extraocular muscles. The spell is characterized by involuntary, occasionally painful, sustained contraction of one or more extraocular muscles innervated by the oculomotor, trochlear, or abducens nerve. ONM usually occurs as a late consequence of radiotherapy around the parasellar area, although idiopathic cases have been reported. Most cases are unilateral; however, bilateral ONM has occasionally been described. CASE PRESENTATION: A 60-year-old woman presented with a 4-month history of episodic, painful, horizontal binocular diplopia. She underwent external beam radiotherapy to the skull base for treatment of nasopharyngeal carcinoma. The tumor was well controlled. General neurological examination findings were unremarkable. Neuro-ophthalmic examination revealed normal visual acuity, visual fields, pupils, and fundi. Ocular alignment showed orthotropia with normal ocular motility. Myasthenic eyelid signs were absent. However, she developed episodes of involuntary sustained contraction of the medial rectus muscle following prolonged eccentric gaze toward the affected medial rectus muscle, which resulted in esotropia upon returning to the primary position. The esotropic episodes spontaneously resolved after approximately 2 min. These spells affected both medial rectus muscles. Both pupils remained normal throughout the examination. Magnetic resonance imaging revealed neither brain parenchyma/brain stem lesions nor tumor recurrence. Her symptoms were successfully treated with carbamazepine. CONCLUSIONS: Episodic esotropia in the adducting eye following prolonged horizontal eccentric gaze is a significant characteristic of ONM affecting the bilateral medial rectus muscles (i.e., bilateral oculomotor ONM). In spite of its extreme rarity, ONM should be considered as a differential diagnosis of episodic diplopia, especially in patients with a history of radiotherapy around the parasellar area. Careful examination with prolonged eccentric gaze should be performed to achieve a correct diagnosis and avoid an extensive unnecessary workup.

Isolated optic neuritis with a concurrent abnormal trigeminal nucleus on imaging: case report of a rare complication of herpes zoster ophthalmicus.

BACKGROUND: Herpes zoster ophthalmicus (HZO) is an inflammation related to reactivation of the latent varicella zoster virus (VZV), involving the ophthalmic branch of the trigeminal nerve. Optic neuritis (ON), a rare ocular complication following HZO, has been reported in 1.9% of HZO-affected eyes. Most previous cases occurred simultaneously with other ocular complications, especially orbital apex syndrome. Moreover, detailed magnetic resonance imaging (MRI) with diffusion weighted imaging of the optic nerve and trigeminal nucleus in HZO-related ON has been rarely reported. We report a case of postherpetic isolated ON with a concurrent abnormal trigeminal nucleus on imaging. CASE PRESENTATION: A healthy 58-year-old female presented with sudden painful visual loss in her right eye for 2 days. Four weeks before the presentation, her right eye was diagnosed with HZO, and she received intravenous acyclovir for 10 days. Ophthalmic examination revealed a visual acuity of light perception and 20/20 in the right and left eyes, respectively. A relative afferent pupillary defect was present in the right eye. Neurological examination was significant for hypoesthesia in the area of the HZO. A clinical diagnosis of HZO-related right retrobulbar ON was made, and other causes of atypical ON were excluded. MRI showed enhancement and restricted diffusion of the right-sided optic nerve with linear hyperintense T2 of the right-sided spinal trigeminal nucleus and tract (STNT) along the brainstem. She received 14 days of intravenous acyclovir and 5 days of methylprednisolone. Both were switched to an oral route for 2 months. After the completion of treatment, the visual acuity was counting fingers and 20/20 in the right eye and left eye, respectively. Stable brainstem STNT abnormalities and resolution of ON were found radiologically. CONCLUSIONS: Isolated ON is a rare ocular complication following HZO. An abnormal high signal of STNT on a T2 weighted image may be present, which may be a clue for VZV-associated complications, such as HZO-related ON, especially in cases lacking an obvious history of HZO or other concomitant ocular complications. Prompt treatment with both acyclovir and corticosteroids should be started. Restricted diffusion of the optic nerve may be a predictor for poor visual recovery.

Atypical central retinal artery occlusion as the first presentation of POEMS syndrome: a case report.

BACKGROUND: POEMS syndrome is a plasma cell disorder, which clinically manifests from paraneoplastic syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. The most common ocular manifestation is optic disc swelling, whereas other ocular manifestations; cystoid macular edema, serous macular detachment, venous sinus thrombosis, infiltrative orbitopathy, uveitis, neovascularization of the disc, peripapillary choroidal neovascularization and optic disc drusen, had also been reported. CASE PRESENTATION: A 52-year-old Thai man presented with 5-day sudden painless visual loss in the left eye. Ocular examination revealed visual acuity of 20/20 and no light perception in the right and left eye, respectively. Right fundoscopic examination was significant for hyperemic generalized optic disc swelling. Left fundoscopic examination revealed opaque and edematous entire retina giving the appearance of central retinal artery occlusion (CRAO) along with pallid "chalky white" optic disc swelling. Fluorescein angiography showed profound leakage of bilateral optic nerve heads and arteriolar filling defect in macular area along with leakage of small retinal arterioles in the left eye. Indocyanine green angiography demonstrated choroidal filling defect in the left eye only. Neuroimaging showed enhancement and luminal narrowing of left internal carotid artery, early subacute watershed infarctions in the left cerebral hemisphere and pachymeningeal enhancement. Cerebrospinal fluid analysis revealed high protein level with normal opening pressure. Intravenous methylprednisolone was initially started without any benefit. After extensive investigations, diagnosis of "POEMS syndrome" was made based on polyneuropathy, elevated lambda light chain level, elevated plasma vascular endothelial growth factor (VEGF), hepatomegaly, spinal sclerotic bone lesions, and thrombocytosis. Furthermore, sural nerve biopsy demonstrated neuropathy and positive VEGF staining. He was treated with eight cycles of bortezomib, cyclophosphamide and dexamethasone (BorCyDex). Polyneuropathy and thrombocytosis had remarkably improved after 2nd cycle, whereas, visual impairment had shown no recovery. Hepatomegaly was significantly reduced after the completion of BorCyDex. Our case eventually received autologous hematopoietic stem cell transplantation with high dose melphalan. CONCLUSIONS: To our knowledge, we illustrated the first patient given CRAO as the first presentation and ocular finding ever reported in POEMS syndrome. Both cerebral and ocular infarctions were presumably the result of VEGF-induced cranial vasculopathy as evidenced by neuroimaging.

Occipital lobe infarction: a rare presentation of bilateral giant cavernous carotid aneurysms: a case report.

BACKGROUND: Cavernous carotid aneurysm (CCA) represents 2-9% of all intracranial aneurysms and 15% of internal carotid artery (ICA) aneurysms; additionally, giant aneurysms are those aneurysms that are > 25 mm in size. Bilateral CCAs account for 11-29% of patients and are commonly associated with structural weaknesses in the ICA wall, secondary to systemic hypertension. CCAs are considered benign lesions, given the low risk for developing major neurologic morbidities (i.e., subarachnoid hemorrhage, cerebral infarction, or carotid cavernous fistula). Moreover, concurrent presentation with posterior circulation cerebral infarction is even rarer, given different circulation territory from CCA. Here, we report on a patient with bilateral giant CCAs who presented with both typical and atypical symptoms. CASE PRESENTATION: An 88-year-old hypertensive woman presented with acute vertical oblique binocular diplopia, followed by complete ptosis of the right eye. Ophthalmic examination showed dysfunction of the right third, fourth, and sixth cranial nerves. Further examination revealed hypesthesia of the areas supplied by the ophthalmic (V1) and maxillary (V2) branches of the right trigeminal nerve. Bilateral giant cavernous carotid aneurysms, with a concurrent subacute right occipital lobe infarction, were discovered on brain imaging and angiogram. Additionally, a prominent right posterior communicating artery (PCOM) was revealed. Seven months later, clinical improvement with stable radiographic findings was documented without any intervention. CONCLUSIONS: Dysfunction of the third, fourth, and sixth cranial nerves, and the ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerves, should necessitate brain imaging, with special attention given to the cavernous sinus. Despite unilateral symptomatic presentation, bilateral lesions cannot be excluded solely on the basis of clinical findings. CCA should be included in the differential diagnosis of cavernous sinus lesions. Although rare, ipsilateral posterior circulation cerebral infarction (i.e., occipital lobe infarction) can occur in CCA patients, presumably as a result of distal embolization through an ipsilateral, prominent PCOM. Spontaneous clinical improvement with stable radiographic support may occur.

Outcomes of descemet stripping automated endothelial keratoplasty using imported donor corneas.

BACKGROUND: The lack of development of local donor tissue acquisition in several regions of the world has resulted in the necessity of performing keratoplasty with imported donor corneas. The greatest concern about the use of donor corneas supplied by foreign eye banks is the effect of the increased donor death-to-operation time which inevitably occurs during the tissue recovery, tissue processing, and tissue transfer between the countries. The purpose of this study was to report the outcomes of descemet stripping automated endothelial keratoplasty (DSAEK) using imported donor corneas. METHODS: This retrospective, non-comparative case series investigated the outcomes of the 102 consecutive DSAEK procedures using imported donor corneas performed at a single university-based hospital between August 2006-2014. The main outcome measures were postoperative best-corrected visual acuity (BCVA), endothelial cell density (ECD), and complications. RESULTS: The mean death-to-operation time was 9.52 ± 1.48 days (range, 8-13). The mean preoperative ECD was 2761 ± 285 cells/mm2. Fuchs' endothelial dystrophy was the predominant indication for grafting. The mean follow-up duration was 65.3 months. Ninety-three eyes had improved vision postoperatively (91.18%). BCVA unchanged in 3 eyes due to preexisting macular scar and advanced glaucoma. Primary graft failure occurred in 6 eyes (5.88%). Of the 93 eyes with improved BCVA, 100% had their best corrected vision within the first 1 year. The mean ECD at 6, 12, 24, 36, and 60 months after surgery was 1762 ± 294 cells/mm2, 1681 ± 284 cells/mm2, 1579 ± 209 cells/mm2, 1389 ± 273 cells/mm2, and 1251 ± 264 cells/mm2 respectively. The mean ECD loss at 6 months, 1 year, 2 years, 3 years, and 5 years after surgery was 36.2%, 39.1%, 42.8%, 49.7%, and 54.7% respectively. The most common complication was graft detachment/dislocation (10.78%). There were no cases of any postoperative infection. CONCLUSIONS: DSAEK with imported donor corneas provides rapid and good visual rehabilitation. The percentages of endothelial cell loss were comparable to those achieved in Western series using domestic corneas in which fresher tissues were available for transplantation.

Syphilitic Optic Neuropathy: Re-emerging Cases Over a 2-Year Period.

We report 7 cases of syphilitic optic neuropathy during a 2-year period. All patients were newly diagnosed with human immunodeficiency virus (HIV) infection. Six cases (86%) initially presented with swollen optic disc either unilaterally or bilaterally. Blind spot enlargement was the most common type of visual field defect. Final visual acuity of at least 20/25 was achieved together with visual field improvement and resolution of swollen optic disc. Optic nerve involvement can be the first manifestation of syphilis and HIV co-infection. Syphilitic optic neuropathy has an excellent prognosis if the disease diagnosed promptly and treated properly.

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.

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.

Optimal Duration of Monocular Occlusion to Eliminate Fusion Effect in Intermittent Exotropia.

Purpose: To determine the optimal duration of monocular occlusion in patients with intermittent exotropia. Patients and Methods: This prospective cohort study enrolled 40 patients with intermittent exotropia at Ramathibodi Hospital between May 2023 and September 2023. Angles of deviation at distance (6 m) and near fixation (0.33 m) were measured before and after monocular occlusion for 30, 60, and 90 minutes. All measurements were performed by a single ophthalmologist. The mean angle of deviation was calculated at each time point. Results: The mean age at presentation was 12.5 years (range, 5-33 years). The mean angles of deviation before monocular occlusion were 27.8 ± 15.1 prism diopters (PD) at distance and 26 ± 13.7 PD at near fixation. At distance, mean angles of deviation after monocular occlusion were 32.2 ± 14.6, 32.9 ± 15.0, and 32.6 ± 14.7 PD at 30, 60, and 90 minutes, respectively. At near fixation, mean angles of deviation after monocular occlusion were 37.4 ± 15.0, 39.8 ±14.3, and 39.5 ± 14.6 PD at 30, 60, and 90 minutes, respectively. All angles significantly differed from the pre-occlusion deviation (all P < 0.01). For deviation at distance, there was no statistically significant difference between 60 and 30 minutes of monocular occlusion (P = 0.48). For deviation at near fixation, there was a significant difference between 60 and 30 minutes of monocular occlusion (P = 0.048), but the difference of 2.5 PD was not clinically significant. There were no statistically significant differences between 90 and 60 minutes of monocular occlusion at distance or near fixation (both P = 0.82). Conclusion: Monocular occlusion is necessary to eliminate the fusion effect in patients with intermittent exotropia; 30 minutes of monocular occlusion is sufficient to achieve the maximum angle of deviation.

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.

Health-related quality of life of daily-life-affected benign essential blepharospasm: Multi-center observational study.

PURPOSE: To compare Thais' health-related quality of life (HRQOL) and severity grading, efficacy and safety in daily-life-affected benign essential blepharospasm (BEB) patients at baseline and after Botulinum toxin type A (BTX-A) treatment. DESIGN: Prospective-observational study. PARTICIPANTS: BEB patients with Jankovic rating scale (JRS) at least 3 in both severity and frequency graded from 14 institutes nationwide were included from August 2020 to June 2021. METHODS: Demographic data, HRQOL evaluated by the Thai version of EQ-5D-5L and NEI-VFQ-25 questionnaires, and severity grading score evaluated by Jankovic rating scale (JRS) at baseline, 1, and 3 months after the treatment were collected. The impact of the BTX-A injections and their complications were recorded. RESULTS: 184 daily-life-affected BEB patients were enrolled; 159 patients (86.4%) had complete data with a mean age of 61.40±10.09 years. About 88.05% were female, and 10.1% were newly diagnosed. Most of the patients had bilateral involvement (96.9%) and 12.6% had history of BEB-related accident. After BTX-A treatment, HRQOL improved significantly in 4 dimensions of EQ-5D-5L, except self-care. The EQ_VAS (mean±SD) was 64.54±19.27, 75.13±15.37, 73.8±15.85 (p<0.001) and EQ-5D-5L utility score was 0.748±0.23, 0.824±0.19 and 0.807±0.19 at baseline, 1, 3 months after treatment, respectively. From NEI-VFQ-25, HRQOL also improved in all dimensions, except eye pain. The JRS improved in all patients. Self-reported minor adverse events were 22.6%, which mostly resolved within the first month. CONCLUSION: Daily-life-affected BEB impacted HRQOL in most dimensions from both generic and visual-specific questionnaires. BTX-A treatment not only decreased disease severity, but also improved quality of life.

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.

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.

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.

Comparison of Visual Acuity Measurement Using Three Methods: Standard ETDRS Chart, Near Chart and a Smartphone-Based Eye Chart Application.

PURPOSE: To validate the Rosenbaum near vision card (Near Chart) and a smartphone-based visual acuity (VA) test (Eye Chart) against a standard retro-illuminated Early Treatment Diabetic Retinopathy Study (ETDRS) chart within participants. MATERIALS AND METHODS: A cross-sectional study of participants aged ≥18 years was conducted. VA was measured in all participants using the ETDRS chart, Near Chart and smartphone-based Eye Chart application, respectively. VA was converted to logarithm of the minimum angle of resolution (logMAR) for statistical analysis. Eyes with ETDRS VA worse than 1.0 logMAR (20/200) were excluded. The main outcome measures were levels of agreement between VA measured using the Near Chart or Eye Chart application vs the ETDRS chart. RESULTS: A total of 295 eyes of 151 participants were included. One hundred participants (66.2%) were female and the mean age was 64.3 ± 12.5 years. Educational level was high school or below for 49% of participants and at Bachelor's degree or above for 51%. The median logMAR VAs of all eyes tested using the ETDRS chart, Near Chart and Eye Chart application were 0.1, 0.0 and 0.1, respectively. The median VA difference between the Near Chart vs ETDRS chart and Eye Chart application vs ETDRS chart was 0.0 logMAR in both cases for both the right eye (OD) and left eye (OS). Intraclass correlation coefficient (ICC) demonstrated a strong positive correlation between VA tested with the Near Chart vs ETDRS chart (OD: ICC=0.85; p<0.001, OS: ICC=0.77; p<0.001) and Eye Chart application vs ETDRS chart (OD: ICC=0.88; p<0.001, OS: ICC=0.74; p<0.001). CONCLUSION: VA measurements with the Near Chart and smartphone-based Eye Chart application corresponded well to the standard ETDRS chart, suggesting potential utility of alternative portable VA tests for in-office or remote vision monitoring, particularly during periods of physical distancing such as the Coronavirus disease 2019 (COVID-19) era.

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.

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.