Myelin oligodendrocyte glycoprotein-antibody-associated disorder: a new inflammatory CNS demyelinating disorder
- PDF / 1,282,470 Bytes
- 15 Pages / 595.276 x 790.866 pts Page_size
- 15 Downloads / 148 Views
ORIGINAL COMMUNICATION
Myelin oligodendrocyte glycoprotein‑antibody‑associated disorder: a new inflammatory CNS demyelinating disorder Manjunath Netravathi1 · Vikram Venkappayya Holla1 · Atchayaram Nalini1 · Ravi Yadav1 · Seena Vengalil1 · Abel Thomas Oommen1 · Sultana Shaik Reshma1 · Nitish Kamble1 · Priya Treesa Thomas2 · Bhat Maya3 · Pramod Kumar Pal1 · Anita Mahadevan4 Received: 19 September 2020 / Revised: 28 October 2020 / Accepted: 1 November 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020
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. Keywords MOGAD · MOG · Myelin oligodendrocyte protein · LETM · Longitudinally extensive myelitis · NMOSD · Neuromyelitis optica spectrum disorders · ADEM · Acute demyelinating encephalomyelitis · Leukodystrophy Abbreviations MOG Myelin oligodendrocyte glycoprotein M Male F Female ON Optic neuritis ADEM Acute demyelinating encephalomyelitis CNS Central nervous system MOGAD MOG-associated disorders MOG-EM MOG-IgG associated encephalomyelitis MYE Myelopathy BS Brainstem syndrome OM Opticomyelopathy
HEK Human embryonic kidney cells CSF Cerebrospinal fluid VEP Visual evoked potential BAER Brainstem auditory evoked potential SSEP Somatosensory evoked potential DIR Double inversion recovery, ADEMON ADEM followed by recurrent attacks of optic neuritis EDSS Expanded disability status scale NMOSD Neuromyelitis optica spectrum disorders NMDAR N-Methyl-d-aspartate encephalitis
* Manjunath Netravathi [email protected] Extended author informati
Data Loading...
