Manjunath Netravathi1, Vikram Venkappayya Holla2, Atchayaram Nalini2, Ravi Yadav2, Seena Vengalil2, Abel Thomas Oommen2, Sultana Shaik Reshma2, Nitish Kamble2, Priya Treesa Thomas3, Bhat Maya4, Pramod Kumar Pal2, Anita Mahadevan5. 1. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, 560029, India. sundernetra@yahoo.co.in. 2. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, 560029, India. 3. Department of Psychiatry Social Work, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India. 4. Department of Neuroimaging & Interventional Neuroradiology (NIIR), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India. 5. Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.
Abstract
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.
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.
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Authors: S Jarius; F Paul; O Aktas; N Asgari; R C Dale; J de Seze; D Franciotta; K Fujihara; A Jacob; H J Kim; I Kleiter; T Kümpfel; M Levy; J Palace; K Ruprecht; A Saiz; C Trebst; B G Weinshenker; B Wildemann Journal: J Neuroinflammation Date: 2018-05-03 Impact factor: 8.322
Authors: Giulia Fadda; Cesar A Alves; Julia O'Mahony; Denise A Castro; E Ann Yeh; Ruth Ann Marrie; Douglas L Arnold; Patrick Waters; Amit Bar-Or; Arastoo Vossough; Brenda Banwell Journal: JAMA Netw Open Date: 2021-10-01