Immunoglobulin A Antibodies Against Myelin Oligodendrocyte Glycoprotein in a Subgroup of Patients With Central Nervous System Demyelination.

Importance: Differential diagnosis of patients with seronegative demyelinating central nervous system (CNS) disease is challenging. In this regard, evidence suggests that immunoglobulin (Ig) A plays a role in the pathogenesis of different autoimmune diseases. Yet little is known about the presence and clinical relevance of IgA antibodies against myelin oligodendrocyte glycoprotein (MOG) in CNS demyelination. Objective: To investigate the frequency of MOG-IgA and associated clinical features in patients with demyelinating CNS disease and healthy controls. Design, Setting, and Participants: This longitudinal study comprised 1 discovery and 1 confirmation cohort derived from 5 centers. Participants included patients with suspected or confirmed demyelinating diseases and healthy controls. MOG-IgA, MOG-IgG, and MOG-IgM were measured in serum samples and cerebrospinal fluid (CSF) of patients, who were assessed from September 2012 to April 2022. Main Outcomes and Measures: Frequency and clinical features of patients who were seropositive for MOG-IgA and double-seronegative for aquaporin 4 (AQP4) IgG and MOG-IgG. Results: After the exclusion of 5 participants with coexisting AQP4-IgG and MOG-IgA, MOG-IgG, and/or MOG-IgM, 1339 patients and 110 healthy controls were included; the median follow-up time was 39 months (range, 0-227 months). Of included patients with isolated MOG-IgA, 11 of 18 were female (61%), and the median age was 31.5 years (range, 3-76 years). Among patients double-seronegative for AQP4-IgG and MOG-IgG (1126/1339; 84%), isolated MOG-IgA was identified in 3 of 50 patients (6%) with neuromyelitis optica spectrum disorder, 5 of 228 patients (2%) with other CNS demyelinating diseases, and 10 of 848 patients (1%) with multiple sclerosis but in none of the healthy controls (0/110). The most common disease manifestation in patients seropositive for isolated MOG-IgA was myelitis (11/17 [65%]), followed by more frequent brainstem syndrome (7/16 [44%] vs 14/75 [19%], respectively; P = .048), and infrequent manifestation of optic neuritis (4/15 [27%] vs 46/73 [63%], respectively; P = .02) vs patients with MOG-IgG. Among patients fulfilling 2017 McDonald criteria for multiple sclerosis, MOG-IgA was associated with less frequent CSF-specific oligoclonal bands (4/9 [44%] vs 325/351 [93%], respectively; P < .001) vs patients with multiple sclerosis who were MOG-IgG/IgA seronegative. Further, most patients with isolated MOG-IgA presented clinical attacks after recent infection or vaccination (7/11 [64%]). Conclusion and Relevance: In this study, MOG-specific IgA was identified in a subgroup of patients who were double-seronegative for AQP4-/MOG-IgG, suggesting that MOG-IgA may be a novel diagnostic biomarker for patients with CNS demyelination.

Optic Nerve Sheath Fenestration as Adjuvant Treatment for Severe Pseudotumor Cerebri Syndrome Induced by All-Trans Retinoic Acid.

All-trans retinoic acid (ATRA) is a vitamin A derivative which can increase intracranial pressure, causing visual loss and papilledema. Those patients should be treated similarly to others patients with idiopathic intracranial hypertension. We described a case of a 32-year-old woman presenting with severe visual loss and intracranial hypertension induced by ATRA for acute promyelocytic leukemia, which was treated clinically and with optic nerve sheath fenestration. Patients receiving ATRA therapy should be monitored to neurological and ophthalmic signs and symptoms of intracranial hypertension.

Contractile peripapillary staphyloma: OCTA documentation of increased peripapillary vessel density during transient visual loss episodes.

PURPOSE: to describe a patient with a contractile peripapillary staphyloma and transient visual loss (TVL) that underwent repeated OCTA examination documenting disc contraction and increased peripapillary vessel density as the mechanism of TVL. OBSERVATIONS: a 28-year-old male presented multiple daily episodes of TVL for the last 5 years. Fundoscopic examination revealed a peripapillary staphyloma. The fundus photographs and SS-OCT demonstrated flattening of the posterior polo and crowding of the contracted optic disk, which became hyperemic with tortuous and dilated veins during visual loss episodes. OCTA showed temporary increased peripapillary vessel density, presumably from severe venous congestion leading to TVL during the contraction. CONCLUSION AND IMPORTANCE: increased peripapillary vessel density can be demonstrated by OCTA during TVL in contractile peripapillary staphyloma. These findings indicate that severe venous stasis during disc contraction is the cause of TVL.

Homonymous quadrantic macular ganglion cell complex loss as a sign of trans-synaptic degeneration from occipital lobe lesion.

PURPOSE: to describe a patient with visual field (VF) defect from an occipital lobe lesion that was found to have macular ganglion cells complex (GCC) quadrantic reduction without significant peripapillary retinal nerve fiber layer (RNFL) loss on optical coherence tomography (OCT). To emphasize that macular GCC loss may be the major ocular manifestation of trans-synaptic optic pathway degeneration in occipital lobe lesions. OBSERVATIONS: A 15-year-old female was investigated after a VF examination revealed a right homonymous inferior quadrantanopia. Fundoscopic examination was completely normal as were the peripapillary retinal nerve fiber layer (RNFL) thickness measurements on OCT. Macular thickness measurements however, revealed superior homonymous quadrantic GCL reduction evidencing retinal neuronal loss in direct correspondence with her VF defect. Magnetic resonance imaging showed a localized left occipital lobe gliotic lesion as the explanation for her VF defect. CONCLUSIONS AND IMPORTANCE: Small post-geniculate optic pathway lesions may lead to retrograde trans-synaptic degeneration that can be detected on OCT-measured macular GCL measurements despite normal peripapillary RNFL estimates. Awareness of such occurrence in important to avoid diagnostic confusion with other anterior visual pathway diseases.

Autologous Internal Limiting Membrane Fragment Transplantation for Large, Chronic, and Refractory Macular Holes.

OBJECTIVE: To evaluate a technique of autologous internal limiting membrane (ILM) fragment transplantation for the treatment of large, chronic, and/or refractory macular holes (MH). DESIGN: This was a 6-month prospective interventional case series. METHOD: Ten eyes of 10 patients with MH underwent pars plana vitretomy (PPV) and ILM peeling followed by transplantation of an autologous ILM fragment to the MH. Six patients had primary MH with an internal diameter greater than 500 µm and a duration of more than 18 months, including 1 patient with nonproliferative diabetic retinopathy previously treated with panretinal photocoagulation. Four eyes with MH had previously been submitted to PPV (i.e. 1 for retinal detachment and 3 to attempt to close large MH). One of the latter also displayed juxtapapillary choroidal neovascularization due to age-related macular degeneration. The primary and secondary outcomes were MH closure and improvement of the best corrected visual acuity (BCVA), respectively. RESULTS: Complete MH closure was achieved in all cases. A statistically significant improvement in the average BCVA was observed after 6 months of follow-up (p = 0.018; paired t test). The BCVA improved in 8 eyes (80%), and in 6 of those eyes it improved by ≥ 15 letters. In 1 patient, the BCVA remained unchanged after the surgery, but the visual field reportedly improved. One patient experienced a slight worsening (0.16 logMAR). Two cases developed atrophy of the retinal pigment epithelium despite MH closure and BCVA improvement. CONCLUSION: Treatment with autologous ILM fragment transplantation seems to be an efficient alternative for large, chronic, and refractory MH.

Structural and functional assessment of macula in patients with high-risk proliferative diabetic retinopathy submitted to panretinal photocoagulation and associated intravitreal bevacizumab injections: a comparative, randomised, controlled trial.

PURPOSE: To compare the efficacy of therapy with panretinal photocoagulation (PRP) and intravitreal bevacizumab (IVB) injections versus PRP alone in patients with high-risk proliferative diabetic retinopathy (HR-PDR) with a 6-month follow-up. METHODS: Forty-two patients with HR-PDR were prospectively studied in a randomised, masked, controlled trial. Both eyes of each patient were randomised either to the study group (SG) receiving PRP plus IVB injections or the control group (CG) receiving PRP alone. Mean change in visual acuity (VA), optical coherence tomography-measured foveal thickness (FT) and macular volume (MV) were compared. RESULTS: Intergroup comparisons showed no significant difference in VA while FT exhibited a significant (p < 0.05) difference at 1 month of follow-up and MV was significantly reduced at the 1- and 3-month follow-up. Compared to baseline, VA was significantly worse at all follow-ups in the CG and was stable in the SG. FT increased significantly in the CG from baseline to the 1- and 6-month follow-ups and in the SG, no significant difference was observed. MV was significantly increased in the CG during all follow-up periods. CONCLUSION: In HR-PDR, using IVB injections as adjuvant treatment to PRP reduces the VA deterioration and results in decreased FT and MV measurements compared to PRP alone.

Bilateral visual loss complicating liposuction in a patient with idiopathic intracranial hypertension.

A 34-year-old obese woman developed blurred vision in both eyes soon after large-volume liposuction of the dorsum and gluteus region bilaterally associated with abdominal dermolipectomy. An ophthalmic examination revealed severe bilateral visual loss and pallid optic disc edema. The patient gave a history of transient obscurations of vision in the past. Neuroimaging studies were non-revealing, but a lumbar puncture disclosed a markedly elevated intracranial pressure. The patient was diagnosed as having had bilateral ischemic optic neuropathy superimposed on pre-existing idiopathic intracranial hypertension (IIH). Acetazolamide treatment was used. Some visual improvement occurred, and optic disc edema evolved into severe optic disc pallor. This case shows that visual loss from optic disc infarction may be a devastating complication of high-volume liposuction in patients with underlying IIH. Because liposuction is frequently performed on obese patients, physicians should screen for signs and symptoms of IIH before undertaking this procedure.