The specificity of MOG-IgG1 testing was 97.8%.Ĭonclusions and Relevance This study confirms MOG-IgG1 as a highly specific biomarker for MOGAD, but when using a cutoff of 1:20, it has a low PPV of 72%. The PPV was higher for children (94% ) vs adults (67% ) and patients with high pretest probability (85% ) vs low pretest probability (12% ). The median titer was higher with true-positive results (1:100 ) than false-positive results (1:40 P < .001). Alternative diagnoses included multiple sclerosis (n = 11), infarction (n = 3), B 12 deficiency (n = 2), neoplasia (n = 2), genetically confirmed adrenomyeloneuropathy (n = 1), and other conditions (n = 7). Twenty-six results (28%) were designated as false positive by the 2 raters, with an overall agreement on 91 of 92 cases (99%) for true and false positivity. A total of 92 of 1260 (7.3%) were positive for MOG-IgG1. Among 1260 included patients tested over 2 years, the median (range) age at testing was 46 (0-98) years, and 792 patients were female (62.9%). Results A total of 1617 patients were tested, and 357 were excluded. Cases positive for MOG-IgG1 were independently designated by 2 neurologists as true-positive or false-positive results at last follow-up, based on current international recommendations on diagnosis or identification of alternative diagnoses consensus was reached for cases in which disagreement existed. Testing of MOG-IgG1 used a live-cell fluorescence-activated cell-sorting assay an IgG binding index value of 2.5 or more with an end titer of 1:20 or more was considered positive. Main Outcomes and Measures Medical records of patients who were tested were initially reviewed by 2 investigators blinded to MOG-IgG1 serostatus, and pretest probability was classified as high or low (suggestive of MOGAD or not). Patients without research authorization were excluded. Patients in the Mayo Clinic who were consecutively tested for MOG-IgG1 by live cell-based flow cytometry during their diagnostic workup were included. Objective To determine the positive predictive value (PPV) of MOG-IgG1 testing in a tertiary referral center.ĭesign, Setting, and Participants This diagnostic study was conducted over 2 years, from January 1, 2018, through December 31, 2019.
Positive results on MOG-IgG1 testing by live cell-based assays can confirm a MOGAD diagnosis, but false-positive results may occur. Importance Myelin oligodendrocyte glycoprotein-IgG1–associated disorder (MOGAD) is a distinct central nervous system–demyelinating disease. Shared Decision Making and Communication.Scientific Discovery and the Future of Medicine.
Health Care Economics, Insurance, Payment.Clinical Implications of Basic Neuroscience.Challenges in Clinical Electrocardiography.False-positive cases (E-K): sagittal FLAIR image showing Dawson-finger T2-hyperintense lesions perpendicular to the ventricle, typical of multiple sclerosis (E) axial postgadolinium T1-weighted images showing multiple areas of nodular enhancement in the pons that brainstem biopsy confirmed to be a histiocytic disorder (F) axial T2-weighted image showing a peripheral dorsolateral hyperintense lesion abutting the surface of the spinal cord, in another patient with multiple sclerosis (G) sagittal T2-weighted image showing a faint longitudinally extensive T2-hyperintense lesion accompanied by marked cervical spinal cord swelling with an intralesional cyst (H), also appreciable on axial images (I), which biopsy confirmed to be a glioma sagittal (J) and axial (K) T2-weighted spinal cord MRI showing normal signal intensity and initial atrophy in a young adult man with X-linked adrenomyeloneuropathy. True-positive cases (A-D): axial fluid-attenuated inversion recovery (FLAIR) image showing large, multifocal, poorly demarcated lesions on brain MRI in a patient with an acute disseminated encephalomyelitis attack in MOG-IgG1–associated disorder (MOGAD) (A) axial postgadolinium T1-weighted orbit MRI showing longitudinally extensive enhancement of the left optic nerve sheath in a patient with optic neuritis as a manifestation of MOGAD (B) sagittal T2-weighted images showing a longitudinally extensive myelitis lesion along the lower thoracic spinal cord, with predominant involvement of the central gray-matter on axial images in MOGAD (C and D).
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