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Myelin oligodendrocyte glycoprotein-antibody-associated disorder: a new inflammatory CNS demyelinating disorder

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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.

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Data availability

The anonymised data of each patient are available with unique alphanumeric code, that will be shared if required by the authors.

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

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Authors and Affiliations

  1. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, 560029, India

    Manjunath Netravathi, Vikram Venkappayya Holla, Atchayaram Nalini, Ravi Yadav, Seena Vengalil, Abel Thomas Oommen, Sultana Shaik Reshma, Nitish Kamble & Pramod Kumar Pal

  2. Department of Psychiatry Social Work, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India

    Priya Treesa Thomas

  3. Department of Neuroimaging & Interventional Neuroradiology (NIIR), National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India

    Bhat Maya

  4. Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India

    Anita Mahadevan

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Contributions

NM: neurologist involved in the concept of the proposal, the guidance of the study, evaluation of the patients, and treatment, analyzing the article and formatting of the final article. VVH: neurologist involved in the evaluation of the patients and treatment, analyzing the results, statistics, and formatting of the final article. AN, RY, SV, LKN: neurologist involved evaluation of the patients and treatment, and formatting of the final article. ATO, SSR: investigator, evaluation of the patients under the neurologist, analyzing the article, and formatting of the final article. PTT: psychiatry social Work involved in psychosocial aspects of the neuropsychiatric features, evaluation of the patients under the neurologist, analyzing the article, and formatting of the final article. BM: neuroradiologist involved in the concept of the study and radiological evaluation of the brain MRI of the patients, analyzing the article and formatting of the final article. PPK: investigator, evaluation of the patients under the neurologist, analyzing the article and formatting of the final article.AM: neuropathologist involved in CBA analysis for CASPR2 antibodies, article formation, analyzing the article and formatting of the final article.

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Correspondence to Manjunath Netravathi.

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Netravathi, M., Holla, V.V., Nalini, A. et al. Myelin oligodendrocyte glycoprotein-antibody-associated disorder: a new inflammatory CNS demyelinating disorder. J Neurol 268, 1419–1433 (2021). https://doi.org/10.1007/s00415-020-10300-z

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  • DOI: https://doi.org/10.1007/s00415-020-10300-z

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