Myelin Oligodendrocyte Glycoprotein (MOG)-IgG Associated Demyelinating Disease

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.

Comparison of Clinical and Radiological Features of Aquaporin4 (AQP-4) Antibody Positive Neuromyelitis Optica Spectrum Disorder (NMOSD) and Anti Myelin Oligodendrocyte Glycoprotein (Anti-MOG) Syndrome-Our Experience from Northwest India

“…[ 21 22 25 26 27 ] The larger studies of MOG antibody disease reported a high incidence in paediatric age group and no gender bias, similar to our study. [ 21 22 25 26 27 ] Preceding clinical events were more frequent in MOG positive group while number of patients with relapse were similar in both the groups.…”

“…However, in the previous larger studies ON was found to be more common than myelitis. [ 21 22 25 26 27 ] The spinal cord involvement in all myelitis cases in both the groups of our study was in the form of LETM and we did not find any short segment spinal cord lesions.…”

“…In MOG antibody disease anterior segment of optic nerve was more involved and in AQP-4 antibody disease posterior segment and optic chiasma was more involved as reported in other larger studies. [ 21 22 25 26 27 28 29 ]…”

Efficacy and Safety of Rituximab in Central Nervous System Demyelinating Disorders

“…From Western India, 4 out of 31 MOGAD patients were shifted to rituximab therapy in view of severe disease. [ 25 ] The mean ARR reduced from 1.08 to 0.43 on rituximab with no further relapses on follow up. [ 25 ] In the present study, 4 MOGAD patients received rituximab as a second or third-line agent.…”

“…[ 25 ] The mean ARR reduced from 1.08 to 0.43 on rituximab with no further relapses on follow up. [ 25 ] In the present study, 4 MOGAD patients received rituximab as a second or third-line agent. As mentioned earlier, 2 of them were free of relapses over a period of 2.5 years after initiation of therapy.…”