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Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome

BACKGROUND: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. AIMS: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatm...

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Autores principales: Pujari, Shripad S., Kulkarni, Rahul V., Nadgir, Dattatraya B., Ojha, Pawan K., Nagendra, Shashank, Aglave, Vikram, Nadgir, Rashmi D., Sant, Hemant, Palasdeokar, Nilesh, Nirhale, Satish, Bandishti, Sunil
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8061523/
https://www.ncbi.nlm.nih.gov/pubmed/33911382
http://dx.doi.org/10.4103/aian.AIAN_627_19
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author Pujari, Shripad S.
Kulkarni, Rahul V.
Nadgir, Dattatraya B.
Ojha, Pawan K.
Nagendra, Shashank
Aglave, Vikram
Nadgir, Rashmi D.
Sant, Hemant
Palasdeokar, Nilesh
Nirhale, Satish
Bandishti, Sunil
author_facet Pujari, Shripad S.
Kulkarni, Rahul V.
Nadgir, Dattatraya B.
Ojha, Pawan K.
Nagendra, Shashank
Aglave, Vikram
Nadgir, Rashmi D.
Sant, Hemant
Palasdeokar, Nilesh
Nirhale, Satish
Bandishti, Sunil
author_sort Pujari, Shripad S.
collection PubMed
description BACKGROUND: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. AIMS: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients. METHOD: Retrospective data of MOG-IgG positive patients was collected. Demographics, clinical manifestations at onset and at follow up and relapses, anti AQ-4-IgG status, imaging and all investigations were performed, treatment of relapses and further immunomodulatory therapy were captured. RESULTS: In our 30 patients, F: M ratio was 2.75:1 and adult: child ratio 4:1. Relapses at presentation were optic neuritis {ON}(60%), longitudinally extensive transverse myelitis {LETM}(20%), acute disseminated encephalomyelitis {ADEM}(13.4%), simultaneous ON with myelitis (3.3%) and diencephalic Syndrome (3.3%). Salient MRI features were ADEM-like lesions, middle cerebellar peduncle fluffy infiltrates, thalamic and pontine lesions and longitudinally extensive ON {LEON} as well as non-LEON. Totally, 50% patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, Azathioprine, Mycophenolate and Rituximab were effective attack preventing agents. CONCLUSIONS: MOG-IgG related manifestations in our cohort were monophasic/recurrent/simultaneous ON, myelitis, recurrent ADEM, brainstem encephalitis and diencephalic Syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, LETM, LEON and non-LEON. Where indicated, patients need to go on immunomodulation as it has a relapsing course and can accumulate significant disability. Because of its unique manifestations, it needs to be considered as a distinct entity. To the best of our knowledge, this is the largest series of MOG-IgG disease reported from India.
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spelling pubmed-80615232021-04-27 Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome Pujari, Shripad S. Kulkarni, Rahul V. Nadgir, Dattatraya B. Ojha, Pawan K. Nagendra, Shashank Aglave, Vikram Nadgir, Rashmi D. Sant, Hemant Palasdeokar, Nilesh Nirhale, Satish Bandishti, Sunil Ann Indian Acad Neurol Original Article BACKGROUND: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD. AIMS: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients. METHOD: Retrospective data of MOG-IgG positive patients was collected. Demographics, clinical manifestations at onset and at follow up and relapses, anti AQ-4-IgG status, imaging and all investigations were performed, treatment of relapses and further immunomodulatory therapy were captured. RESULTS: In our 30 patients, F: M ratio was 2.75:1 and adult: child ratio 4:1. Relapses at presentation were optic neuritis {ON}(60%), longitudinally extensive transverse myelitis {LETM}(20%), acute disseminated encephalomyelitis {ADEM}(13.4%), simultaneous ON with myelitis (3.3%) and diencephalic Syndrome (3.3%). Salient MRI features were ADEM-like lesions, middle cerebellar peduncle fluffy infiltrates, thalamic and pontine lesions and longitudinally extensive ON {LEON} as well as non-LEON. Totally, 50% patients had a relapsing course. Plasma exchange and intravenous immunoglobulin worked in patients who showed a poor response to intravenous methylprednisolone. Prednisolone, Azathioprine, Mycophenolate and Rituximab were effective attack preventing agents. CONCLUSIONS: MOG-IgG related manifestations in our cohort were monophasic/recurrent/simultaneous ON, myelitis, recurrent ADEM, brainstem encephalitis and diencephalic Syndrome. MRI features suggestive of MOG-IgG disease were confluent ADEM-like lesions, middle cerebellar peduncle fluffy lesions, LETM, LEON and non-LEON. Where indicated, patients need to go on immunomodulation as it has a relapsing course and can accumulate significant disability. Because of its unique manifestations, it needs to be considered as a distinct entity. To the best of our knowledge, this is the largest series of MOG-IgG disease reported from India. Wolters Kluwer - Medknow 2021 2020-03-03 /pmc/articles/PMC8061523/ /pubmed/33911382 http://dx.doi.org/10.4103/aian.AIAN_627_19 Text en Copyright: © 2006 - 2021 Annals of Indian Academy of Neurology https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Original Article
Pujari, Shripad S.
Kulkarni, Rahul V.
Nadgir, Dattatraya B.
Ojha, Pawan K.
Nagendra, Shashank
Aglave, Vikram
Nadgir, Rashmi D.
Sant, Hemant
Palasdeokar, Nilesh
Nirhale, Satish
Bandishti, Sunil
Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome
title Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome
title_full Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome
title_fullStr Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome
title_full_unstemmed Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome
title_short Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease: Our Experience with this Distinct Syndrome
title_sort myelin oligodendrocyte glycoprotein (mog)-igg associated demyelinating disease: our experience with this distinct syndrome
topic Original Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8061523/
https://www.ncbi.nlm.nih.gov/pubmed/33911382
http://dx.doi.org/10.4103/aian.AIAN_627_19
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