MOG encephalomyelitis: international recommendations on diagnosis and antibody testing

Abstract

Over the past few years, new-generation cell-based assays have demonstrated a robust association of autoantibodies to full-length human myelin oligodendrocyte glycoprotein (MOG-IgG) with (mostly recurrent) optic neuritis, myelitis and brainstem encephalitis, as well as with acute disseminated encephalomyelitis (ADEM)-like presentations. Most experts now consider MOG-IgG-associated encephalomyelitis (MOG-EM) a disease entity in its own right, immunopathogenetically distinct from both classic multiple sclerosis (MS) and aquaporin-4 (AQP4)-IgG-positive neuromyelitis optica spectrum disorders (NMOSD). Owing to a substantial overlap in clinicoradiological presentation, MOG-EM was often unwittingly misdiagnosed as MS in the past. Accordingly, increasing numbers of patients with suspected or established MS are currently being tested for MOG-IgG. However, screening of large unselected cohorts for rare biomarkers can significantly reduce the positive predictive value of a test. To lessen the hazard of overdiagnosing MOG-EM, which may lead to inappropriate treatment, more selective criteria for MOG-IgG testing are urgently needed. In this paper, we propose indications for MOG-IgG testing based on expert consensus. In addition, we give a list of conditions atypical for MOG-EM (“red flags”) that should prompt physicians to challenge a positive MOG-IgG test result. Finally, we provide recommendations regarding assay methodology, specimen sampling and data interpretation.

Magnetic resonance imaging diagnosis of demyelinating diseases: An update

“…22,103 Ovoid lesions are not usually seen. 27 Cortical lesions and leptomeningeal enhancement are seen in 16% and 6% of patients, respectively (Fig. 18).…”

“…102 The frequency of spinal cord abnormalities is reportedly variable (12-78%). 27 According to earlier reports, lower portions of the spinal cord are more frequently involved than the upper spinal cord. some spinal cord lesions might be missed if the whole spinal cord is not scanned); further studies are required to confirm the frequency of spinal cord lesions among MOG encephalomyelitis patients who are diagnosed according to international preliminary criteria for the diagnosis of MOG encephalomyelitis or diagnostic criteria that might be established in the near future.…”

“…The most important roles of magnetic resonance imaging (MRI) in demyelinating diseases include: (i) diagnosis; [1][2][3][4][5] (ii) imaging biomarkers; [5][6][7][8][9][10][11][12][13][14][15][16][17] and (iii) monitoring of sideeffects from disease-modifying drugs. 2,3,[22][23][24][25][26][27] In the present review, characteristic lesions of demyelinating diseases, and differentiation between MS and other demyelinating diseases are discussed. Precise diagnosis of demyelinating diseases is crucial, because treatment strategies differ between pathologies and thus can greatly influence patient prognosis.…”

“…Precise diagnosis of demyelinating diseases is crucial, because treatment strategies differ between pathologies and thus can greatly influence patient prognosis. 2,3,[22][23][24][25][26][27] In the present review, characteristic lesions of demyelinating diseases, and differentiation between MS and other demyelinating diseases are discussed.…”

The widening spectrum of myelin oligodendrocyte glycoprotein antibody‐associated disorders in children

“…In 2018 an international panel of experts on demyelinating disorders met and established guidelines on when to suspect MOG antibody related disorders . In the recent case series by Ramanathan et al, the authors describe four MOG antibody positive paediatric cases who initially presented with focal seizures, without features of optic neuritis, brainstem encephalitis, transverse myelitis, or ADEM.…”