ABSTRACT
PURPOSE: To characterize the clinical phenotype of myelin oligodendrocyte glycoprotein antibody (MOG-IgG) optic neuritis. DESIGN: Observational case series. METHODS: Setting: Multicenter. Patient/Study Population: Subjects meeting inclusion criteria: (1) history of optic neuritis; (2) seropositivity (MOG-IgG binding index > 2.5); 87 MOG-IgG-seropositive patients with optic neuritis were included (Mayo Clinic, 76; other medical centers, 11). MOG-IgG was detected using full-length MOG-transfected live HEK293 cells in a clinically validated flow cytometry assay. MAIN OUTCOME MEASURES: Clinical and radiologic characteristics and visual outcomes. RESULTS: Fifty-seven percent were female and median age at onset was 31 (range 2-79) years. Median number of optic neuritis attacks was 3 (range 1-8), median follow-up 2.9 years (range 0.5-24 years), and annualized relapse rate 0.8. Average visual acuity (VA) at nadir of worst attack was count fingers. Average final VA was 20/30; for 5 patients (6%) it was ≤20/200 in either eye. Optic disc edema and pain each occurred in 86% of patients. Magnetic resonance imaging showed perineural enhancement in 50% and longitudinally extensive involvement in 80%. Twenty-six patients (30%) had recurrent optic neuritis without other neurologic symptoms, 10 (12%) had single optic neuritis, 14 (16%) had chronic relapsing inflammatory optic neuropathy, and 36 (41%) had optic neuritis with other neurologic symptoms (most neuromyelitis optica spectrum disorder-like phenotype or acute disseminated encephalomyelitis). Only 1 patient was diagnosed with MS (MOG-IgG-binding index 2.8; normal range ≤ 2.5). Persistent MOG-IgG seropositivity occurred in 61 of 62 (98%). A total of 61% received long-term immunosuppressant therapy. CONCLUSIONS: Manifestations of MOG-IgG-positive optic neuritis are diverse. Despite recurrent attacks with severe vision loss, the majority of patients have significant recovery and retain functional vision long-term.
Subject(s)
Autoantibodies/blood , Magnetic Resonance Imaging , Myelin-Oligodendrocyte Glycoprotein/immunology , Optic Neuritis/diagnostic imaging , Optic Neuritis/immunology , Adolescent , Adult , Age of Onset , Aged , Child , Child, Preschool , Eye Pain/diagnosis , Female , Flow Cytometry , Fluorescent Antibody Technique, Indirect , HEK293 Cells , Humans , Immunoglobulin G/blood , Immunosuppressive Agents/therapeutic use , Male , Middle Aged , Optic Neuritis/drug therapy , Papilledema/diagnosis , Phenotype , Retrospective Studies , Transfection , Vision Disorders/physiopathology , Visual Acuity/physiologyABSTRACT
OBJECTIVE. Morphologic changes associated with papilledema may be masked by partial volume averaging effects in images obtained at a slice thickness greater than normal optic nerve thickness. We aimed to compare the diagnostic accuracy of high-resolution 3D T2-weighted imaging performed at submillimeter slice thickness with conventional T2-weighted imaging performed at 5-mm slice thickness for detection of papilledema. MATERIALS AND METHODS. Two blinded neuroradiologists evaluated conventional and high-resolution axial T2-weighted imaging across orbits from 25 patients with clinically proven papilledema and 66 control participants without papilledema. They graded optic nerve sheath distention and optic nerve head configuration, also making a binary determination for presence or absence of papilledema for each set of images. The diagnostic accuracy of each technique was assessed in terms of sensitivity, specificity, positive likelihood ratio, and interobserver agreement. These parameters were compared using the homogeneity of odds ratio and McNemar tests. RESULTS. High-resolution T2-weighted imaging was associated with higher sensitivity (83.3% vs 56.2%, p = 0.0072 for reader 1; 87.5% vs 54.2% for reader 2, p = 0.0001) but unchanged specificity. High-resolution T2-weighted imaging was significantly better than conventional T2-weighted imaging in detecting optic nerve head deformity in patients with papilledema, but there was no difference between two techniques in detection of optic nerve sheath distention. High-resolution imaging also enabled greater interobserver agreement (κ = 0.82) compared with conventional T2-weighted image (κ = 0.62). CONCLUSION. Improved visualization of the optic nerve head afforded by high-resolution T2-weighted imaging translates into better diagnostic performance of MRI in detection of papilledema, with higher sensitivity and interobserver reliability.
Subject(s)
Imaging, Three-Dimensional , Magnetic Resonance Imaging/methods , Papilledema/diagnosis , Adolescent , Adult , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Retrospective Studies , Young AdultABSTRACT
Nonarteritic ischemic optic neuropathy is a rare event in childhood, occurring mostly in the setting of major surgical procedures or hypovolemia. We report on a child who presented with this unusual complication during the course of therapy with sildenafil citrate, which is gaining acceptance as a treatment modality for pulmonary hypertension in the pediatric age group. The ocular complications of this drug have been widely reported in the adult literature, but there are sparse reports, if any, in the pediatric age group. The likely pathophysiology of this condition is reviewed, as are the other ophthalmologic complications of this drug.
