Myelin oligodendrocyte glycoprotein-antibody-associated disorder: a new inflammatory CNS demyelinating disorder

• Published in: Journal of neurology 2021-04, Vol.268 (4), p.1419-1433
• Main Authors: Netravathi, Manjunath, Holla, Vikram Venkappayya, Nalini, Atchayaram, Yadav, Ravi, Vengalil, Seena, Oommen, Abel Thomas, Reshma, Sultana Shaik, Kamble, Nitish, Thomas, Priya Treesa, Maya, Bhat, Pal, Pramod Kumar, Mahadevan, Anita
• Format: Article
• Language: English
• Subjects: Area postrema; Brain stem; Central nervous system diseases; Demyelination; Encephalomyelitis; Glycoproteins; Leukodystrophy; Magnetic resonance imaging; Medical prognosis; Medicine; Medicine & Public Health; Meninges; Myelin; Neuritis; Neuroimaging; Neurological diseases; Neurology; Neuroradiology; Neurosciences; Oligodendrocyte-myelin glycoprotein; Optic neuritis; Original Communication; Patients; Pediatrics; Phenotypes; Population studies; Spinal cord; Substantia alba; Vomiting
• ISSN: 0340-5354; 1432-1459
• DOI: 10.1007/s00415-020-10300-z

Background and aims Myelin oligodendrocyte glycoprotein (MOG) is an oligodendrocytopathy resulting in demyelination. We aimed to determine the frequency of MOG-associated disorders (MOGAD), its various clinical phenotypes, and imaging characteristics. Methods All patients with MOGAD were included. Description of the various clinical phenotypes, investigation profile, therapeutic response, differences between pediatric and adult-onset neurological disorders, determination of poor prognostic factors was done. Results The study population consisted of 93 (M:F = 45:48) (Pediatric:40, Adult-onset:47, Late-onset:7) patients with a median age of 21 years. Among the 263 demyelinating episodes; 45.8% were optic neuritis (ON), 22.8% were myelopathy, 17.1% were brainstem, 7.6% were acute demyelinating encephalomyelitis(ADEM), 4.2% were opticomyelopathy and 2.3% with cerebral manifestations. There was exclusive vomiting in 24.7% prior to onset of clinical syndrome, none of them had area postrema involvement. ADEM was exclusively seen in pediatric patients. Poor prognostic indicators included: (i) incomplete recovery from an acute attack, (b) brainstem syndrome, (c) ADEM with incomplete recovery, (d) MRI suggestive of leukodystrophy pattern, (e) severe ON, (f) ADEMON. Conclusions The Spectrum of MOG-associated disorders is wider affecting the brain (grey and white matter) and the meninges. There are various clinical phenotypes and MRI patterns, recognition of which may help in the determination of therapeutic strategies, and long-term prognosis.