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

Shripad S Pujari; Rahul V Kulkarni; Dattatraya B Nadgir; Pawan K Ojha; Shashank Nagendra; Vikram Aglave; Rashmi D Nadgir; Hemant Sant; Nilesh Palasdeokar; Satish Nirhale; Sunil Bandishti

doi:10.4103/aian.AIAN_627_19

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

Ann Indian Acad Neurol. 2021 Jan-Feb;24(1):69-77. doi: 10.4103/aian.AIAN_627_19. Epub 2020 Mar 3.

Authors

Affiliations

¹ Department of Brain and Spine, Deenanath Mangeshkar Hospital and Research Center, Pune, Maharashtra, India.
² Department of Neurology, Noble Hospital, 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, D. Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India.
⁷ Department of Neurology, Ruby Hall Clinic, Pune, Maharashtra, India.

Abstract

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.

Keywords: Acute disseminated encephalomyelitis; Neuromyelitis optica spectrum disorders; longitudinally extensive transverse myelitis; myelin oligodendrocyte glycoprotein; optic neuritis.