ObjectiveMyelin oligodendrocyte glycoprotein–immunoglobulin G (MOG-IgG) associated disorder (MOGAD) often manifests with recurrent CNS demyelinating attacks. The optimal treatment for reducing relapses is unknown. To help determine the efficacy of long-term immunotherapy in preventing relapse in patients with MOGAD, we conducted a multicenter retrospective study to determine the rate of relapses on various treatments.MethodsWe determined the frequency of relapses in patients receiving various forms of long-term immunotherapy for MOGAD. Inclusion criteria were history of ≥1 CNS demyelinating attacks, MOG-IgG seropositivity, and immunotherapy for ≥6 months. Patients were reviewed for CNS demyelinating attacks before and during long-term immunotherapy.ResultsSeventy patients were included. The median age at initial CNS demyelinating attack was 29 years (range 3–61 years; 33% <18 years), and 59% were female. The median annualized relapse rate (ARR) before treatment was 1.6. On maintenance immunotherapy, the proportion of patients with relapse was as follows: mycophenolate mofetil 74% (14 of 19; ARR 0.67), rituximab 61% (22 of 36; ARR 0.59), azathioprine 59% (13 of 22; ARR 0.2), and IV immunoglobulin (IVIG) 20% (2 of 10; ARR 0). The overall median ARR on these 4 treatments was 0.3. All 9 patients treated with multiple sclerosis (MS) disease-modifying agents had a breakthrough relapse on treatment (ARR 1.5).ConclusionThis large retrospective multicenter study of patients with MOGAD suggests that maintenance immunotherapy reduces recurrent CNS demyelinating attacks, with the lowest ARR being associated with maintenance IVIG therapy. Traditional MS disease-modifying agents appear to be ineffective. Prospective randomized controlled studies are required to validate these conclusions.

Fingolimod (FTY-720), a sphingosine-1-phosphate receptor modulator, is the first US Food and Drug Administration (FDA)-approved oral agent for the treatment of relapsing forms of multiple sclerosis (MS). Two recent phase III clinical studies (TRANSFORMS [Trial Assessing Injectable Interferon vs FTY720 Oral in RRMS] and FREEDOMS [FTY720 Research Evaluating Effects of Daily Oral Therapy in MS]) demonstrated a significant reduction in the annualized relapse rate in patients with relapsing-remitting MS, compared to once weekly interferon β-1a and placebo. Macular edema was a prominent adverse event reported in these and prior studies of fingolimod. Thirteen of 2,564 (0.5%) patients treated with fingolimod in FREEDOMS and TRANSFORMS developed macular edema. Fingolimod-associated macular edema (FAME) appears to be dose-dependent (observed in only 2 patients taking the FDA-approved 0.5 mg dose) and typically resolves upon cessation of therapy. Although a relatively common condition in ophthalmology, most neurologists have not encountered macular edema in clinical practice. The purpose of this review is to educate the neurologist on the etiology, clinical manifestations, diagnostic modalities, and treatment approaches in patients with FAME. We also discuss the use of fingolimod in patients with uveitis and diabetes mellitus, highlight the guidelines for surveillance ophthalmic examination, and outline the key distinguishing features between FAME and optic neuritis.

Purpose of review To review the clinical characteristics, radiological manifestations and treatment of myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) optic neuritis. Recent findings Serum antibodies to MOG have recently been found to be a biomarker of MOG-IgG-associated disorder (MOGAD), a demyelinating disease distinct from both multiple sclerosis (MS) and aquaporin-4-IgG neuromyelitis optica spectrum disorder (AQP4-IgG-positive NMOSD). The phenotype of MOGAD is broad and includes optic neuritis, transverse myelitis, and acute demyelinating encephalomyelitis (ADEM). Optic neuritis is the most common presentation in adults, whereas ADEM is the most common presentation in children. Clinical characteristics suggestive of MOG-IgG optic neuritis include recurrent optic neuritis, prominent disc edema, and perineural enhancement of the optic nerve on magnetic resonance imaging. Although the nadir of vision loss is severe with MOG-IgG optic neuritis, the recovery is typically better than AQP4-IgG optic neuritis and therefore has a favorable overall prognosis. Patients with relapsing disease will often need chronic immunotherapy. Rituximab, azathioprine, mycophenolate mofetil, and monthly intravenous immune globulin are the most commonly utilized treatments. Summary MOGAD is a unique entity that is separate from both MS and AQP4-IgG-positive NMOSD. Recognition of the clinical and radiologic features allow for the correct diagnosis. Future randomized trials will determine the optimal treatment for MOGAD.

Visual loss is a rare, but catastrophic, complication of surgery in the prone position. The prone position increases intraocular pressure (IOP), which may lead to visual loss by decreasing perfusion of the anterior optic nerve. We tested whether the reverse Trendelenburg position ameliorates the increase in IOP caused by prone positioning. Furthermore, we compared two prone positioning set ups. The IOP of 10 healthy awake volunteers was measured in the prone position at 3 different degrees of inclination (horizontal, 10 degrees reverse Trendelenburg, and 10 degrees Trendelenburg) and in the sitting and supine positions in a randomized crossover study comparing the Jackson table and the Wilson frame. In a given eye, all prone IOP values (median [25th-75th percentile] exceeded those of the sitting (15.0 mm Hg [12.8-16.3 mm Hg]) and supine (16.8mm Hg [14.0-18.3 mm Hg]) positions. IOPs in the reverse Trendelenburg, horizontal, and Trendelenburg positions were 20.3 mm Hg (16.3-22.5 mm Hg), 22.5 mm Hg (19.8-25.3 mm Hg), and 23.8 mm Hg (21.5-26.3 mm Hg), respectively (P < 0.001 versus reverse Trendelenburg; dagger P < 0.001 versus horizontal). The reverse Trendelenburg position ameliorated the increase in IOP caused by the prone position. Furthermore, the reverse Trendelenburg position decreased the number of grossly abnormal IOP values (>23 mm Hg) by 50% and 75% compared with the prone horizontal and Trendelenburg positions, respectively. The prone positioning setups did not differ in their effect on IOP. The increase in IOP caused by prone positioning was ameliorated by the reverse Trendelenburg position and was aggravated by the Trendelenburg position. The short time period between changes in position and changes in IOP suggests an important role for ocular venous pressures in determining IOP. Therefore, IOP can be beneficially manipulated by operating table inclination in the prone position.

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