Annals of Indian Academy of Neurology
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Year : 2021  |  Volume : 24  |  Issue : 1  |  Page : 69-77

Myelin Oligodendrocyte Glycoprotein (MOG)-IgG associated demyelinating disease: Our experience with this distinct syndrome

1 Department of Brain and Spine, Deenanath Mangeshkar Hospital and Research Center; Department of Neurology, Noble Hospital, Pune, Maharashtra, India
2 Department of Brain and Spine, Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India
3 Brain and Nerve Clinic, Neurology and Neuro- Opthalmology, Hubli, Karnataka, India
4 Department of Neurology, Grant Medical College and Sir J. J. group of Hospitals, Mumbai, Maharashtra, India
5 Department of Neurology, Sahyadri Speciality Hospital, Pune, Maharashtra, India
6 Department of Neurology, Noble Hospital, Pune, Maharashtra, India
7 Department of Neurology, D. Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India
8 Department of Neurology, Ruby Hall Clinic, Pune, Maharashtra, India

Correspondence Address:
Dr. Shripad S Pujari
425/57, T. M. V. Colony, Gultekadi, Pune 411 037, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aian.AIAN_627_19

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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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